Detestable or marvelous: Neuroanatomical correlates of character judgments

Croft KE, Duff MC, Kovach CK, Anderson SW, Adolphs R, Tranel D.

1. Neuropsychologia. 2010 May;48(6):1789-801. Epub 2010 Mar 6.
Department of Neurology, Division of Behavioral Neurology and Cognitive Neuroscience, University of Iowa College of Medicine, IA, USA. kcroft@utdallas.edu




Abstract

As we learn new information about the social and moral behaviors of other people, we form and update character judgments of them, and this can profoundly influence how we regard and act towards others. In the study reported here, we capitalized on two interesting neurological patient populations where this process of complex "moral updating" may go awry: patients with bilateral damage to ventromedial prefrontal cortex (vmPFC) and patients with bilateral damage to hippocampus (HC). We predicted that vmPFC patients, who have impaired emotion processing, would exhibit reduced moral updating, and we also investigated how moral updating might be affected by severe declarative memory impairment in HC patients. The vmPFC, HC, and brain-damaged comparison (BDC) participants made moral judgments about unfamiliar persons before and after exposure to social scenarios depicting the persons engaged in morally good, bad, or neutral behaviors. In line with our prediction, the vmPFC group showed the least amount of change in moral judgments, and interestingly, the HC group showed the most amount of change. These results suggest that the vmPFC and hippocampus play critical but complementary roles in updating moral character judgments about others: the vmPFC may attribute emotional salience to moral information, whereas the hippocampus may provide necessary contextual information from which to make appropriate character judgments. 2010 Elsevier Ltd. All rights reserved.



PMCID: PMC2862792 [Available on 2011/5/1]

Damage to right ventromedial prefronal area abolishes judgment of harmful intent

Department of Brain and Cognitive Sciences, Massachusetts Institute of Technology, Cambridge, MA 02139, USA. lyoung@mit.edu


Neuron. 2010 Mar 25;65(6):845-51.

Abstract

Moral judgments, whether delivered in ordinary experience or in the courtroom, depend on our ability to infer intentions. We forgive unintentional or accidental harms and condemn failed attempts to harm. Prior work demonstrates that patients with damage to the ventromedial prefrontal cortex (VMPC) deliver abnormal judgments in response to moral dilemmas and that these patients are especially impaired in triggering emotional responses to inferred or abstract events (e.g., intentions), as opposed to real or actual outcomes. We therefore predicted that VMPC patients would deliver abnormal moral judgments of harmful intentions in the absence of harmful outcomes, as in failed attempts to harm. This prediction was confirmed in the current study: VMPC patients judged attempted harms, including attempted murder, as more morally permissible relative to controls. These results highlight the critical role of the VMPC in processing harmful intent for moral judgment. (c) 2010 Elsevier Inc. All rights reserved.

Hippocampal damage abolishes stress response

Department of Psychology, Saint Louis University, St. Louis, MO 63103, USA. tbuchan7@slu.edu




Abstract

The hippocampus (HC) is necessary for learning and memory, but it also plays a role in other behaviors such as those related to stress and anxiety. In support of the latter idea, we show here that bilateral HC damage abolishes the cortisol response to psychosocial stress. We collected salivary cortisol, heart rate, and affective responses to the Trier Social Stress Test (TSST) from 7 participants with bilateral HC lesions, 12 participants with damage outside the HC, and 28 healthy normal comparison participants matched to the HC participants on age and sex. HC participants showed elevated pre-stress cortisol, but no cortisol response to the TSST. Heart rate and affective responses in the HC group were similar to those of the comparison groups. Participants with brain damage outside the HC showed stress responses that were comparable to those of the healthy comparison group. These findings support the idea that the functions of the human HC extend beyond learning and memory, and suggest that the HC is necessary for producing the cortisol response to psychosocial stress.



PMCID: PMC2692817 [Available on 2010/6/1]

Localization of Benton Facial Recognition Test and J. Lo.

Department of Neurology, Division of Behavioral Neurology and Cognitive Neuroscience, University of Iowa College of Medicine, Iowa City, IA, USA. daniel-tranel@uiowa.edu




Abstract

Two of the most successful and widely used tests developed by Arthur Benton and colleagues are the Facial Recognition Test (FRT) and Judgment of Line Orientation Test (JLO), which probe visuoperceptual and visuospatial functions typically associated with right hemisphere structures, especially parietal, occipitoparietal, and occipitotemporal structures. Taking advantage of a large database of focal lesion patients (the Iowa Neurological Patient Registry), we used a new lesion-deficit mapping technique to investigate the neuroanatomical correlates of FRT and JLO performance. For the FRT, there were 201 patients with relevant data; of these, 38 were impaired on the FRT, and failure was most strongly associated with lesions in the right posterior-inferior parietal and right ventral occipitotemporal (fusiform gyrus) areas. For the JLO, there were 181 patients with relevant data; of these, 23 were impaired on the JLO, and failure was most strongly associated with lesions in the right posterior parietal region. These findings put new empirical teeth in the localizing value of the FRT and JLO tests, and they extend and sharpen previous work that had pointed to right posterior structures as being important for FRT and JLO performance



PMCID: PMC2853018 Free PMC Article

PMID: 19051129 [PubMed - indexed for MEDLINE]

Focality of clock drawing test determined by error type

Division of Behavioral Neurology and Cognitive Neuroscience, University of Iowa, IA, USA. daniel-tranel@uiowa.edu




Abstract

The Clock Drawing Test (CDT) is widely used in clinical neuropsychological practice. The CDT has been used traditionally as a "parietal lobe" test (e.g., Kaplan, 1988), but most empirical work has focused on its sensitivity and specificity for detecting and differentiating subtypes of dementia. There are surprisingly few studies of its neuroanatomical correlates. The authors investigated the neuroanatomical correlates of the CDT, using 133 patients whose lesions provided effective coverage of most of both hemispheric convexities and underlying white matter. On the CDT, 30 subjects were impaired and 87 were unimpaired (16 were "borderline"). Impairments on the CDT were associated with damage to right parietal cortices (supramarginal gyrus) and left inferior frontal-parietal opercular cortices. Visuospatial errors were predominant in patients with right hemisphere damage, whereas time setting errors were predominant in patients with left hemisphere lesions. These findings provide new empirical evidence regarding the neuroanatomical correlates of the CDT, and together with previous work, support the use of this quick and easily administered test not only as a screening measure but also as a good index of focal brain dysfunction. PsycINFO Database Record (c) 2008 APA, all rights reserved.



PMCID: PMC2834527 Free PMC Article

Neurology of lying-- localizaion

Spence et al. Neuroreport 2001.  longer rt's and activation in VLPFC.
Langleben et al. Neuroimage 2002-- guilty knowledge test (GKT) and fMRI showed activation in SFG and ACC. 
Ganis et al. Cereb Cortex 2003.  right anterior prefrontal cortex was involved in well rehearsed more elaborate lies, whereas a network involving anterior prefrontal cortex involved in spontaneous lies.
Kozel et al J Neuropsychiatry and Clin Neurosci 2005- OF cortex and ACC involved in deceptio
Abe et al. (Brain 2009) noted PD patients are "honest" and have trouble lying, and that left DLPFC is dominant for inhibiting replies, esp truth telling, ie feigning ignorance. 
Karim et al. Cereb Cortex-- TMS of left cortex FACILITATES lying perhaps by relieving moral conflict.
Sellal JNNP 1993- reflex epilepsy, patient had seizures when he lied.  Had meningioma in right anterior clinoid.  Tumor pressed on amygdala. 
Hakun et al Neurocase 2008-- fMRI as lie detector-ventrolateral frontal activated even when lying was not demanded explicitly
Seth et al.  Neuroimage 2006- MEEG can be used for trial by trial detection of lies.
Modell et al.  J Neuropsychiatry and Clin Neurosci 1992-- pathological lying associated with decreased tracer uptake in right thalamus.
Yang et al. Br J Psych 2007.  Increased white matter among pathological liars especially OF, IFG, MFG.
Fenelon et al. BMJ 1991 and other cits.-- Munchhausen's s- associated with bilateral frontotemporal atrophy, hyperperfusion of right thalamus, cerebral palsy, high signal in PVWM bihemispheric. 
Grezes et al J Neurosci 2004.  detecting deception activates amygdala and rostral ACC.
Harada et al.  Neurosci Research 2009- moral and lie judgments activate VMPFC, lateral OF, left temporal, left temp-par junction, and right cerebellum.
Etcoff et al. Nature 2000- loss of language due to left MCA stroke was associated with increased ability to detect deception.
Stuss et al. Brain 2001-  bilateral esp right OF lesions impaired ability to detect deception.
Autistics have trouble lying.

Go, no go task, parcellated and localized within the VLPFC and pre SMA

Chikazoe J.  Localizing performance of go/no-go tasks to prefrontal cortical subregions.  Curr Opin Psych 2010; 23: 267-272. 

