In order to meet the needs for medical care after discharge from hospital of serious or intractable neurological disease patients, in 1978, Shinrakuen Hospital in Niigata City launched the Continuing Medical Care Section providing home visit by doctors and periodical home nursing. In the article published in 1990 reporting the 13-year practice of this section, the author advocated the necessity of the across-the-board and continuing regional care system in support of homecare and proposed a desirable model. Since then, with the policy shift from hospital care to homecare facing the progress of aging society, various homecare services have been developed. The author opened in 1997 an organized clinic of internal and neurological medicine in Niigata City, and, on the basis of long term care insurance program founded in 2000, put into practice the homecare system of cooperation between the prefectural and municipal administration, medicine and nursing. In this article, based on her article in 1990, the author adds the report on the development of this regional care system and evaluates the efficacy of homecare supported by regional system in intractable neurological disease medical care.

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Adverse reactions to genral anesthesia, which partly resembled malignant hyperthermia (MH), were more frequent in muscular dystrophy than in controls. In the present study, 35 cases so far reported in Duchenne or Becker muscular dystrophy (DMD or BMD) were analyzed and their pathogenesis was discussed. Cardiac involvements were sole manifestations in 7 cases. In other 28 cases, the acute rhabdomyolysis was the most prevailing manifestation. About 60% of myolysis cases were associated with muscle contracture (rigidity) or other hypermetabolic signs such as hypercapnia, hyperthermia and metabolic acidosis. Cases with BMD were more hyperthermic than with DMD. These results suggest Ca ion-induced hypermetabolic reactions are also present in dystrophinopathy, which have been assumed as core syndromes of the classical (gene-defined) MH. However, question whether the abnormal Ca ion is from the extracellular or intracellular stores is still unclear. Circumstancial evidences suggest that the Ca-induced Ca release (CICR) mechanism might also be involved. Endogenous redox modulators such as nitric oxide or reactive oxygen species in the dystrophic muscle might contribute to the perturbed Ca ion homeostasis.

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The Ministry of Health, Labour and Welfare (MHLW) of Japan has funded special research programs to investigate etiology and treatment of intractable neurodegenerative diseases. Individual health-care expenditures for registered patients with Parkinson's disease (PD) (Hoehn and Yahr Stage 3-5) have been evaluated by the government. The total number of patients registered with a diagnosis of degenerative parkinsonism including PD, progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) was 75,026 in 2004. We analyzed the data from a survey of application forms completed by 23,058 PD patients, which had been entered into the MHLW's computer. The male to female ratio was 1 : 1.47, and the mean ages of all patients at registration and at onset were 71.3 years and 62.7 years, respectively. Incidence of young-onset Parkinson's disease was 2.7% of all PD patients. The percentage of PD patients at Hoehn and Yahr Stage 3 was 48.8%, at Stage 4, 23.1%, and at Stage 5, 25.2%. About 60% of patients resided at home, whereas 20% were admitted into the hospital and 20% into a nursing home. The percentages of patients with diurnal fluctuations, dyskinesia and psychotic symptoms were 37.3%, 16.8% and 18.4%, respectively. Ages of patients at registration and at disease onset who presented with diurnal fluctuations and dyskinesia were typically younger and the duration of disease was longer than for those presented without them (p<0.0001). Patients with psychotic symptoms were older and the duration of disease was longer than those without them (p<0.0001). Ages of patients receiving ablative surgery were younger than those who received deep brain stimulation (DBS) (57.4 versus 63.1 years, p<0.01). Approximately 70% of all the patients who underwent stereotactic surgeries were treated within 10 years from onset. The percentage of patients with feeding tube was 6.6%, and the age at registration was older (75.6 versus 71.0 years, p<0.0001) and duration of disease was longer (11.0 versus 8.4 years, p<0.0001) in patients with feeding tube than in patients with oral feeding. This is the first analysis of results from the survey of questionnaire for PD provided by MHLW.

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[Purpose] On plain brain computed tomography (CT), it is difficult to evaluate stenosis of internal carotid artery (ICA) because ICA is surrounded by structures, even though we can observe calcification of carotid siphon in some patients by using bone condition. However the pathologic significance has not been well known. We studied the pathologic significance of carotid siphon calcification observed on bone condition of brain CT. [Methods] A total of 112 patients who were diagnosed or suspected as cerebrovascular diseases were registered. We classified the calcification into four levels (none, mild, moderate, severe) based on the degree of calcification. Then we compared it with the degree of stenosis of carotid siphon seen on brain magnetic resonance angiography (MRA) and with max intima-medial thickness (IMT) from common carotid artery (CCA) to ICA on carotid ultrasonography. [Result] The mean±standard deviation of max IMT to none, mild, moderate and severe in the degree of calcification were 1.03±0.64 (0.4-2.8), 1.65±0.83 (0.5-4.1), 2.03±0.83 (0.8-4.1) and 2.81±1.15 (0.7-6.5) mm, respectively. The calcification on brain CT significantly correlated with the degree of stenosis on brain MRA and with max IMT on carotid ultrasonography. [Conclusion] The calcification of carotid siphon on bone condition of brain CT correlated with stenosis of the same portion and atherosclerosis of CCA bifurcation. Recently, on DICOM viewer, clinicians can convert plain condition into bone condition on brain CT due to popularization of PACS. We should pay attention to calcification of carotid siphon in patients with ischemic cerebrovascular diseases because we can estimate the atherosclerosis of both carotid siphon and CCA bifurcation easily and immediately.

