Daniel L. Menkes

Amusia after right frontal resection for epilepsy with singing seizures: case report and review of the literature.

Although many authors consider aprosodia and amusia to be synonymous, they actually represent two distinct communication disorders. Amusia refers to a profound deficit involving musical abilities, whereas aprosodia refers to deficits regarding the emotional content of speech. Many authors have presumed a similar etiology and localization for these conditions and assumed that these disorders would not occur independently. We report the case of a 31-year-old choir director who developed amusia without aprosodia after a right frontal lobe resection for intractable seizures. His ictal onset manifested with rhythmic slapping of his thighs while communicating with melodic speech. Video EEG monitoring documented right hemispheric discharges that occurred simultaneously with this ictal behavior. While a right frontal lobe resection made him seizure-free, his postoperative amusia was so profound that he could no longer continue his occupation as a choir director. This case suggests that the right frontal cortex has different sites for musical ability distinct from the centers regarding prosody. Patients scheduled to undergo right frontal lobectomy ought to be counseled regarding the potential loss of musical abilities.

Murine central and peripheral nervous system transcriptomes: comparative gene expression.

The central and peripheral nervous systems exhibit significant embryological, morphological, and functional differences. Moreover, the pathology of most acquired and hereditary neurological diseases preferentially targets specific components of the nervous system. In order to test the hypothesis that central and peripheral neural transcriptomes show fundamental quantitative differences, Affymetrix GeneChip expression arrays were used to compare murine lumbar spinal cord (SC) and dorsal root ganglion (DRG) gene expression. As the crucial component of a novel technique to preserve RNA integrity, mice were perfusion-fixed with RNAlater before the SC and DRG were harvested. As per Affymetrix terminology, a total of 111 transcripts were present (P) on all DRG arrays, absent (A) on all SC arrays, and demonstrated at least 10-fold greater expression in DRG than in SC. Conversely, a total of 112 transcripts were present on all SC arrays, absent on all DRG arrays, and showed at least 10-fold greater expression in SC than in DRG. For a subset of transcripts, quantitative real-time RT-PCR was used to corroborate and validate microarray results. Among those genes enriched in DRG, many belonged to a few distinct functional classes: G-protein coupled receptor-protein signaling pathways, potassium transport, sodium transport, sensory perception, and cell-surface receptor-linked signal transduction. In contrast, genes associated with synaptic transmission, organic acid transport, neurotransmitter transport, and circulation were enriched in SC. Notably, the majority of genes causally associated with hereditary neuropathies were highly enriched in DRG. These differential neural gene expression profiles provide a robust framework for future molecular and genetic studies of neuropathy and SC diseases.

Comorbid HIV and myasthenia gravis: case report and review of the literature.

A 27-year-old woman without known risk factors contracted HIV and was given highly active antiretroviral therapy in 2006. She subsequently developed myasthenia gravis (MG) that responded to treatment with pyridostigmine bromide and azathioprine. The medical literature, consisting primarily of case reports, indicates that MG occurs in relatively immunocompetent HIV-infected persons who generally present with mild MG symptoms. As such, we recommend a high index of suspicion for MG in HIV-infected patients presenting with fatigue and weakness, especially those receiving highly active antiretroviral therapy.

Structured Didactic Teaching Sessions Improve Medical Student Neurology Clerkship Test Scores: A Pilot Study

OBJECTIVE: To determine the effectiveness of didactic case-based instruction methodology to improve medical student comprehension of common neurological illnesses and neurological emergencies. SETTING: Neurology department, academic university. PARTICIPANTS: 415 third and fourth year medical students performing a required four week neurology clerkship. MAIN OUTCOME MEASURES: Raw test scores on a 1 hour, 50-item clinical vignette based examination and open-ended questions in a post-clerkship feedback session. RESULTS: There was a statistically significant improvement in overall test scores (p<0.001). CONCLUSION: Didactic teaching sessions have a significant positive impact on neurology student clerkship test score performance and perception of their educational experience. Confirmation of these results across multiple specialties in a multi-center trial is warranted.

Pregnancy-related Increases in Sensory Perception Thresholds Are Not Correlated with Serum Progesterone Levels

Background: Why pregnant women require smaller doses of anesthetic agents still remains speculative. One hypothesis proposes that pregnancy raises sensory perception thresholds, perhaps through a progesterone‐mediated effect. This study was undertaken in order to quantify any changes in sensory perception thresholds after parturition and to correlate these changes with the expected decrease in postpartum serum progesterone levels.