Author divides go/no go tasks into components including working memory, stimulus-driven attention ( reorienting of attention), error monitoring, top down control processes, and response inhibition.  The literature suggests the VLPFC is key to response inhibition (see Buxbaum et al, Hum Brain Mapping 2005).  However, DLPFC and pre-SMA also are recruited for this task and for stop-signal and for antisaccade tasks.

Transcranial magnetic stimulation of right VLPFC disrupts response inhibition, but stimulation of left IFG, DLPFC, dorsal premotor cortex, and right angular gyrus does not.  Subregions of the VLPFC play different roles.  The subregions are posterior IFG, inferior frontal junction, and IFG/insula. 

pIFG usually activates with go/no go stimuli.  IFJ, located at border of inferior frontal sulcus and precentral sulcus, is associated with processing infrequent stimuli.  Thus pIFG is the "core region" for go/no go response inhibition. The same area is involved in reorienting attention (see Corbetta M, Neuron 2008).  The reorienting attention network involves pIFG, IFJ, IFG/insula, and temporal parietal junction.  This is segregated from a dorsal network involving the frontal eye fields (FEF), and intraparietal sulcus that employs a top down mechanism . pIFG may activate with both networks and provide the communication between the two. 

IFJ is involved in stimulus driven attention and is more involved in processing infrequent stimuli, such as infrequent go or infrequent no go on go/no go tasks.  By contrast pIFG is only activated on infrequent no go. 

IFG insula is activated, but is tough to differentiate from insula and IFG insula is also activated in many other tasks.  Its function is not specific and it may be more related to task awareness. 

Pre SMA on right is also core to response inhibition.  This is true on both lesion studies and TMR studies.  Pre SMA is robustly activated during go/no go whether the task is simple or complex or whether the test is oral or manual.  Pre SMA is thought to be important to conflict resolution of task.  The anteriror cingulate gyrus (ACC) activates for error processing.  DLPFC activation may be related to top down processes and working memory. 

Hemispheric asymmetry with right sided dominance is noted in most studies especially for pIFG.  However the left pre SMA and VLPFC may be important.  "Balance" may be disrupted with left sided lesions that can thus cause deficits on task also. 

Coin rotation test validation

 

Hill BD et al. The Neurologist. 2010; 16: 249-253

 

Authors validate a longstanding easy test for fine motor processing used for decades at LSU in 86 normals.  Task consists of counting number of 180 degree rotations of a quarter in ten seconds by the dominant and nondominant hands.  A correction for drops is used, but not that important.  Task is to rotate a quarter using thumb and fingers one and two in ten seconds with an examiner using a timer and counting.  If the coin is dropped, the subject gets another ten seconds.  The adjusted score is the number of rotations in ten seconds minus (0.1 x rotations x drops).  Traditionally, LSU has used a cut score of ten to indicate impairment.  Authors believe a cut score (for both hands) of 13 is better, with increased sensitivity and some loss of specificity.

 

Hyperfamiliarity for faces (HFF) in epilepsy- left temporal localization?

Devinsky O  et al.  Neurology 2010;74: 970-974

Author reviews literature and attributes the first case to Kraepelin (1886).

Case one-- 46 yo policeman had a GTCS preceded by an aura of anxiety, deja vu and sulfuric smell. One month later the patient developed HFF (everyone seemed familiar), memory problems, distractibility and hyperemotionality for sadness.  FRT performance was low average but patient was impaired on Rey O delayed recall.

Case 2-  62 yo rh attorney developed CPS at age 31 with sense of doom, palpitations and nightmares.  Seizures involved HFF,

table has 2 more new cases and 5 old cases

Pertinent facts about HFF- specifically absent are delusions, hyperfamiliarity for nonface objects, or auditory hallucinations.  Lesions involved left hemisphere (5 cases), bilateral (2 cases) or unknown (2 cases). 

Authors cite that the hippocampus is important for recollection and the perirhinal cortex for feeling of familiarity.  Deja vu and deja vecu occur more often with right hemisphere lesions establishing "right sided dominance for familiarity."  The normal electrical response for familiarity is suppression v. activation for novelty stimulus.  Authors speculate the left (impaired) side is unable to recognize faces (nvelty signallig) whereas the right (unipaired) side is in overdrive and falsely signal familiarity. Patients quickly accept their familiarity is false, due to intact right hemisphere structures otherwise.   Contrtast to reduplication syndromes (Capgras) with right hemispheric and bifrontal abnormality. 

Emotional perception deficits in ALS

Zimmerman EK, Eslinger PJ, Simmons Z, Barrett AM.  Cog Behav Neurol 2007; 20: 79-82

Cognitive deficits occur in up to half of ALS patients, particularly bulbar ALS, including early bulbar ALS patients who are not demented. 

13 subjects with bulbar ALS  from teh Hershey ALS Center were given 2 tasks: the emotional faces task, in which they were required to point or say the word denoting the emotional facial expression (happy, sad, angry, afraid, disgusted).  Second, they were asked to respond to tape recording of emotionally intoned sentenced and identify the emotion conveyed. 

The ALS group did worse on the MMSE and GDS, control tasks for dementia and depression.  They also did worse on the facial task, especially for faces connoting sad, disgusted and surprised faces.  They did normally on the prosody task, but by emotion did worse on the surprised prosody recognition task. 

Scales ADHD

 

* Conners parent-teacher rating scale

 

*ADHD Rating Scale

 

* SNAP IV Parent Teacher Rating (www.adhd.net)

 

* *** (best)Vanderbilt Assessment Scale (Parents and teachers) (www.nichq.org) this is easy to get online and is free

 

In adults get

*adult ADHD report scale available at  http://www.med.nyu.edu/psych/psychiarist/adhd.html

 

others

*Conner's Adult ADHD Rating scales

 

*Adult ADHD rating scale IV

 

*Wender Utah Rating Scale

 

*Barfkley's Current Symptoms Scale

 

 

Psychosis and EEG abnormalities as manifestations of Hashimoto' sencephalopathy

Sporis D, Habek M, Mubrin Z et al.  Cog Behav Neurol 2007: 20:138-140. 

Authors claim there are two forms:  a vasculitis type with strokelike manifestations  and seizures, and a diffuse progressive type with dementia and psychiatric manifestations.  Authors present a 38 year old woman (type 2) with a history of RA, and depression, and a history of hypothyroidism (not mandatory for HE), with acute psychosis with auditory hallucinations and bizarre behavior, a CSF pleocytosis (37 WBC, normal protein) normal memory, EEG showing slowing.  Behavioral exam abnormal calculations, constructional praxis, and attention.  CSF showed 12 cells. MRi showed punctuate lesions in left frontal gyrus. Treatment with methylprednisoline 1000 mg per day for three days followed by prednisone 50 mg per day for one month resulted in resolution of all symptoms and EEG, 

Authors discuss that half of patients have MRI abnormalities that can mimic tumor, granuloma, infection, stroke, degeneration, or leukodystrophy.  Several patients had other autoimmune abnormalities including RF, or p ANCA. Etiology of condition is not known.

Isolated inability to write cursively after transient ischemic attack (TIA)

Popescu I-M, Vaidya NA.  Cog Behav Neurol 2007; 131-135.  Case report

78 year old right handed truck driver whose TIA was bilateral upper extremity weakness that resolved the same day. Post TIA he could write block letters but not cursively.  On neurologic/behavioral examination, he complained of being unable to remember what he had read.  He was diagnosed with obstructive sleep apnea.  Elemental neurological examination was normal.  Behavioral examination showed a normal MMSE (29/30, minus one for recall) with perseverations noted  on an alternating programs test and Luria loops.  The patient had trouble with cursive copying and writing.  MRI could not be done (pacemaker); Spect showed bilateral hypoperfusion of the parietal and occipital lobes. Literature review of lesions suggests implied lesion of left superior parietal area (Alexander et al, Arch neurol 1992), parieto occipital white matter bilaterally (Baxter and Warrington, JNNP 1986).  Hanley JR, and Peters , S(Cortex, 1996) showed the opposite , in a patient who had a left hemisphere lesion, with impaired spelling of lower case letters  in block and had excellent cursive writing.  Ohno  et al. (Neurology, 2000) had a patient with pure apractic agraphia due to a thalamic lesion which blocked motor programming, and who could not write kana, kanji, Roman alphabet or Arabic numerals.  Otsuki et al. (JNNP, 1999) reported a patient with apractic agraphia due to a hemorrhage in the left superior parietal lobe. Authors believe cursive and block writing are represented differentially.

Finger agnosia in Alzheimer's disease

Shenal BV, Jackson MD, Crucian GP, Heilman KM.  Cog Behav Neurol 2006; 19:202-203.

