Hiroki Harayama

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Joseph's disease is a hereditary ataxia found among descendants of Portuguese from the Azores Islands. We describe the clinical and pathological features of 4 members of a Japanese family who were diagnosed as having Joseph's disease. The illness began with cerebellar ataxia between the ages of 18 and 45 years. Nystagmus, dysarthria, and pyramidal signs(More)
A case of adrenoleukodystrophy showing neurological features of olivopontocerebellar atrophy is described. A CT scan demonstrated marked atrophy in both cerebellum and pons. ACTH stimulation produced no rise in the plasma cortisol level but a significant rise in the plasma aldosterone level. The ratios of C26:0 to C22:0 in fatty acids of sphingomyelin from(More)
Reciprocal Ia inhibition from ankle flexors to extensors was studied during voluntary tonic isometric dorsiflexion and plantar flexion in five normal subjects. The Ia inhibition was examined as the short-latency suppression of the soleus H-reflexes by stimulation of the low-threshold afferents in the common peroneal nerve (Mizuno et al. 1971). At rest, weak(More)
Patients with multiple sclerosis sometimes show subthalamic lesions presenting syndrome of inappropriate secretion of ADH (SIADH), hypothermia, hyperprolactinemia, weight loss, and cachexia. Hyperprolactinemia also has been found in the patients with active systemic lupus erythematosus, because prolactin can be produced from human activated lymphocytes. We(More)
An auditory discrimination paradigm was employed to elicit the P300 component of the event-related potentials from three patients with clinically diagnosed corticobasal degeneration (CBD). Presenile and senile dementia of Alzheimer type (DAT), vascular dementia (VD) and age-matched control subjects were also examined. All patients with CBD manifested limb(More)
We have devised a reconstructive procedure to repair chronic injuries to the collateral ligament of metacarpophalangeal (MCP) joints. It comprises palmaris longus tendon grafting into a bone tunnel and suturing onto the base of the residual ligament without involving fixation of the tendon graft stump. Thirteen patients were treated for injured MCP joints:(More)
A new method of measuring the distribution of conduction velocities in human motor fibers is described. In this method, a modification of Kimura's collision technique is combined with Hopf's technique. This enabled us to determine the collision end-point in Hopf's technique, from which the minimum velocity is derived. The size of the distorted compound(More)
To clarify physiological aspects of Machado-Joseph disease (MJD), we studied auditory brainstem response (ABR) and somatosensory evoked potential (SEP) in 17 clinically diagnosed patients with MJD aged 32-64 in Japanese families. ABR was recorded in 13 patients. In 8 patients, ABR were abnormal. In 5 patients, the latency of I wave was normal, but other(More)
Postural abnormalities in a 66-year-old woman with ataxic myxoedema were recorded by stabilography during treatment. After replacement therapy, 3 Hz power in the sagittal plane was greater than before treatment; this peak shifted to a higher frequency and finally its ratio became normal. This is the first report of recovery of a 3 Hz body oscillation in(More)
Human cord dorsum potential (CDP) was recorded from the posterior epidural space at the levels of cervical and lumbar enlargements, using epidural catheters as the recording electrodes, in 15 normal volunteers and six patients suffering from amyotrophic lateral sclerosis. In normal subjects the CDP elicited by descending volleys consisted of spike(More)