To clarify the social life of patients with spondyloepiphyseal dysplasia congenita, we sent questionnaires to the patients who were registered in some orthopedic departments of university hospitals or children's hospitals. Among 63 patients we obtained the answers from 29 patients (twenty males, nine females) for the questionnaires consisting of gross motor development, school attendance, employment, body height at birth and present, site of pain, walking ability and so on. The age ranged from 2 to 69 with the mean of 20 year old. There are three pairs of parent and child, and one pair of siblings. Ninety percent of patients had achieved head control by the age of 8 months, sat without support by 13 months, and walked by themselves by 30 months. Most patients were able to go to ordinal primary and secondary school, and many of them who finished high school went on to university or professional school. Among 7 patients in employment four were desk work and three were manual labor. Except cases of cervical myelopathy walking ability was not limited in juvenile patients, whereas a large number of adult patients complained of gait disturbance. From the analysis of these data, body height at birth and age of walking by themselves correlated to each other regardless of body height at present. In adult patients walking ability correlated to body height at present regardless of their age.

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The specific effect of general dynamic water exercise in 41 year-old woman with chronic cerebellar ataxia induced by meningitis was observed. She was conservatively treated and took bed rest for two weeks, and she suffered from severe dynamic and static ataxia. And exercise under weight-bearing and bandage pressure and Frenkel exercise was applied for nine months. However her disability was not improved. And then she took a 30-minute walking practice in water twice weekly. After that training of 3 months, dramatic improvements in trunk balance and walking speed were demonstrated. She could stand up and walk by herself. The FIM scores for walking, stair stepping and transfer also improved. We suggested that walking practice in water could contribute to improve her ataxia.

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We reported a case of auditory agnosia following bilateral intracerebral bleeding. A 56-year-old man was admitted to our rehabilitation hospital with disorder in cognition of human voices and environmental sounds. He suffered from intracerebral bleeding in right temporal lobe and in left putaminal hemorrage prior to this admission. He had no other cognitive disorders, and bilateral moderate sensorineural hearing loss was revealed by pure-tone audiometry. He could not recognize human voices or other kinds of environmental sounds. However he could communicate by writing, and his spontaneous talking was normal. Auditory brainstem response (ABR) was normal, but middle latency response (MLR) indicated impairments of left auditory radiation and right primary auditory cortex. We introduced lip-reading rehabilitation and educated the patient and his family. After 2 months, he could communicate with his family by listening and lip-reading, but he sometimes needed writing when he communicated with other medical staffs.

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We have developed a quantitative analysis system, which is constructed on a personal computer and a transparent digitizer attached to a crystal display, for estimating functions of voluntary movements in the upper limb. We tried to identify parameters for distinguishing the movement functions of normal subjects and patients with spino-cerebellar degeneration (SCD), and normal ones and patients with Parkinson's disease (PD) using the visually guided tracking method in the system. The subject pursued a target moving on a circle at a fixed speed with a stylus pen, and the position of the pen was digitized and led to the computer. We decomposed the locus of pen into X and Y axes, and calculated the mean velocity (MV), the mean acceleration (MA), the sum of power spectrum of the acceleration waveform (SPS), coefficient of variation of the moving distance per each sampling time (CV), the distance and the delay from the target and the pen pressure. These parameters in normal subjects were statistically compared with those in SCD or PD patients. Parameters of MV, MA, SPS in both X and Y axes, CV and the distance from the target were significantly (p<0.001) larger in SCD patients than in normal subjects. While, PD patients were clearly distinguished from normal subjects by parameters of MA and SPS in the X axis, and CV. Also, we examined the recovery process in patients with normal pressure hydrocephalus (NPH) and patients with cerebral apoplexy (CA) as compared with a doctor's estimation. In NPH patients, it was found that the distance and the delay from the target, and SPS were very effective parameters as quantitative estimation of the recovery process. While, in CA patients, parameters of the delay from the target and SPS in the pen pressure were presented the recovery process similar to doctor's estimation. Thus, it is suggested that the analysis system may be useful to quantify voluntary movement disorders in the upper limb.

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