Authors compared patients with Alzheimer's disease seen at Memory disorders clinic at University of Florida, comparing 38 AD patients and 10 normal age matched controls.  Subjects with Alzheimer's disease had trouble naming fingers, especially their index finger (correct answers allowed were index finger, forefinger, and "dog" finger."  All normal controls named all three fingers tested (including thumb, index finger, and pinky). AD patients 37 % could not name index finger, 5% could not name their thumb, 13 % could not name their pinky.   None could name index finger and not name thumb or pinky. 

Stratified by severity, with mild AD patients (mean MMSE of 23), moderate (mean MMSE of 19) and severe (mean MMSE of 11), 14 % of mild patients cold not name their index finger, 45 % of moderates could not, and 54 % of severe patients could not.

Ventromedial prefrontal cortex modulates fatigue after penetrating TBI

Pardini M, Krueger F, Raymont V, Grafman J.  Neurology 2010; 74:749-754.

97 PBI patients and 37 controls from Vietnam Head Injury Study were enrolled.  Fatigue was assessed via questionnaires (Krupp Fatigue Scale).  CT scan localization of lesions was made to nonfrontal, ventromedial prefrontal, and dorsolateral /lateral prefrontal.  All 3 areas were associated with fatigue, but only VM was associated with increasing fatigue with larger lesion volume.  Authors note this may allow linkage of fatigue to other ventromedial functions such as  effort and reward participation. 

Note one of references was a John Deluca functional MRI paper showing role for same area in MS related fatigue J Neurol Sci 2008; 270:28-39.

Cognitive effects of pregabalin in healthy volunteers

a double blind, placebo controlled trial  Salinsky M, Storzbach D, Munoz S.  Neurology 2010; 74: 755-761.

32 healthy volunteers were randomized to pregabalin (300 bid) or placebo for twelve weeks and underwent cognitive tests before and after.  Subjects on pregabalin showed worse performance on 3 of 6 target cognitive measures, including digit symbol test, Stroop, and Controlled Oral Word Association Test.  They were paralleled by changes on the Portland Neurotoxicity Scale.

Parcelling frontal functions

Van Snellenberg JC, Wager TD.  Cognitive and motivational functions of the human prefrontal cortex.   from Christensen AL, Goldberg E, Bougakov D.  Luria's Legacy in the 21st Century. Oxford, University Press, 2009.  pp. 30-61.


Chapter summarized authors' (esp Wager's) meta-analyses of functional imaging studies.  These involve working memory, task switching, response inhibition, and emotion. cites Stuss and Alexander, 2007. See chapter.

Luria-- Semantic fragments for repetition and recall

These stories have layers of meaning and complexity

1. Tolstoy's  The Hen and the Golden Eggs-  A man had a hen which laid golden eggs.  He wanted to get more of the eggs at once, and so he killed the hen.  But inside he found nothing; it was just a hen like any other.

2.  Tolstoy's The jackdaw and the Pigeons.  A jackdaw heard that some pigeons had plenty of food.  She painted herself white and flew into the pigeons house.  The pigeons thought she was a pigeon, and took her in.  However, she forgot herself and cried like a jackdaw.  The pigeons then realized she was a jackdaw and sent her away.  She went back to her family, but they did not recognize her, and would not have her either.

3.  Tolstoy's The Ant and the Pigeon.  An ant went down to the stream to drink.  A wave swamped him and he began to drown.  A pigeon flew past him and threw him a  twig.  He climbed on the twig and was saved.

The next day, a hunter set a net and caught the pigeon.  When he took it from the net, however, the ant crawled up stealthily and bit the hunter on his hand.  The hunter cried out and the pigeon flew away. 

4.  The Lion and the mouse.  A lion was asleep and a mouse ran over his body.  The lion awoke and caught the mouse.  The mouse begged him to let her go.  The lion laughed and let her go.  The next day, a hunter caught the lion and tied him with a rope to a tree.  The mouse came through stealthily, gnawed through the rope, and set the lion free.

5.  The Lion and the Fox.  The Lion grew old and  could no longer hunt animals.  So the lion had to learn how to live by cunning.  He lay in his den and pretended to be ill.   The animals came up to him and he ate them.  One day a fox came but would not go into his den.  The lion asked her "Why don't you come in?" The fox replied, " I can see tracks coming into your den but none coming out." 

6.  The Stupid Dog.  A dog was walking over a bridge at night and saw the reflection of the moon in the water. The dog decided it was cheese and jumped into the water.  But of course, she found nothing and only got wet.



Results  patients who had trouble recalling individual words sometimes had no trouble with semantic fragments, and normals had no trouble with 2 successive fragments.  Amnesics would present the semantic content of one part of the story but forget the second part (retroactive inhibition). 

Luria-- sentence repetition-- aphasia or amnesia??

AR Luria.  The Neuropsychology of Memory VH WInston and Sons, Washington , D.C. 1976 p. 103

Patients with vascular lesions could repeat one sentence, had trouble with the second one.  Patient either reverts to first sentence or contaminates elements, displaying "recency effect" but also simplifying the structure of the sentence while maintaining its elements.  However, patients with temporal lesions showed no "freezing or inert repetition" as in frontal lesions.

Sentence recall-- Luria

Although list learning requires multiple presentations, normals can easily recall one and then a second 7 or 8 word sentence (simplified if aphasic) and then repeat both sentences in order. 

Examples
first sentence:  "Apple trees grew in the garden  behind the high fence" or "The hunter killed a wolf on the edge of the forest.  "  Second sentence  :  " In Tashkent there was an earthquake and many houses were destroyed" or "In Tushino there was an air display and parachute jumps." 

Simpler versions "The boy is cold" or "the girl is asleep" or "the boy hit the dog" or "the girl drinks tea"

Results: Amnesics could repeat the sentences immediately, or after empty or filled pauses, with some trouble if the pause was filled with rapid speech.  However, when given a second sentence, they either lost the first or mixed up the elements. 

Disturbance of seriality due to left temporal lesions

Luria in Neuropsychology of memory states that left temporal lesions cause a disturbance of retroactive inhibition in which the last word of the list inhibits recall of predecessors on the list, especially with longer lists.  The effect only occurs with lists presented and reproduced verbally.  Luria gives original credit to Lashley

Lashley KS.  Functional determination of cerebral localization.  Arch Neurol Psych 38: 1937.

Learned motor responses.

Subject is asked to raise his left hand to one tap, and his right hand to 2 taps, or to raise his hand to one tap and to refrain from raising his hand to 2 taps. 

More complicated version: patient is asked to respond to a fist with a finger, or to a finger with a fist.  Or, he is asked to responnd to two taps with one tap, and to one tap with 2 taps. 

More complicated yet.  the subject is asked to respond to a weak tap with a powerful hand movement, and to a powerful tap with a weak hand movement.  Or, to respond to a short acoustic stimulus with a slow movement, and a long stimulus with a prolonged movement. (cf  Marushevskii M, Disturbance of the simplest forms of voluntary action in local lesions of the frontal lobes.  in Luria AR, Khomskaya ED (eds), The frontal lobes and the regulation of psychological processes. Moscow, MGU Press, 1966.

Luria's adaptation:  He would give one task, and when mastered give a second, then without repeating instructions, go back to the first, and then the second task.  Normals had little trouble either learning the task or switching.  Neither empty or filled paused had much effect, (except occipital lesions for visual tasks and temporal lesions for auditory tasks).  Massive tumors had problems with task. 

Patients with thalamic lesions could learn the task, but not do it verbally.  These patients learned the task, were not affected by interference, but could not talk about it. 

Unadze illusion fixed set test

From Luria's book Neuropsychology of  Memory

Unadze DN.  Experimental psychological investigations.  Moscow.  Nauka.  1966.

A subject is given 2 balls of different sizes to squeeze (one per hand) 10-15 times for several seconds per squeeze.  He compares their sizes and reports his finding.  Then, without visual inspection he is given 2 balls of equal size and asked to compare the sizes.  The result is that the hand that held the larger ball now reports it is holding a smaller ball, and is maintained even after 12-18 trials.

Luria's variation was to test normal, mildly and severely impaired subjects on the test.  He found that in severely amnesic subjects only, and only with interference, the fixed set effect was achieved but rapidly forgotten, underlying the importance in interference on somatic memory. Pauses less than 2 minutes had little effect on retention, as with other types of learning.

Gerstmann's syndrome reader

Kombos T,  Picht t, Suess O.  Electrical Excitability of the Angular Gyrus. Journal of Clinical Neurophysiology 25(6) December 2008 .
Comment.  This is the first report of direct electrical stimulation of AG in humans. Stimulation resulted in responses in contralateral upper extremity in 3/5 patients.  No lower extremity stimulation was found.


Anema HA.a,  Kessels RPCcd, de Haan EHFe,  Kappelle L,  Leijten F, van Zandvoort Martine , Dijkerman,H.  Differences in finger localisation performance of patients with finger agnosia

 19(14) September 2008 . 
Comment : 3 patients with AG lesions were able to point with their finger effectively
to  a tactile stimulus (body schema intact) but unable to do so with a drawing  of hand or to name the finger. 
Other body parts did not have this dissociation between body schema and body image.

