Cortical reflex negative myoclonus.

Three patients with progressive myoclonic epilepsy (PME), two of them clinically manifesting only negative myoclonus and the other manifesting both positive and negative myoclonus, were electrophysiologically investigated, and compared with two other patients with PME presenting with only positive myoclonus. Electric stimulation of the median nerve during sustained active wrist extension in the three patients with negative myoclonus often elicited a short lapse of the posture in the stimulated hand associated with a silent period in the muscle discharge with or without being preceded by an abrupt increase in the muscle discharge (C reflex). The occurrence of the stimulus-induced silent period was significantly correlated with that of the giant somatosensory evoked potentials (SEPs), and in two patients the silent period was elicited also in the opposite (non-stimulated) hand when the giant SEP was recorded at the hemisphere ipsilateral to the stimulus as well. In one patient, the duration of the silent period was positively correlated with the amplitude of the cortical SEP. Furthermore, the duration of the induced silent period was closely related to the recovery function of SEP in each individual case. In contrast, in the two patients manifesting only positive myoclonus, the silent period was not elicited by the peripheral stimulation, and the somatosensory cortex was hyperexcitable immediately after the peripheral stimulus. Thus, this stimulus-sensitive negative myoclonus is mediated by a transcortical reflex mechanism, and corresponds to the negative form of the cortical reflex myoclonus ('cortical reflex negative myoclonus').

[An autopsy case of neuronal type Charcot-Marie-Tooth disease (HMSN type II) with nerve deafness and psychiatric symptoms].

The clinical and pathological findings of a 41-year-old male patient with atypical Charcot-Marie-Tooth disease were reported. There were 3 cases of subarachnoid haemorrhage, 2 nerve deafness and 2 hereditary motor and sensory neuropathy (HMSN) in his family. He had suffered from progressive nerve deafness since 5 years old and gait disturbance since 37 years old. He had been admitted to the psychiatric hospital 3 times because of hallucinatory-delusional state and behavior abnormalities. Neurological examinations at 39 years old revealed that he had mental deterioration (IQ 66), nerve deafness, diffuse muscle atrophy, most marked distally, sensory disturbance, areflexia, positive Romberg's sign, orthostatic hypotension, dysphagia and slurred speech. MCV of median nerve was 27.8 m/sec, and SCV was not evoked. EEG revealed nonspecific dysfunction of the brain. He died of ileus-like condition at 41 years old. General autopsy showed haemorrhagic infarction of the jejunum and ileum due to compression of the superior mesenteric artery and vein by an adhesion band of connective tissue formed after previous appendectomy. Neuropathological examinations revealed axonal degeneration and loss of myelinated fibers with schwannosis of anterior and posterior spinal nerve roots as well as peripheral nerves. The posterior roots were more severely affected than the anterior ones. Ganglion cells of the posterior root ganglia showed remarkable degeneration and loss. There was severe degeneration of the posterior columns, especially in the gracilis, of the spinal cord. Nerve cells in the anterior horns and Clarke's columns also displayed conspicuous atrophy or central chromatolysis followed by gliosis. There was slight degeneration of the posterior spinocerebellar tracts.(ABSTRACT TRUNCATED AT 250 WORDS)

[Recovery in aphasia (Part 3)--Study by multivariate analysis].

In order to elucidate the factors which have influence on the prognosis of aphasia, correlation between recovery rate of aphasia and the five factors, i.e. initial evaluation of speech test, age, educational level, time between onset and institution of therapy and size of abnormal findings in CT (computed tomography), was studied in 76 right-handed aphasic patients, using multivariate analysis. Cluster analysis and factor analysis were used for analysis of five factors and multiple regression analysis was used for estimation of recovery rate of aphasia. The method of calculation of recovery rate of aphasia is the same to our previous report. The results obtained were as follows: As to analysis of factors, first similarity group among initial evaluation of speech test, recovery rate of aphasia and educational level and second similarity group among time between onset and institution of therapy, size of abnormal findings in CT and age were observed. As to the estimation of recovery rate of aphasia, multiple correlation coefficient of regression analysis using five factors were 0.758, 0.444, 0.627, 0.620, 0.810 and 0.375 respectively in total score, hearing, speaking, reading, writing and calculation on standard language test of aphasia. As to the weight or partial correlation coefficient of regression analysis to the five factors, initial evaluation of speech test, time between onset and institution of therapy and educational level were 129.46, -49.93 and 45.65 respectively and the one of the size of abnormal findings in CT was not high. Stepwise multiple regression analysis of five factors were made.(ABSTRACT TRUNCATED AT 250 WORDS)