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.

One Case Report of Creutzfeldt-Jakob Disease

The original article was reported by Creutzfeld in 1920, and Jakob in 1921 respectively. CJD is an encephalopathy with worldwide distribution caused by an unidentified unconventional virus. The principal clinical features of CJD are dementia, upper and in some instances, lower motor neuron disorders; basal ganglionic and cerebellar dysfunction; and myoclonus including a heightened startle reaction. The disease is rapidly progressive, leading in several months to stupor, coma, and death. Pathologic changes are widespread, involving the cerebral cortex, basal ganglia, cerebellum, pyramidal tracts, and lower motor neruons of the brain stem and/or spinal cord. This case was 51 years old Korean male, who presented progressive dementia, myoclonus, heightened startle reaction, pyramidal, extrapyramidal symptoms, decerebrated rigidity, and died about 4 months after onset. We diagnosed this case as CJD with typical serial EEG pattern and clinical feature.