Tamura I, Kikuchi S, Otsuki M, Tashiro K. The writing of arabic numerals, kanji, and kana in brain-damaged patients. 14(6), 6 May 2003, pp 861-865.
Comment.  4 patients with Gerstmann's syndrome and 4 with Wernicke's aphasia were compared.  All patients could copy letters, and were righthanded.  Subjects were presented with coins, and then asked to write the respective number value in kana , Kanji and Arabic numerals. For GS patients, kanji writing was better than Arabic, that was better than kana writing.  For WA aphasia patients, Arabic numeral writing was better than kana and kanji. Discussion: "In the case of GS, there was a problem in the concept of number.  In the case of WA, the concept of number was relatively well preserved." GS subjects made substitution errors in Arabic number writing.  Authors postulate a deficiency processing recall of somesthetic graphemes linked to the  concept of number.


Wingard E, Barrett  A, Crucian G, Doty, L, Heilman, KM.  The Gerstmann syndrome in Alzheimer's disease. Journal of Neurology, Neurosurgery & Psychiatry  72(3) March 2002 .
Comment  :  among 38 patients with Alzheimer's disease, the four constituent components of Gerstmann's syndrome did not cluster together, leading the authors to speculate that their association is fortuitous due to contiguous networks, and that the four features of Gerstmann's syndrome do not share a common network.

Baciul M, Koenig O, Vernier M,  Bedoin N, Rubin C, Segebarth C. Categorical and coordinate spatial relations: fMRI evidence for hemispheric specialization. Neuroreport 10(6) April 1999.
Comment: 16 healthy males were examined on a coordinate and a category task using fMRI paradigm.  The category task was whether a dot was above or below a horizontal line. The coordinate task was whether the distance between the dot and the line was within a reference distance.  The coordinate task related to right AG activation which decreased over time.  The category task depended upon Left AG activation which did not decrease over time and in fact increased over time.  Results subserve the hypothesis that the left hemisphere subserves the development of new spatial categorization.

Cognition and Fragile X syndrome

Fragile X problems persist into adulthood (even though it is considered a pediatric disease). Two separate conditions exist. Patients with the premutation (55-200 repeats of the FMR1 gene) have RNA toxicity but not full blown cognitive abnormalities. RNA toxicity can include primary ovarian insufficiency, fragile X associated tremor ataxia syndrome (FXTAS), fibromyalgia, hypothyroidism, and psychiatric issues such as anxiety and depression. Boys in this range can experience shyness, social anxiety disorders, ADHD and autism spectrum disorders. This is due to excess FMR1 gene.

Patients with the full mutation (greater than 200 repeats) have LACK of FMR1 and Fragile X syndrome (opposite of above in which there is excess RNA present). They present with severe cognitive abnormalities. In boys this manifests as autism, in girls as severe learning disabilities. They also have connective tissue disorders, agression, and psychiatric problems as above. Males with FXS usually have IQ below 70 (severe) but 15 % are "high functioning" with IQ above. High functioning is due to mosaicism within cells or within methylation and the degree of impairment is related to the amount of FMRP present. Among girls, 40 % have a normal IQ, 35 % are borderline, and 25 % have an IQ below 70. The activation percentage (pct of normal X that is active) correlates with disease and that and amount of FMRP available also affects functioning of offspring.

FXS is the most common cause of intellectual impairment and of autism spectrum disease. The full mutation occurs in 1:2500 in population, and the premutation occurs in 1:250 girls and 1:800 boys in the general population. Genetics: FXS patients always have their mother as a carrier. Fathers often pass the premutation on, to their daughters and it does not expand to a full mutation. Mothers with premutation often have expansion to full mutation in their offspring, especially if the number of repeats is greater than 100.

See also posts on FXS and FXTAS on www.neurologyminutiae.blogspot.com

Cognition and dystrophinopathy

Duchenne's muscular dystrophy has associated delayed milestones especially language, global cognitive impairment, and occassionally autism. Becker's muscular dystrophy has less common findings, but also, compared to general population, has more learning disabilities with normal intelligence, autism, attention and behavioral problems. Orofacial problems may occur.

Botteron S, Verdabout CM, Jeannet PY, Kiliardis S. Orofacial dysfunction in Duchenne Muscular Dystrophy. Arch Oral Biol 2009; 54(1): 26-31.

Cyrulnik SE, Fee RJ, De Vivo DC, et al. Delayed developmental language milestones in children with Duchenne's muscular dystrophy. J Pediatr 2007; 150 (5): 474-478.

Hinton VJ, Fee RJ, Goldstein EM, De Vivo DC. Verbal and memory skills in males with Duchenne muscular dystrophy. Dev Med Child Neurology 2007; 49 (2): 123-128.

Young HK, Barton BA, Waisbren S, et al. Cognitive and psychological profile of males with Becker muscular dystrophy. J Child Neurol 2008; 23 (2): 155-162.

Luria audiomotor integrative tasks

Pitch tests should be kept simple, give 2 sounds ask which is higher or lower.

reproduction of tones

reproduction of rhythmic taps first by imitation, then by verbal instruction, then by reproduction with verbal enforcement



Tactile functions p. 444

discriminative sensation "epicritic" (Head, 1920).
Ideas test with head and point of pin, patient must discriminate which, or use 3 items and ask patient to discriminate. Localize touch by asking patient to point to spot, and make it harder by asking him to point to same spot on the opposite limb. Use Weber's touch compass to discriminate 2 point differentiation threshold in millimeters. Also use it to differentiate Double simultaenous stimulation which is difficult in patients with right parietal injury. Finally measure up and down movements and ability to trace numbers and letters in the hand. Cites Teuber, 1959. Notes that lesions is left hemisphere for are tightly organized in postcentral area, but right hemisphere lesions are more distributed and even left hemisphere lesions occassionally cause left arm abnormalities.

Kinesthetic function (position sense)
simple move a finger, toe, arm and ask whether its up or down (or mimic with other side)
More complicated-- mimic posture in the other hand.

Asterognosis
differentiates from amorphosythesis (denny brown 1952, 1958).

More Luria tests

from "Higher Cortical Functions in Man" p. 428

Complex motor functions

1. Reaction tests-- basic format is for subject to raise his hand in response to a tap or another stimulus. This then can be the basis of more complex tests.
Abnormalities are rarely seen with simple reaction tests, exceptions being "freezing" in position in htn, motor impersistence in frontal lesions, or premature responses in frontal lesions.
Next step is to respond alternately, eg. raise the left hand to one tap, and the right to two taps, or raise the hand with one tap and refrain from moving the hand with two. Start with strict alternating pattern, eg a-b-a-b-a-b- then suddenly break the stereotype and go out of order eg b-b and see what the patient does. Alternately, can ask for an oral answer to a stimulus eg. to one tap, say "I must" or "left" and to 2 taps "I must not" or "right." The third stage is to tell the subject to respond orally and perform appropriate motor action at same time (cites Kohomskaya, 1956, 1958). Then give a complex response, eg to one stimulus give a weak response, and to another a strong response of same hand.

2. " Raise finger if I raise fist, and fist if I raise my finger"
3. Ask patient to copy a series of figure eg. circle, triangle, square and cross, then a second series eg square square triangle, cross and see if they perseverate. May use five elements. May see forget last response, perseverate, not remember etc. Temporal lobe lesions may make it difficult to copy to verbal instruction but OK to copy from sight. Differentiate the two by asking patient to repeat the command before performing it.

4.

Apathy with subthalamic stimulation (and face recognition)

apathy evaluation scale
http://www.tbims.org/combi/aes/AES.PDF

Le Jeune et al. Neurology 2009; 73:1746-1751.

Studied STN stimulation in PET study. All had bilateral STN implantation. 12 patients showed worse apathy 3 months after STN stimulation. Motor function was used. The AES was used. PET showed positive correlation with right Brodmann's areas 10 (r frontal middle gyrus) and area 46 (R IFG) and negative correlation with right posterior cingulum (are 31) and left media frontal lobe ( area 9).

