The Relationship between Subclinical Asperger's Syndrome and Frontotemporal Lobar Degeneration

Background/Aims: The existence of the behavioral variant of frontotemporal dementia (bv-FTD), including senile Asperger’s syndrome (AS), has been proposed. However, there are no empirical case reports to support the proposal. In this report, we present 3 patients who showed symptoms of bv-FTD and demonstrated signs of autistic spectrum disorder, especially AS. Methods: We evaluated 3 subjects using the diagnostic criteria for bv-FTD, and their caregivers retrospectively provided data to calculate the Autism-Spectrum Quotient, Japanese version [Wakabayashi et al.: Shinrigaku Kenkyu 2004;75:78–84]. We also compared these data with those obtained from 3 individuals with Alzheimer’s disease. Results: All 3 patients met the criteria for bv-FTD and had a higher Autism-Spectrum Quotient score than did comparable Alzheimer’s disease subjects. Conclusion: It is possible that some senile persons with frontotemporal lobar degeneration-like maladaptive behavior may suffer from subclinical AS

Spin and chirality orderings of the one-dimensional Heisenberg spin glass with the long-range power-law interaction

The ordering of the one-dimensional Heisenberg spin glass interacting via the long-range power-law interaction is studied by Monte Carlo simulations. Particular attention is paid to the possible occurrence of the ``spin-chirality decoupling'' for appropriate values of the power-law exponent \sigma. Our result suggests that, for intermediate values of $\sigma$, the chiral-glass order occurs at finite temperatures while the standard spin-glass order occurs only at zero temperature.Comment: Proceedings of the Highly Frustrated Magnetism (HFM2006) conference. To appear in a special issue of J. Phys. Condens. Matte

Parietal Ataxia: 13 Cases Plus a Review of Relevant Literature

Parietal ataxia is a rare condition of hemi- or mono-ataxia caused by contralateral lesions in the parietal lobe. Parietal ataxia is categorized into sensory ataxia and pseudocerebellar ataxia, and is usually reported in single-case studies. Here we report 13 cases of hemi- or mono-ataxia caused by acute brain infarction in the parietal lobe, and discuss these cases with respect to past case studies and the MRI lesion visualizations. Patients with motor paresis were excluded from this study. Patients with sensory disturbance were categorized into a sensory ataxia group and pseudocerebellar ataxia group. Lesions on cranial MRI were classified as white matter inferior to the posterior central gyrus, posterior central gyrus, or superior or inferior parietal lobules. Six patients in the sensory ataxia group showed lesions in the gray or white matter of the posterior central gyrus. We found that parietal ataxia patients showed a high rate of decomposition and slowness in the finger-nose test and decomposition in diadochokinesis. Oscillation in the finger-nose test and slowness in diadochokinesis were more frequently observed in the patients with sensory ataxia than in those with pseudocerebellar ataxia, although the difference was not lesion dependent. This study thus found clinical differences between the sensory ataxia and pseudocerebellar ataxia groups of patients with parietal ataxia

Unilateral Abducens Nerve Palsy as an Early Feature of Multiple Mononeuropathy Associated with Anti-GQ1b Antibody

Patients with anti-GQ1b antibody syndrome show various combinations of ophthalmoplegia, ataxia, areflexia, or altered sensorium as clinical features. We describe herein a unique case with unilateral abducens nerve palsy as an early feature of multiple mononeuropathy involving dysfunctions of the inferior dental plexus and the ulnar nerve, which was thought to be associated with anti-GQ1b antibody. A 27-year-old man presented with acute-onset diplopia. He subsequently experienced numbness not only in the right lower teeth and gums but also on the ulnar side of the left hand. Neurological examinations revealed dysfunctions of the right abducens nerve, the right inferior dental plexus, and the left ulnar nerve, suggesting multiple mononeuropathy. Serum anti-GQ1b antibody was positive. This is a rare case report of a patient with unilateral abducens nerve palsy as an early feature of multiple mononeuropathy associated with anti-GQ1b antibody. We suggest that anti-GQ1b antibody syndrome should be taken into consideration as a differential diagnosis of acute multiple mononeuropathy if ophthalmoplegia is present unilaterally

Diagonistic Apraxia: A Unique Case of Corpus Callosal Disconnection Syndrome and Neuromyelitis Optica Spectrum Disorder

Diagonistic apraxia is a corpus callosal disconnection syndrome. Callosal lesions in Neuromyelitis optica spectrum disorder (NMOSD) have been reported, but callosal disconnection syndrome are rare. A 48-year-old woman was treated for fever and a cough before hospitalization. Her fever abated immediately, but she had balance problems in walking and standing. She also had slurred speech. On neurological examination, she had diagonistic apraxia. Her left hand moved in an uncoordinated way when she moved her right hand: changing her clothes for example or using a knife and fork. She had to instruct her left hand to stop. She had dysarthria and her gait was wide-based. She also had many callosal disconnection syndrome symptoms such as alexia of left visual field, left ear extinction, crossed optic ataxia. Using FLAIR and DWI MRI, a mixture of low and high signals, a so-called “marbled pattern,” was seen in the corpus callosum. Since the patient was positive for anti-aquaporin-4 antibody, she was diagnosed with NMOSD. After two courses of steroid pulse therapy, the symptoms improved. Here we report diagonistic apraxia and other symptoms of callosal disconnection syndrome in anti-AQP4-positive NMOSD

Is this a new type of primary prosopagnosia, both progressive and apperceptive?

Prosopagnosia, the inability to recognize faces, has a history going back to Charcot and Hughlings-Jackson, but was first named by Bodamer in 1947. Its anatomical loci are still unclear. However, progressive prosopagnosia is normally linked to right dominant temporal lobe atrophy, and diagnosed as part of frontotemporal lobar degeneration. Here we report a case of prosopagnosia linked to posterior cortical atrophy. Although case reports of posterior cortical atrophy-prosopagnosia do already exist, it is normally described as an accessory symptom. The interest of our own posterior cortical atrophy patient, possibly the first such case, is that he had a rare apperceptive type of prosopagnosia unrelated to the associative, frontotemporal lobar degeneration-type