Conduction block in acute motor axonal neuropathy.

Guillain-Barré syndrome is divided into two major subtypes, acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy. The characteristic electrophysiological features of acute motor axonal neuropathy are reduced amplitude or absence of distal compound muscle action potentials indicating axonal degeneration. In contrast, autopsy study results show early nodal changes in acute motor axonal neuropathy that may produce motor nerve conduction block. Because the presence of conduction block in acute motor axonal neuropathy has yet to be fully recognized, we reviewed how often conduction block occurred and how frequently it either reversed or was followed by axonal degeneration. Based on Ho's criteria, acute motor axonal neuropathy was electrodiagnosed in 18 patients, and repeated motor nerve conduction studies were carried out on their median and ulnar nerves. Forearm segments of these nerves and the across-elbow segments of the ulnar nerve were examined to evaluate conduction block based on the consensus criteria of the American Association of Electrodiagnostic Medicine. Twelve (67%) of the 18 patients with acute motor axonal neuropathy had definite (n=7) or probable (n=5) conduction blocks. Definite conduction block was detected for one patient (6%) in the forearm segments of both nerves and probable conduction block was detected for five patients (28%). Definite conduction block was present across the elbow segment of the ulnar nerve in seven patients (39%) and probable conduction block in two patients (11%). Conduction block was reversible in seven of 12 patients and was followed by axonal degeneration in six. All conduction blocks had disappeared or begun to resolve within three weeks with no electrophysiological evidence of remyelination. One patient showed both reversible conduction block and conduction block followed by axonal degeneration. Clinical features and anti-ganglioside antibody profiles were similar in the patients with (n=12) and without (n=6) conduction block as well as in those with (n=7) and without (n=5) reversible conduction block, indicating that both conditions form a continuum; a pathophysiological spectrum ranging from reversible conduction failure to axonal degeneration, possibly mediated by antibody attack on gangliosides at the axolemma of the nodes of Ranvier, indicating that reversible conduction block and conduction block followed by axonal degeneration and axonal degeneration without conduction block constitute continuous electrophysiological conditions in acute motor axonal neuropathy.

Correlation between severity of obstructive sleep apnea and prevalence of silent cerebrovascular lesions.

STUDY OBJECTIVES: We investigated the prevalence of silent cerebrovascular lesions in patients with obstructive sleep apnea (OSA) and the correlation between OSA severity and prevalence of silent cerebrovascular lesions in Japanese patients. METHODS: Study subjects were 192 polysomnography (PSG)-confirmed patients who visited the sleep disorders clinic in our university hospital. None had a history of cerebrovascular disease (CVD). We performed a cross-sectional study on OSA severity and the prevalence of silent cerebrovascular lesions detected by brain MRI analysis. RESULTS: The control (AHI < 5/h) group included 19 subjects with a mean AHI of 1.7 +/- 1.6/h, the mild OSAS (AHI 5 to < 15/h) group included 25 patients with a mean AHI of 9.5 +/- 3.7/h, the moderate OSAS (AHI 15 to < 30/h) group included 35 patients with a mean AHI of 22.0 +/- 7.0/h while the severe OSAS (AHI > or = 30/h) group included 113 patients with a mean AHI of 59.9 +/- 20.5/h. A larger percentage of patients with severe OSAS had a higher BMI and hyperglycemia than those with mild or moderate OSAS and control subjects (p < 0.05). Silent lacunar infarction was identified in 4 (21.1%) control subjects, 3 (12.0%) patients with mild OSA, 17 (48.6%) with moderate OSA and 61 (54.0%) with severe OSA. Among control subjects and the mild, moderate, and severe OSA groups, 4 (21.1%), 5 (20.0%), 19 (54.3%) and 61(54.0%), respectively, had periventricular hyperintensity (PVH); most PVH was mild to moderate. CONCLUSION: Results indicate that patients with moderate to severe (AHI > or = 15/h) OSA have a higher prevalence of silent cerebrovascular lesion than those with less severe OSA.

123I-MIBG cardiac scintigraphy provides clues to the underlying neurodegenerative disorder in idiopathic REM sleep behavior disorder.