Postop neuropsych differed on WCST perseverative errors and Stroop.

raloxifene improves verbal memory in postmenopausal women

Raloxifene improves verbal memory in late postmenopausal women: a randomized, double-blind, placebo-controlled trial; Jacobsen DE, Samson MM, Emmelot-Vonk MH, Verhaar HJ; Menopause (Nov 2009)

OBJECTIVE:: The aim of this study was to examine the effects of raloxifene compared with those of placebo on verbal memory, mental processing speed, depression, anxiety, and quality of life. METHODS:: A randomized, double-blind, placebo-controlled trial of 213 healthy women 70 years or older was conducted between July 2003 and January 2008 at the University Medical Centre Utrecht, the Netherlands. Participants were randomly assigned to receive raloxifene (60 mg) or placebo daily for 12 months. Measurements were taken at baseline and after 3, 6, and 12 months. The change in scores from baseline was calculated. The main outcome measures were direct and delayed verbal memory (Groningen 15 Words test), mental processing speed (Trails B test), mood/depression (Geriatric Depression Scale), anxiety (State-Trait Anxiety Inventory 1 and 2), and quality of life (Women's Health Questionnaire and EuroQol-5 dimensional questionnaire). RESULTS:: Direct verbal memory improved significantly with raloxifene compared with placebo: the women receiving raloxifene repeated more words in the words A + B test than did the women receiving placebo (P = 0.025). At 12 months, the change from baseline was 16 words in the raloxifene group and 10 words in the placebo group. In the words A test, direct repetition was also significantly better among women receiving raloxifene than among women receiving placebo (P = 0.023), with the change from baseline in the number of words repeated being nine words in the raloxifene group and six words in the placebo group at 12 months. CONCLUSIONS:: In postmenopausal women, raloxifene gave significantly improved verbal memory when compared with placebo.

WHIMS MRI study brain atrophy and estrogen

NEUROLOGY 2009;72:135-142

 

1403 women aged 71-89, a subset of WHI trial, were scanned on average four years apart.  Frontal lobe volume was less at followup in women in estrogen or estrogen plus progesterone arms.  Temporal lobe and total brain volume were not affected as markedly. The adverse effects are most evident in women experiencing cognitive deficits before initiating hormone therapy.

The cognitive measure used was the modified MMSE 3M

18. Teng EL, Chui H. The Modified Mini-Mental State (3MS) Exam. J Clin Psychiatry 1987;48:314–318.

Confabulation and other delusional syndromes

Confabulation is usually associated with memory (medial temporal or diencephalic) and executive (bifrontal) dysfunction.^7-9

 

7. Benson DF, Djenderedjian A, Miller BL, et al. Neural basis of confabulation. Neurology 1996;46:1239–1243.[Abstract/Free Full Text]
8. Moscovitch M, Melo B. Strategic recall and the frontal lobes: evidence from confabulation and amnesia. Neuropsychologia 1997;35:1017–1034.[Medline]
9. Johnson MK, Hayes SM, D'Esposito M, Raye CL. Confabulation. In: Grafman J, Boller F. Handbook of Neuropsychology,

The anterior parahippocampal cortex (perirhinal cortex, Brodmann areas 35 and 36) is activated by familiarity, while the hippocampus and posterior parahippocampal cortex mediate recollection.⁴⁹ Perirhinal cortex stimulation evokes déjà vu and déjà vécu (already experienced).⁵⁰ Further, the right hemisphere dominates in familiarity decisions^14,48; déjà vu is more common with right than left temporal lobe seizures or stimulation.^48,51 Lesions that destroy or isolate stimuli from right perirhinal cortex may lead to loss of familiarity (e.g., Capgras syndrome) while hyperfamiliarity (i.e., misidentifying strange people as familiar [Fregoli syndrome]) may result from overactivity in right perirhinal cortex from stimulation or disinhibition. Two cases of nondelusional hyperfamiliarity for faces resulted from left-sided lesions (lateral temporal-occipital and anterior cingulate),^52,53 possibly disinhibiting right hemisphere areas that imbue faces or places with familiarity.

48. Gainotti G. Face familiarity feelings, the right temporal lobe and the possible underlying neural mechanisms. Brain Res Rev 2007;56:214–235.[Medline]
49. Bowles B, Crupi C, Mirsattari SM, et al. Impaired familiarity with preserved recollection after anterior temporal-lobe resection that spares the hippocampus. Proc Natl Acad Sci 2007;104:16382–16387.[Abstract/Free Full Text]
50. Vignal J-P, Maillard L, McGonigal A, Chauvel P. The dreamy state: hallucinations of autobiographic memory evoked by temporal lobe stimulations and seizures. Brain 2007;130:88–89.[Abstract/Free Full Text]
51. Mullan S, Penfield W. Illusions of comparative interpretation and emotion. Arch Neurol Psychiatry 1959;81:269–284.[Abstract/Free Full Text]
52. Vuilleumier P, Mohr C, Valenza N, Wetzel C, Landis T. Hyperfamiliarity for unknown faces after left lateral temporo-occipital venous infarction: a double dissociation with prosopagnosia. Brain 2003;126:889–907.[Abstract/Free Full Text]
53. Nente F, Carrillo-Mezo R, Mendez MF, Ramirez-Bermudez J. Pathological hyperfamiliarity for others from a left anterior cingulate lesion. J Neuropsychiatry Clin Neurosci 2007;19:345–346.[Free Full Text]

Hughlings Jackson and right hemisphere expression, and callosal syndromes

56. Jackson JH. Evolution and dissolution of the nervous system. In: Taylor J, ed. Selected Writings of John Hughlings Jackson, Volume 2. New York: Basic Books; 1884/1958:45–75.
57. Sperry RW, Zaidel E, Zaidel D. Self recognition and social awareness in the deconnected minor hemisphere. Neuropsychologia 1979;17:153–166.[Medline]
58. Gazzaniga MS. Cerebral specialization and interhemispheric communication. Brain 2000;123:1296–1326.
59. Gazzaniga MS. The split brain revisited. Sci Am 1998;279:50–55.[Medline]

ego boundaries response to next patient syndrome

55. Bogousslavsky J, Regli F. Response-to-next-patient-stimulation: a right hemisphere syndrome. Neurology 1988;38:1225–1227.[Abstract/Free Full Text]

Neuropsych and carotid stenosis

M. Silvestrini, MD, I. Paolino, MD, F. Vernieri, MD, C. Pedone, MD, R. Baruffaldi, MD, B. Gobbi, MD, C. Cagnetti, MD, L. Provinciali, MD and M. Bartolini, MD . Cerebral hemodynamics and cognitive performance in patients with asymptomatic carotid stenosis. NEUROLOGY 2009;72:1062-1068

Objective: The aim of this study was to investigate whether the presence of severe internal carotid artery stenosis may be associated with different cognitive performance in relation to the side of the stenosis and its hemodynamic consequences.

Methods: Eighty-three patients with asymptomatic severe unilateral internal carotid stenosis were included. A neuropsychological investigation including Verbal Fluency using phonemic and category access, Coloured Progressive Matrices, and Complex Figure Test Copy was performed. Each patient underwent an assessment of cerebrovascular reactivity (CVR) to hypercapnia with transcranial Doppler ultrasonography using the breath-holding index (BHI). Thirty healthy subjects comparable for demographic characteristics and vascular risk profile served as controls. Subjects with carotid stenosis were classified into two groups: preserved CVR (BHI 0.69), 48 patients (25 with left and 23 with right stenosis); and impaired CVR (BHI <0.69), 35 patients (19 with left and 16 with right stenosis).

Results: Subjects with left stenosis and reduced CVR had significantly lower performances at phonemic verbal fluency with respect to controls and the other groups of stenosis. In subjects with right stenosis and reduced CVR, scores obtained in Coloured Progressive Matrices and in Complex Figure Test Copy were significantly lower with respect to the other groups.

Conclusions: These results suggest that an alteration of cerebrovascular reactivity may be responsible for reduction in some cognitive abilities involving the function of the hemisphere ipsilateral to carotid stenosis. Such findings may be of interest for providing a more comprehensive indication to surgical treatment in subgroups of subjects with asymptomatic carotid stenosis.

Devinsky on localization of Capgras, Fregoli,anosognosia and related conditions

Neurology 2009 Views and Reviews

ABSTRACT When the delusional misidentification syndromes reduplicative paramnesia and Capgras syndromes result from neurologic disease, lesions are usually bifrontal and/or right hemispheric. The related disorders of confabulation and anosognosis share overlapping mechanisms and anatomic pathology. A dual mechanism is postulated for the delusional misidentification syndromes: negative effects from right hemisphere and frontal lobe dysfunction as well as positive effects from release (i.e., overactivity) of preserved left hemisphere areas. Negative effects of right hemisphere injury impair self-monitoring, ego boundaries, and attaching emotional valence and familiarity to stimuli. The unchecked left hemisphere unleashes a creative narrator from the monitoring of self, memory, and reality by the frontal and right hemisphere areas, leading to excessive and false explanations. Further, the left hemisphere's cognitive style of categorization, often into dual categories, leads it to invent a duplicate or impostor to resolve conflicting information. Delusions result from right hemisphere lesions. But it is the left hemisphere that is deluded.

(from text of article in Neurology)

Reduplicative paramnesia and Capgras syndrome cases with unilateral brain lesions strongly implicate the right hemisphere, usually frontal with variable temporal or parietal involvement.

