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Spinocerebellar degeneration (SCD) is a progressive neurodegenerative disorder in which cerebellar ataxia causes motor disability. There are no widely applicable methods for objective evaluation of ataxia in SCD. An objective system to evaluate ataxia is necessary for use in clinical trials of newly developed medication and rehabilitation. The aim of this study was to develop a simple method to quantify the degree of upper-limb ataxia. Forty-nine patients with SCD participated in this study. Patients were instructed to trace an Archimedean spiral template, and the gap between the template spiral and the drawn spiral (gap area; GA) was measured using Image J software. Ataxia was rated using the Scale for the Assessment and Rating of Ataxia (SARA) and cerebellar volume was evaluated in 37 patients using an axial cross-section of magnetic resonance images that were obtained within 6 months of clinical evaluation. Regression analysis was performed to assess the relation between GA and patient age, disease duration, SARA score, and cerebellar volume. GA was significantly related to total SARA score (r = 0.660, p < 0.001), the posture and gait (r = 0.551, p < 0.001), speech (r = 0.527, p < 0.001), hand movements (r = 0.553, p < 0.001), and heel-shin slide (r = 0.367, p = 0.036) SARA subscores, and cerebellar volume (r = 0.577, p < 0.001) but was not related to patient age (r = 0.176, p = 0.227) or disease duration (r = 0.236, p = 0.103). GA is a simple, useful method to objectively quantify the degree of cerebellar ataxia, especially upper-limb ataxia, and can be widely adopted in various settings, including clinical trials.

We report a 54-year-old man with right abducent nerve palsy, right facial nerve palsy, and left segmental sensory disturbance, which progressed for 2 weeks. He was found to have cavernous angioma in the lower pons. When he visited our hospital, he had right facial palsy, sensory disturbance of left half of the face and left upper limb, and diplopia. He had suffered right abducent nerve palsy 5 years previously and had recently developed hypertension. Neurological examination further revealed right abducent nerve palsy, right peripheral facial nerve palsy, sensory impairment of the left half of the face, and sensory impairment on the left side from C2 to Th3. Magnetic resonance imaging of the head revealed hemorrhage with a rim at the right dorsal part of the lower pons. No abnormalities were identified on cerebral angiography. He was diagnosed as having hemorrhage originating from a cavernous angioma. We assumed that the segmental sensory disturbance was caused by medial involvement of the lateral spinothalamic tract, which is somatotopically arranged: the fibers from the sacral segments being most lateral. The ventral trigeminothalamic tract, right abducent nerve, and right facial nerve were also disturbed. Segmental sensory disturbance usually accompanies a spinal cord lesion. But several cases with similar symptoms following a brainstem lesion have been reported. Most of them had stroke, showing acute onset of illness. Our case showed subacute onset of illness; cranial nerve palsy and segmental sensory disturbance progressed for 2 weeks.

A-58-year old man presented with fluctuating ptosis and dysphagia. When he was 53 years old, he developed oral candidiasis and serum human immunodeficiency virus (HIV) RNA was detected. After starting highly active antiretroviral therapy, serum HIV RNA became undetectable. Neurological examination revealed ptosis and bulbar symptoms. Myasthenia gravis was comfirmed by a positive edrophonium test, showing 20% decrement of the compound muscle action potential on repetitive stimulation. Anti-acetylcholine receptor antibodies were negative and anti-muscle specific tyrosine kinase (MuSK) antibodies were positive. The chest CT scan was normal. He experienced transient clinical remission with pyridostigmine bromide and prednisolone. However relapse occurred after he returned to work. Persistent clinical remission was first observed after cyclosporin administration. There are eleven reports in which patients had concomitant myasthenia gravis and HIV infection. Most of those cases were benign in clinical course and required only anticholinesterase therapy. In our case, however, anti-MuSK antibodies were positive, and symptoms of myasthenia gravis remained despite prednisolone administration. Cyclosporin is directly active against HIV, and thus, cyclosporine therapy may be helpful in patients with concomitant myasthenia gravis and HIV infection.

Ten subjects were asked to pay attention to green or to red, when each visual stimulus was presented as two small squares, one green and the other red. They were instructed to push a button with the right hand, when the attended color was on the right side, and to push a button with the left hand, when the attended color was on the left side. The P1/N1 peak-to-peak amplitudes of visual event-related potentials were significantly higher when subjects focused attention on green rather than on red. We assume that the attended color had the effect of modulating the P1/N1 components.

A 34-year-old man with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) showed chronic intestinal pseudo-obstruction (CIPO), which was improved by the administration of distigmine bromide. He exhibited generalized tonic clonic seizures at the age of 21, and mitochondrial DNA analysis showed the MELAS mutation. At the age of 34, he became akinetic mutism after nonconvulsive status epilepticus and needed enteral nutrition through a nasogasrtic tube. However, he developed abdominal distention and vomiting, and was diagnosed as CIPO, therefore tube feeding was stopped. Although the administration of domperidone, mosapride citrate, butyric acid bacteria, sodium picosulfate, prostaglandin F2 alpha, pantothenic acid, dioctyl sodium sulfosuccinate, and so on, was ineffective, the administration of distigmine bromide improved his bowel motion disturbance and abnormal distention. The present case is the first MELAS patient with CIPO to be ameliorated by distigmine bromide, which might work acetylcholine receptor on the interstitial cells of Cajal.