A Case of Chronic Inflammatory Demyelinating Polyneuropathy in a 13-year-old Girl with Pes Cavus Deformity

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated polyneuropathy with a relaps-ing or progressive course. Although the occurrence of CIDP is very rare in childhood compared to the occurrence in adulthood, it does occur in children. In childhood CIDP, the main clinical features are somewhat different from that of adults, which includes a more precipitous onset of symptoms, a high frequency of gait abnormalities, and significant neurological dysfunction. Also, the response to immune-modulating therapy is more reliable and rapid in children than in adults. The recognition of childhood CIDP is important as it may mimic hereditary neuropathies which usually pre-sent with progressive gait disturbance associated with pes cavus deformity. A careful history, clinical examination, and electrophysiological study is crucial for the accurate diagnosis of this treatable disease. Here, we report a case of CIDP in a 13-year-old Korean girl whose initial clinical presentation strongly suggested hereditary motor-sensory neuropathy.

Familial Occurrence of Moyamoya Disease: Report of Two Families

BACKGROUND: Whether the changes of risk factors (hypertension, diabetes mellitus, smoking, alcohol) can precipitate stroke remains unknown, and antecedent infection and psychologic stress are described inss case-control study, 113 consecutive patients with acute cerebrovascular disease (38 small vessel disease, 43 large vessel disease, 11 cardiogenic infarction, 4 infarction of undetermined cause, and 17 intracerebral hemorrhage) and 23 control subjects were evaluated. Changes of the risk factors (and their management) were interviewed. A sign/symptom based questionnaire was used to characterize the prevalence of recent prior infection and exposure to coldness. Psychologic stress was measured with the use of Social Readjustment Rating Scale. RESULTS: The negative change of alcohol drinking was significantly higher in the stroke group. However, there was no significant difference between stroke and control groups in the changes of thether risk factors. The prevalence of previous (within 1 month) infection was significantly higher in the stroke group compared with control subjects (p=0.03). However, there were no significant differences among the stroke subtypes in the prevalence of infection(p=0.08). Upper respiratory tract infections constituted the most common type of infection. The exposure to coldness was significantly higher in the stroke group compared with control subjects (p=0.002). The level of stress within the prior 1 month/1 year was significantly higher in the stroke group than control group (p=0.01). CONCLUSION: Our data suggest that preceding infection, exposure to coldness, psychologic stress, and the negative change of alcohol drinking may be comm.