^{Other points of interest}

Luria tests from Higher Cortical Functions in Man

Motor:
p.417 and on
1. copy hand postures- suggested to do with little visual input.
2. bimanual task-- closes patient eyes, places one hand in posture and asks patient to place opposite hand in same posture.
3. cites Head's test with mirror image imitation and imitation with same (ie left or right hand). Also, do imitation while touching left eye, right ear etc.
4. cites Ozeretskii (1930)- place one hand in fist, the other outstretched and alternate movement (premotor)reciprocal coordination
5. Place both hands in front, alternately tap twice with right hand, once with the left, with smooth transitions, and then switch the sides. Request the test to be done quickly.
6. Ozeretskii again- if one hand is paretic, do "fist ring" of other hand, thrust hand forward alternately making a fist, and a ring, testing premotor again.
7. Eidinova and Pravdina-Vinarskaya-- (1959)-- Start with a bent elbow, straight fingers, and ask patient to extend elbow, and at same time change hand position to a fist, rapidly. repeating. May eventually change the instruction to the opposite.
8. More difficult-- fist edge palm
9. Piano imitation-- ask patient on table to imitate playing piano with thumnb and first finger of one hand, and fingers 1-5 of other at same time. (and various combinations).
10. Doing above tasks with self spoken cues
11. writing alternating components (Russian equivalent of m and n)
12. Observation of everyday tasks (buttoning, lacing a shoe)
13. States that performance of imaginary acts such as pouring tea are sensitive but not equally susceptible to analytics.

Oral praxis p 425
1. Basic exam-- if patient can bare teeth once, ask him to sustain task for a long time.
2. touch tongue to upper lip, protrude it and keep it out for a while, looking for synkinesis, smoothness of movement
3. Stretch lips, roll up tongue, puff the cheeks and alternate these.
4. "Integrative tests" are chewing, spitting, kissing and whistling
5. Rid mouth of actual edible food which may be impossible even though above are preserved.

Selnes and Gordon: Quick office neuropsych testing

A few novel suggestions by these two:
Orientation: Add time of day to questions to increase sensitivity
Attention: Use forward-reverse digit span rather than serial sevens
Memory-- NCSE (AnnInt Med) 1987 uses 4 words and a recognition memory function is better than three words recall.
Language-- use brief screening test, progress to more difficult
Visuoconstruction-- draw a clock face or Necker cube is better than copy test of MMSE
Perceptualability-- tell time from clock face
Frontal lobe-- 3 hand test of Luria no suggestions
Psychomotor speed-- write alphabet as quickly as possible. Normal isless than 20 seconds, abnormal ismore than 30 seconds.

Clock Drawing Test notes on

The test incorporates a broad range of cognitive requirements making it an effective screening instrument. These include comprehension, planning, visuoconstruction abilities, motor programming, numerical knowledge, abstract thinking, and concentration.

There are several different tests that are used. One test, using a predrawn circle is standard for some, whereas others use a requirement for the subject to draw the circle. In typical case, subjects are given a circle and asked to place the hands at ten minutes past eleven.

In the Boston Diagnostic Aphasia battery, subjects are given four predrawn clocks, with ticks for the hours and asked to mark each clock to a different time: 1:00, 3:00. 9:15 and 11:30. One point is awarded for placing the hands in correct place, another for correct hand lengths, with three points per clock and maximum twelve points for entire test.

Shulman used a five point scale, with five points for a perfect clock, four for minor visuospatial errors, three for inaccurate representation of 10 minutes past eleven with visuospatial organization is well done, two for disorganized visuospatia representation such that telling ten minutes past eleven is immpossible, and one for inability to discern a clock.

Shulman cited roughly 85 % sensitivity and specificity, noting that the Clock Drawing Test adds frontal, visuospatial constructive ability to MMSE.

The CLOX test is a modified test used in CERAD. The subject is given a blank paper, and told "Draw me a clock and set the hands to 1:45 so that even a child could read them." That is CLOX 1. CLOX 2 the subject is given the clock and told to copy it. On Clox 1, subjects are graded as normal, mildly, moderately, or severely immpaired.

Tests can be used in a variety of settings that are not at first blush apparent, including screening patients undergoing hip replacement surgery for postop delirium, (better than MMSE)or subtyping dementia. Curiously, vascular dementia patients make more segmentation errors on clock test than do Alzheimer's patients.

Dysfluency pearls: Stuttering, cluttering & palilalia

1. Developmental stuttering usually is made better by singing, repetitive reading of same passage (adaptation) and playing white noice in patient's own ear to prevent him from hearing his own speech . There is no neurologic lesion or evaluatin required.

2. Acquired stuttering can occur with multiple location small brain lesions and does require MRI. Unlike developmental stutterers, acquired stutterers are dysfluent throughout a sentence, not just at the beginning, and are generally not bothered or embarassed by the deficiency.

3. Cluttering is characterized by rapid speech, repetitions, omissions, interjections and disturbed prosody. Sounds and syllables may be inverted or omitted. Clutterers may repeat initia sounds and prolong sounds within the word. Clutterers are not concerned about the problem and usually do not need neurologic evaluation.

4. Palilalia is characterized by compulsive repetition of words and phrases at increasing speed and with a decrescendo phonatory volume, and occurs in Parkinson's disease and pseudobulbar palsy. It improves considerably when the patient speaks with a metronome.

Akinetopsia in posterior cortical variant of Alzheimer's

Akinetopsia is inability to perceive motion (Zeki, Brain 1991). It occurs due to bilateral lesions at posterior parieto-occipital junction. (Also see Rizzo, Brain Res., 2008). Authors (Tsai and Mendez, Neurology 2009) report 2 patients with degenerative disease that reported double vision with vision as "a series of successive stills" from moving images. Neuropsych testing showed impaired constructions of figures (Rey-Ost.), impaired figure-ground, visual synthesis, search, depth discrimination Akinetopsia was elicited with vision to LEFT and not relieved with alternate eye cover/uncover. Laterality and preference for one lateralization corresponds with primate work.

Origin of delirium in DT's

Fisher CM.  Ethanol in treatment of AWS.  The Neurologist 2009; 15: 242-244.  In category "Notes from a Legend."

 

CM Fisher writes in the Neurologist that behaviorist Watson (1925) believed the terror feeling of having one's arms and legs restrained was a primitive inborn reflex and that was re-expressed when alcoholics were restrained and became agitated.

 

In the same article, Fisher writes about the utility of intravenous and oral alcohol being superior than benzodiazepines in some moderate social drinkers who are admitted and who suffer DT's

Laterality in catamential seizures

Quigg M et al. Laterality and location influence catamenial seizure expression in women with partial epilepsy.  Neurology 2009; 73: 223-227. 

 

Authors studied 100 women enrolled in a clinical trial of hormonal therapy for localization related epilepsy  (NIH progesterone trial study group).  Extratemporal  (10), and multifocal (14) seizures occurred randomly throught the lunar cycle.  There were 5 patients with unknown seizure origin, 25 with left temporal seizures, and 29 with right temporal seizures.  Patients with left temporal seizures peaked cyclically with onset of menses, whereas right temporal lobe seizures occurred randomly.

Cerebellum and Foreign Accent Syndrome

Cohen DA, Kurowski K, Steven MS, Blumstein SE, Pascual-Leone A. Paradoxical facilitation . The resolution of foreign accent syndrome after cerebellar stroke. Neurology 2009; 73:566-67.

Background: Lesions are usually left parietal. 2 previous cases were described with cerebellar hypoperfusion that resolved with FAS .

Case: 58 yo woman had left frontoparietal infarct with aphemia and right upper limb paresis. It resolved to right arm ataxia and English with an accent. 2 years later, after a right cerebellar hemorrhagic infarct, the FAS resolved. Her speech was analysed after each stroke. Abnormalities which were detected that comprised the FAS included pathologic prosody, vowel formant freqency, vowel durations, and increased variabilities on these vowel measures, and normal voicing and placement of stop consonants and preservation of the distinction between tense and lax vowels.

Alterations in timing and rhythm of speech, as seen in FAS, are characteristic of cerebellar lesions (see Ivry R., Cerebellar timing systems, Int Rev Neurobiol, 1997; 41: 555-573). The author speculates that the prosodic problems in FAS may " reflect deficits in timing resulting in impairments of speech melody, in maintaining the correct vocal posture for the production of vowels, in maintaining the correct durational patterns of speech, and in producing syllabic stress and quantity."

Re mechanism, the authors propose that the lesioned right cerebellum then gives way to the now dominant left cerebellar hemisphere, which is disinhibited and now functional.

Visual hallunications due to cardiac carcinoid

Mekuria S, Tan C, Schoenhagen P, et al. Shortness of breath and visual hallucinations. Am J Med 2009; 122: 338-341.

