recording-horizontal-saccade-performances-accurately-neurological

Recording Horizontal Saccade Performances Accurately in Neurological Patients Using Electro-oculogram

Electro-oculogram (EOG) has been widely used for clinical eye movement recording, especially horizontal saccades, although the video-oculography (VOG) has ...

Kyorin University

This report describes a rare case of hypertrophic pachymeningitis secondary to antiphospholipid syndrome in a 37-year-old woman. The patient had a history of antiphospholipid syndrome and developed hypertrophic pachymeningitis subsequently. Pathological examination of the dura mater showed not only fibrous thickening, the typical finding of hypertrophic pachymeningitis, but also thrombosis in the dural microvessels and T cell infiltration without B cell or plasma cell infiltration, suggesting cell-mediated immunity. The dural thickening spontaneously improved and did not deteriorate during corticosteroid therapy. The histological findings and spontaneous remission observed in this case might be characteristic of secondary hypertrophic pachymeningitis with antiphospholipid syndrome. Further investigations are necessary to elucidate the pathophysiology of this novel type of hypertrophic pachymeningitis.

Secondary hypertrophic pachymeningitis in antiphospholipid syndrome.

This report describes a 60-year-old female patient with Krabbe disease who presented with slowly progressive gait disturbance due to mild spastic paraplegia. Brain magnetic resonance imaging showed high-intensity lesions along the upper parts of the bilateral pyramidal tracts in fluid-attenuated inversion recovery images. Central motor conduction time was prolonged both in the upper and the lower extremities, while central sensory conduction time was normal. The reduced lymphocyte galactocerebrosidase (GALC) activity and two novel mutations in the GALC gene, p.G496S and p.G569S, proved the diagnosis of Krabbe disease. Our findings show that adult-onset Krabbe disease is characterized by isolated pyramidal tract impairment in the central nervous system, both neurophysiologically and radiologically.

Isolated pyramidal tract impairment in the central nervous system of adult-onset Krabbe disease with novel mutations in the GALC gene.

To delineate molecular and clinical characteristics of 3 families with PRNP P105L mutation, a variant of Gerstmann-Sträussler-Scheinker syndrome whose main motor symptoms were parkinsonism and/or involuntary movements.

Atypical parkinsonism caused by Pro105Leu mutation of prion protein: A broad clinical spectrum.

Patients with spinocerebellar ataxia with pure cerebellar presentation (SCD) and multiple system atrophy (MSA-C) show similar symptoms at early stages, although cerebellofugal pathology predominates in SCD, and cerebellopetal pathology in MSA-C. We studied whether saccade velocity profiles, which reflect the accelerating and braking functions of the cerebellum, can differentiate these two disorders.

Distinguishing spinocerebellar ataxia with pure cerebellar manifestation from multiple system atrophy (MSA-C) through saccade profiles.

Saccades abnormalities in posterior cortical atrophy - A case report.

Responses to continuous theta burst stimulation (cTBS) applied to the human primary motor cortex are highly variable between individuals. However, little is known about how to improve the after-effects of cTBS by adjusting the protocol characteristics.

The intensity of continuous theta burst stimulation, but not the waveform used to elicit motor evoked potentials, influences its outcome in the human motor cortex.

Article	Year
Secondary hypertrophic pachymeningitis in antiphospholipid syndrome. Journal of neuroimmunology\| PubMed ID: 22648062	2012
Isolated pyramidal tract impairment in the central nervous system of adult-onset Krabbe disease with novel mutations in the GALC gene. Brain & development\| PubMed ID: 22959700	2013
Atypical parkinsonism caused by Pro105Leu mutation of prion protein: A broad clinical spectrum. Neurology. Genetics\| PubMed ID: 27066585	2016
Distinguishing spinocerebellar ataxia with pure cerebellar manifestation from multiple system atrophy (MSA-C) through saccade profiles. Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology\| PubMed ID: 27866117	2017
Saccades abnormalities in posterior cortical atrophy - A case report. Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology\| PubMed ID: 28063320	2017
The intensity of continuous theta burst stimulation, but not the waveform used to elicit motor evoked potentials, influences its outcome in the human motor cortex. Brain stimulation\| PubMed ID: 29258807	2018

Shin-ichi Tokushige

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