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We report a 31-year-old woman with tumor-induced osteomalacia suffering from slowly progressive bilateral muscle weakness predominantly in the proximal muscles and multiple bone pains for the past 2 years. She was unable to walk or raise her arms above the shoulder. We suspected tumor-induced osteomalacia due to decreased serum phosphate and 1α, 25 (OH)₂-vitamin D3 levels, low percentage of tubular reabsorption of phosphate (%TRP), adult onset, and no family history of osteomalacia. Regular imaging examinations could not detect the location of the primary tumor; however, indium-111 octreotide scintigraphy detected the causative primary mesenchymal tumor in the right sole. Pain and muscle weakness improved promptly after tumor resection, and she was able to walk 6 d postoperatively. This is the first case report in Japan describing the detection of the primary tumor site by indium-111 octreotide scintigraphy.

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We report an 81-year-old woman who suffered from theophylline-associated seizures followed by partial seizures due to vitamin B6 deficiency. She developed complex partial seizures. She had been treated with theophylline for two months because of chronic bronchitis. Brain diffusion-weighted magnetic resonance imaging (MRI) showed high intensity lesions in unilateral cerebral cortex and thalamus. Electroencephalogram presented periodic lateralized epileptiform discharges (PLEDs), and single photon emission computed tomography (SPECT) using ¹²³I-IMP revealed increased blood flow in the same side of the cerebrum. We diagnosed as theophylline-associated seizures though blood theophylline concentration disclosed its therapeutic dose, and her symptom improved after theophylline was discontinued. She developed partial seizures again, after she suffered from diarrhea for two days. Laboratory examination showed that serum vitamin B6 was under the limitation of measurement, and intravenous supplementation of vitamin B6 stopped her seizures immediately. Theophylline may induce seizures independent of its blood concentration, and vitamin B6 deficiency may exist in the case of theophylline-associated seizures.

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A 23-year-old woman was admitted to our department because of gait disturbance, sensory impairment in the lower limbs, and sphincter disturbance, all of which had been developing within 24 hours before admission. Neurological examination disclosed symmetric muscle weakness, sensory impairment, and diminished tendon reflexes in the lower limbs. The urinary bladder was hypoactive, and the anal tone was reduced. The spinal cord MRI performed on the day of admission revealed swelling of the epiconus. The CSF findings were not remarkable, except for the elevated levels of IgE (8IU/ml) and MBP (7.8ng/ml). Besides, there was a marked increase in the serum mite-specific IgE titers. Collectively, we made a diagnosis of atopic myelitis. She was treated with steroid pulse therapy and plasma exchange, which led to a significant amelioration of her neurological manifestations. The repeat MRI carried out on the 21st day of her admission displayed several foci scattered in the lumbar and sacral spinal cord segments, which exhibited high intensity signals on the T2-weighted images. The values of IgE and albumin in the CSF and serum raised the possibility of intrathecal IgE synthesis. We measured her CSF IgE levels at several time points during admission. The temporal profile of her CSF IgE levels was not correlated with that of her neurological disabilities.

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We report a 73-year-old man with alternating abducent hemiplegia (Raymond syndrome) and contralateral supranuclear facial nerve palsy. On admission, he showed lateral gaze palsy of the right eye, left supranuclear facial nerve palsy, dysarthria and left hemiparesis. Brain MRI showed an infarct that was located in the paramedian and lateral area in the base of the caudal pons on the right side. MRA showed a mild stenosis of the basilar artery. Hemiplegia and supranuclear facial nerve palsy were considered to be caused by the involvement of corticospinal tract and corticobulbar tract that run at the ventromedial area of the pons. Abducens nerve palsy was considered to be caused by the involvement of infranuclear abducens nerve fibers. There has been one previously reported case of Raymond syndrome in which MRI determined the precise location of the lesion. In this case, a small hematoma was found at the ventral and medial pontomedullary junction, whereas the infarct in our case was located in the pontine base. We considered that documentation of our case was an important contribution to determine the pathogenesis of supranuclear facial nerve palsy due to caudal pontine lesions.

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We examined the effect of levodopa (L-DOPA) in a patient with a lesion of the amygdala and impaired recognition of emotion. Before L-DOPA, recognition of fear and disgust was significantly impaired compared to recognition scores in controls. After receiving L-DOPA, the patient showed normal recognition of fear and disgust. After the drug wore off, recognition declined to her initial unmedicated performance. We hypothesized that L-DOPA may have activated dopaminergic systems involved in recognition of emotion such as the medial prefrontal cortex and anterior cingulate cortex, compensating for the amygdala dysfunction. Alternatively, L-DOPA may have activated intact portions of the amygdala.

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A 77-year-old woman was admitted to our department due to leg edema of 2-year duration. The patient has been suffered from Parkinson's disease for 12 years and prescribed levodopa, selegiline, and small dosage of pergolide (200μg/day). Leg edema developed one year after she took pergolide. Bilateral peripheral effusion was shown without any findings for malignancy, infection, and heart failure. After discontinuation of pergolide, both pleural effusion and systemic edema were solved. Pergolide was reported to cause cardiac valve fibrosis, pleural effusion and fibrosis, and peritoneal fibrosis. This case suggests that low dose pergolide (200μg/day: cumulative dose is about 200mg) could cause severe pleural effusion and systemic edema.

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