Patient had exertional dyspnea, night sweats, weight loss over six months. Week prior to admission, he developed visual hallucinations including ants crawling on wall and rabbits inside his room. ECHO showed a right heart cardiac tumor, eventually with an elevated urinary 5 HIAA. Brain imaging was negative. Authors postulated hallucinations were due to serotonin from tumor entering circulation, via a patent foramen ovale. Without a right to left shunt, the lung would have metabolized serotonin (adenosine deaminase). Octeotride resulted in less hallucinations, and effects were monitored with serotonin levels. Previous behavioral neurology of carcinoid syndrome includes behavior dysregulation (see Russo et al. Psychosoma Med 2003; 66:422-425).

Problems of neurolinguistics

In his conclusion, Luria notes that the new field of neurolinguistics presents some difficulties for the would-be practitioner because it requires a skillful level of proficiency in "neurological, psychophysiological, and linguistic knowledge" ( Luria, 1974a, p. 2591).

Semantics, Phonetics and Orienting reaction

With respect to their goal of defining the dynamics and structure of the semantic system, the authors come to two conclusions:
the words are divided into three groups: (1) the nucleus of the semantic complex, to which is related the key word and words in direct semantic proximity to it (they evoke a specific pain reaction); (2) the periphery of the semantic system to which are related words linked less directly with the key word (evoking a non-specific orienting reaction); and (3) neutral words, which in our experiment did not evoke any specific or orienting reactions.
The correlation of these groups at various stages of the experiments may be different, and if at the beginning of the experiment the nucleus of the semantic system has a relatively generalized character, then later it becomes concentrated and only the key word continues to provoke a specific reaction, while the remaining words, which earlier were included in this nucleus, move into the semantic periphery and begin to evoke only a non-specific orienting reaction. ( Luria & Vinogradova, 1959, pp. 99-100)

Traumatic Aphasia Scheme

While others might speak of aphasias in general, Luria presents his own organizational schema. First of all, he groups aphasias into three basic groups according to severity: (1) Total Aphasia is characterized by a total block of production and/or comprehension of speech. The disturbance is severe and lasts at least two or three weeks. (2) Well Expressed Aphasia includes those cases where the symptoms are relatively severe but do not involve a total block of speech activity. (3) Subtle, Slight Aphasia involves cases wherein the disturbance is not always evident at all times. It may appear most strongly only during instances of emotional disturbance or fatigue ( Luria, 1970b, pp. 34-35). Because initial trauma to the brain often results in a temporary disturbance of speech activity, Luria is emphatic that judgments with respect to symptoms and severity should be made only during the residual stage, which is the period 2 to 5 months after the trauma.
In addition to grouping by severity, Luria follows the classification schema that was encountered in earlier articles: acoustic aphasia, afferent motor aphasia, efferent motor aphasia, frontal dynamic aphasia, and semantic aphasia.
Of particular interest with respect to the focus of this book is a section wherein Luria, with his usual attention to philosophical bases of research, precedes his presentation of specific types of aphasia with a consideration of "The Structure of Speech Activity." In this brief section, he presents his explanation of when speech phylogenetically became intrinsically linked to thought:
The isolated words of which verbal speech consisted in its earliest stages of development were capable of reflecting separate signs or primitive concepts, but they could not express even elementary thoughts. The meaning of a word shifted depending upon the situation and was nonexistent outside certain situations. Whereas words possessed a nominative function from the beginning, the predicative function derived only from the concrete setting in which they were uttered. A decisive change occurred when speech went from consisting of individual words to consisting of elementary grammatical sentences, when instead of a single word there arose a pair or group of words related to one another, i.e., when the first "syntax" appeared.
The revolution which occurred at this phase in the development of language was truly phenomenal . . . . verbal speech became capable not only of designating an object, but also of formulating a thought. Verbal speech was still bound up with other forms of expression such as gesture and intonation, but with the development of written language thought came to be expressed altogether by means of language and speech became fully capable of performing the predicative function. Speech became an independent system of codes. ( Luria, 1970b, p. 83)
Because the development and structure of speech is so complex, Luria maintains that any consideration of its disturbance cannot be simplified and considered to be merely
disturbances or the speech images of words or . . . the inability to pronounce words. The basic forms of speech disturbances must result from defects in the systems of connections which are concealed behind the word on one hand and in the disintegration of the predicative function of speech on the other. The whole sense of verbal statements resides in this function. Similarly, the cerebral mechanisms which underlie speech processes cannot be at-

Speech and Orienting response

Frontal Lobes and the Regulation of Arousal Processes" ( 1970) was co-authored with E. D. Khomskaya, and appeared in Attention: Contemporary Theory and Analysis. The article reports on the results of studies conducted with normal subjects and brain lesion patients in an effort to ascertain the role of the frontal lobes in the regulation of arousal processes or the orienting reflex. The orienting reflex is
a complex functional system which includes a series of somatic, sensory, vegetative, electrocephalographic, and other components. While having a certain autonomy, all components of the orienting reflex obey common laws: they appear with the presentation of stimuli which are new for the organism; they have a nonspecific character, i.e., they do not depend on the modality of the stimulus; they disappear in proportion to repetition; and reappear with any changes in the experimental situation. Another no less important factor that elicits orienting reactions, is the significance or signal meaning of the stimuli. ( Luria & Khomskaya, 1970, pp. 303-304)
The authors demonstrate that, anatomically and neurologically, a number of connections exist between the frontal lobes and those structures of the limbic system and brain stem that are responsible for states of wakefulness, arousal, and the orienting reflex. The latter structures provide the efferent link in the system while the afferent link seems to be located at the cortical level.
The most significant finding of the experiments for our purposes is the fact that in humans one can regulate the orienting reflex by means of speech, which does physiologically modify the same:
If, by means of speech, stimuli are given signal meaning, a series of changes in the system of the orienting reflex occurs, namely: extinguished orienting reactions reappear and become more intensive (their latent period is shortened, their strength and duration is increased), they do not extinguish any more, and they arise for a wider range of intensities (as a result of the lowering of the threshold of sensation and the elevation of the threshold of the defense reaction). In this situation, the orienting reactions become more discriminating. They are not responsive to any outside nonsignal stimuli. Similar changes in the system of the orienting reflex occurring with the introduction of verbal instructions are observed in all normal subjects who are in an awake state (grownups and children, beginning from 8-10 years of age). ( Luria & Khomskaya, 1970, p. 304)
The authors indicate that this regulation of the orienting reflex by means of speech is such an essential characteristic of human mental functioning that it "may serve as an important indicator of the normally functioning brain" ( Luria & Khomskaya, 1970, p. 305). The balance of the article recounts their endeavors to test this hypothesis, and their conclusion


Reviewing his research findings on the role of the frontal lobes in the activation processes, verbally programmed behavior, and problem solving activity, Luria notes that "Each human activity starts from definite intention, directed at a definite goal, and is regulated by a definite program which demands that a constant state of cortical tone be maintained" ( Luria, 1973a, p. 5). It is his conclusion that the frontal lobes have vital roles in the state of activation that arises when the brain has some task to perform.
In addition, the frontal lobes are vital in the process whereby the orienting reflex is intensified and stabilized as a result of verbal instruction, so Luria concludes that they have a vital role in specifically human forms of attention.

Cole: implications of functional systems on lesions

The first is that the cerebral component of any functional cortical system results from the interaction of a constellation of cerebral areas. Thus, a functional system is localizable in the sense that damage to any one of the areas involved destroys the functional system. Defects will appear in the psychological activities dependent upon this functional cortical system. Second, a given psychological activity, for example, reading, may be performed in different ways, that is, by different functional cortical systems. Thus, a psychological activity is not localizable in the sense that if damage to a structure destroys a functional cortical system upon which reading is based another functional system cannot be developed which would carry out that same activity of reading. Third, the most important adaptive functions that man possesses, such as abstraction, computation, and speech itself, depend upon functional cortical systems which are acquired rather than innate. The fourth major assumption is that the most important determinant of functional cortical systems in man is the organization of the social environment. ( Cole & Maltzman, 1969, p. 278)