OBJECTIVE: RBD is considered to be a manifestation of an evolving synucleinopathy, such as Parkinson disease (PD), dementia of Lewy bodies (DLB), and multiple system atrophy (MSA). We tested whether the degree of accumulation of cardiac 123I-MIBG scintigraphy can distinguish the clinical syndromes associated with Lewy body-related disease from the syndrome of PSP (a tauopathy) and MSA. DESIGN: Cross-sectional. SETTING: University-based sleep disorders laboratory. PATIENTS: Subjects comprised 95 patients (31, idiopathic RBD; 26, PD; 10, MSA; 6, DLB; 13, progressive supranuclear palsy [PSP]) and 9 control subjects. INTERVENTION: To compare tracer uptake of cardiac 123I-MIBG between idiopathic RBD, PD, MSA, DLB, and PSP and control subjects. MEASUREMENTS AND RESULTS: Cardiac 123I-MIBG accumulation was evaluated by the heart/mediastinum (H/M) ratio. Mean value of the H/M ratio (early, delayed) was significantly reduced in patients with idiopathic RBD compared to MSA patients, PSP patients, control subjects (P < 0.001 in each group) and PD patients in early images (P < 0.05). There was a correlation between the H/M ratio and disease duration in the idiopathic RBD group. ROC analysis revealed that an H/M cut-off value of 1.9 was useful for differentiating RBD from MSA and PSP as well as distinguishing control subjects from those with RBD in both early and delayed images. CONCLUSION: Cardiac 123I-MIBG findings are similar among idiopathic RBD and the syndromes of PD and DLB, but differ from those of PSP and MSA.

[Distal hereditary motor neuropathy type II with mutation in heat shock protein 27 gene. A case report].

A 48-year-old man was admitted to our hospital with a tendency to stumble during walking. The family history indicated that the father was diagnosed with Charcot-Marie-Tooth disease (CMT) at the age of 55 and his younger sister (aunt) had similar symptoms that were considered to reflect autosomal dominant inheritance. Examination showed no pes cavus or inverted champagne-bottle thighs. In addition, the patient walked with foot drop due to weakness and atrophy of the distal parts of the lower extremities. Sensory examination revealed no deficits or abnormalities. Nerve conduction study and needle electromyography indicated pure motor axonal neuropathy. The diagnosis of distal hereditary motor neuropathy (distal HMN) type II was made. Genetic analysis detected mutation in the heat shock protein 27 (HSP27) gene. A recent report indicated that mutations in the HSP27 gene cause both distal hereditary motor neuropathy and CMT2F. In Japan, there are only a few reports of distal hereditary motor neuropathy with mutation in the HSP27 gene. Distal HMN should be considered in the differential diagnosis of patients with CMT like distal amyotrophy.

[Progressive multifocal leukoencephalopathy with idiopathic CD4 positive T-lymphocytepenia mimicking a low grade glioma on proton MR spectroscopy. A case report].

A 61-year-old man with no history of HIV infection developed a subacutely progressive dementia and left hemiparesis. Brain MRI showed a high intensity lesion in the right frontal lobe on T2 weighted image. There was no contrast enhancement after gadolinium-DTPA administration. 1H MRS revealed a marked decrease in the n-acetyl aspartate/creatine ratios and an increase in the choline/creatine ratio. A lactate peak also was present. A low-grade glioma was suspected and he was admitted to our hospital. On examination, there was a mild dementia and left hemiparesis. A peripheral blood count revealed lymphocytopenia (426/mm3) with a CD4/CD8 ratio of 0.28. No evidence of HIV infection, malignancies or collagen disease was found. A brain biopsy revealed no tumor cells but instead demyelinated brain tissue with large nucleated cells. JC virus antigen was detected in the cells of the demyelinated lesions. A diagnosis of PML associated with idiopathic CD4 positive lymphocytopenia was made. There are only a few reports concerning 1H-MRS findings in patients with PML and the present case illustrates the difficulty of making a differential diagnosis between PML and glioma.