Sunday, June 13, 2010

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. 

Tuesday, June 8, 2010

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. 

Sunday, June 6, 2010

Scales ADHD

* Conners parent-teacher rating scale
*ADHD Rating Scale
* SNAP IV Parent Teacher Rating (
* *** (best)Vanderbilt Assessment Scale (Parents and teachers) ( this is easy to get online and is free
In adults get
*adult ADHD report scale available at
*Conner's Adult ADHD Rating scales
*Adult ADHD rating scale IV
*Wender Utah Rating Scale
*Barfkley's Current Symptoms Scale

Thursday, June 3, 2010

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.