adynamic aphasia , inner speech and rehab

"The Mechanism of 'Dynamic Aphasia'" ( 1968) was co-authored with L. S. Tsvetkova, and postulates that dynamic aphasia is a disturbance of "inner speech with its predicative function, which takes place in forming the structure or scheme of a sentence, . . ." ( Luria & Tsvetkova, 1968, p. 297). The article reports on their attempts to test this by experiments with 15 patients with dynamic aphasia and 15 normal subjects. The aphasia patients exhibited more difficulty in naming actions than in naming objects, and could not form sentences out of all the separate words necessary to do so. Normal patients had no difficulty with either task. External cues (such as pieces of paper) assisted the subjects in compensating for this loss of the "linear scheme of the phrase . . ." ( Luria & Tsvetkova, 1968, p.
"Frontal Lobe Syndromes" ( Luria, 1969a) constitutes a chapter in the Handbook of Clinical Neurology, Vol. 2, edited by P. J. Vinken and G. W. Bruyn , and provides a comprehensive consideration of the morphology and functioning of the frontal lobes.
Luria provides such basic information as the fact that the frontal lobes are the youngest portion of the cerebral hemispheres evolutionarily; comprise approximately one third of the total human cerebral cortex; and consist of three main regions: the motor area, the premotor area, and the prefrontal area together with the mediobasal aspects of the frontal region. The complexity of this area of the brain is evidenced by the fact that Layer III of the neocortex in Area 6 of the premotor area contains approximately 207 million pyramidal cells. Because each cell may have 2-3,000 synapses, the reader can gain some appreciation of the multiplicity of connections with other areas of the brain of which the frontal lobes are capable.
The most complex zones of the frontal lobes do not complete their physical development until the individual is 7-12 years of age; and the integrative tertiary zones of cortical development are found in the prefrontal region.
A detailed analysis is presented of the morphological, physiological, and clinical data on each of the three regions of the frontal lobes. Of particular interest are the disturbances of higher mental processes and speech that result from lesions in these regions. These have been presented in the reviews of other articles on the frontal lobe syndrome, but this chapter presents the same in more detail and states:
Analysis of these disturbances shows that they are based on difficulty in performing complex movements generalized in time. There is difficulty in denervating one link and moving on smoothly to the next. This disturbance of "kinetic melodies" is the fundamental symptom of a premotor lesion . . . . ( Luria, 1969a, p. 731)
With respect to how frontal lobe lesions affect speech, Luria states that
If the lesion is situated in the inferior portions of the premotor area of the dominant (left) hemisphere, phenomena similar to the disturbances of kinetic melodies described above may also appear in speech and verbal thinking. The patients of this group begin to have difficulty in fluent speech, their speech becomes interrupted, and difficulties arise in the transition from one element of articulation to another. Similar phenomena of the loss of smoothness (and sometimes of perseveration) may also appear in writing. ( Luria, 1969a, p. 733)
If the symptoms are severe, one has a case of efferent (or kinetic) motor aphasia.
The symptoms of frontal lobe lesions vary somewhat, of course, depending upon the location of the lesion; however, Luria indicates that there are two main symptoms that occur
in almost every massive lesion of the prefrontal regions. The first of these symptoms was a disturbance of the complex forms of active purposive behavior, and the second a disturbance of the critical attitude towards the patient's own defects. Both of these components of the "frontal syndrome" were observed as a rule in all massive lesions of the frontal lobes, although they varied in lesions of the convex and basal portions of the frontal region, and differed depending on the severity of the lesion . . . . ( Luria, 1969a, p. 738)
When such characteristics arise from a lesion of the left prefrontal region, they are manifested in speech activity and verbal thinking and distinguish the aphasia Luria labels dynamic aphasia.
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Vocate: more on inner speech

External speech includes the spoken language first of others in the child's social environment and thus is the stage that includes the initial sociocultural origins of spoken language. The child's external speech is initially imitative and then evolves into egocentric speech, which, according to Luria, serves a special function in aiding the child in organizing his behavior, and is characterized by its "coding for self" nature. Such speech is gradually internalized, and becomes internal or inner speech. However, in the process of internalization, it is modified somewhat so that its most distinctive feature becomes its predicative nature and it becomes characterized by its ellipsis, synthesis of meaning, and silence. A. R. Luria presumes a familiarity by his reader with these basic developmental stages,

Vocate: written and inner speech

the functional and structural features of written speech . . . have . . . one important aspect; they inevitably lead to a considerable development of inner speech. Delaying the direct revelation of speech connections, inhibiting them and showing increased requirements for the preliminary speech act not being revealed at once by training, written speech produces such a rich development of inner speech as could not have a place in the earlier phases of development. Therefore, neuropathologists are not working at random when, desiring to investigate the possibilities of inner speech, they turn to the nature of the written speech of their patients. ( Luria, 1961d, p. 738)
De

Vocate Luria on consciousness

"'Brain and Conscious Experience'": A Critical Notice from the U.S.S.R. of the Symposium Edited by J. C. Eccles ( 1966)" ( 1967a) is Luria's review for the British Journal of Psychology of the transcript of an international symposium on brain/consciousness called by the Vatican Academy of Sciences and organized by its President, Sir John Eccles.
Luria chides the majority of the prominent participants for their archaic philosophical orientations to the brain/consciousness question, and notes that
In spite of the fact that the definitions of consciousness given by the participants were varied, not one of them understood consciousness as the reflexion of objective reality, as "conscious being" or as complex activity which has a semantic and systematic structure. ( Luria, 1967a, p. 469)
Rather, according to Luria, the participants were roughly divided into two groups: one group, exemplified by Penfield, sought the material basis for consciousness inside the brain and anticipated discovery of the formations in neuronal structure that give rise to this phenomenon; the other group, typified by MacKay, rejected any study of neuronal structure and called for the study of logical systems concerned with the processing of information, which are somehow the basis for conscious experience.
After reviewing the new directions in brain research represented at the symposium, Luria goes on to note that the central problem of the conference was "the question of the role played by the non-specific system of the brain stem in providing an active and waking state for the cerebral hemispheres . . . ." ( Luria, 1967a, p. 471). This problem arises from the knowledge that the non-specific reticular system of the brain stem interacts with the specific or cortical formations of the brain--stimulating and receiving stimulation.

Vocate: More on PD rehab

The dominance of the cortical level over the sub-cortical level is adduced by Luria's research with Parkinson's disease patients ( 1959b, 1960b, 1961a, 1967d), which demonstrated that it is possible to transfer the control of defective involuntary (sub-cortical) motor behaviors to the voluntary (cortical) motor areas so that the subject can still perform the desired action because the pathology has left the cortical areas intact.
In Parkinson's disease patients:
The injured subcortical apparatus excites repeated tonic responses, and the pathologically perseverating tension of all muscles is an obstacle to the execution of the instruction. It is easy to imagine such a difficulty in carrying out a voluntary movement if one briefly tenses all the muscles of one hand and then tries to move it without relaxing the tension. ( Luria, 1959b, p. 455)
However, if the origin of the motor act is shifted from the automatic movement realm governed by the sub-cortical motor apparatus to the conscious movement domain of the cortical motor areas, the patient is able to carry out the required movements. This may be accomplished by
attaching a symbolic function to his movements. He is asked to reply to the experimenter's questions by beating out the necessary numbers with his finger. If we then ask him, "How many wheels on a car?" or "How many points on a compass?" we see that the same patient who had failed in the previous experiment and could not automatically strike the table with his fingers even two or three times, easily begins to do so, switching his movements into his speech system and subordinating them to the complex dynamic constellation of cortical connections. ( Luria, 1959b, p. 455)
Similarly, the Parkinson's disease patient who is unable to walk more than one or two steps will find that his difficulties
in successive automatic movements may be compensated for temporarily if they are transferred to the cortical level, and if the continuous movement is superseded by a cycle of isolated responses to individual stimuli. Such a patient cannot take several steps on a smooth floor but can easily cross several lines marked on the floor or several objects placed on the floor. ( Luria, 1967d, p. 417)
Such mechanisms force the control of the normally automatic components of walking to the conscious, voluntary control of the cortical level by separating them into individual responses to individual stimuli, thus requiring cortical level processing.

Vocate: What language means (Luria)

The function of generalisation is the main function of human speech, without which mastery of the experience of preceding generations would be impossible. But it would be wrong to think that this is the only basic function of speech. Language is not only a means of generalisation; it is at the same time the source of thought.
When the child masters language he gains the potentiality to organise anew his perception, his memory; he masters more complex forms of reflection of objects in the external world; he gains the capacity to draw conclusions from his observations, to make deductions, the potentiality of thinking.
When the child names something, pronouncing, for example, "that is a steam engine," he is at the same time analysing with the aid of means developed through many generations . . . . Saying the word "steam engine" (paravoz) he begins to understand that in the movement of the machine named steam (par) plays a role and that it moves other objects. In mastering words and using them the child analyses and synthesises the phenomena of the external world, using not only his personal experience but the experience of mankind. He classifies objects, he begins to perceive them differently and with this to remember them differently.
But the speech mastered by the child does not consist of single words; it consists of complex grammatical combinations, of whole expressions. These expressions allow not only for the analysis and synthesis of perception, but also the connection of things with actions, and still more the posing of things in certain relations with each other. Acquiring forms of developed, connected, speech the child acquires the potentiality not only to form concepts but also to draw conclusions from accepted assumptions, to master logical connections, to cognise laws, far surpassing the boundaries of direct, personal experience; in sum, he masters science, gains the potentiality to foresee and foretell phenomena, which he could not do by merely witnessing them.
What has been said up to now does not fully cover the role of language, the role of speech, in the formation of man's mental processes. Speech activity besides being a means of generalising and the source of thought is also a means of regulating behaviour. ( Luria, 1963c, pp. 85-86)