Serial MRI Findings in a Clinically Diagnosed Adult Onset Leigh Syndrome

Adult onset Leigh syndrome is a very rare neurodegenerative disorder of unknown cause. We report the evolution of the lesions on serial MRIs in a 38-year-old man with clinically diagnosed Leigh syndrome. We emphasize that the mammillary bodies can be involved during the disease course and that premortem diagnosis of Leigh syndrome is pos-sible, if a characteristic distribution of lesions can be demonstrated on MRI.

A Case of Lateral Sinus Thrombosis Presenting with Cranial Nerve Syndrome

Cerebral venous thrombosis (CVT) is a disease sometimes associated with a wide variety of clinical signs and symp-toms. Single or multiple cranial nerve palsies without evidence of any other signs and symptoms have not, so far, been considered as a relevant syndrome of CVT. A 49-year-old woman was admitted to our hospital because of diplopia, vertigo with tinnitus and ataxia which had begun approximately three weeks before. Neurological examination revealed a right 6th nerve palsy by a red glass test and an impaired tandem walking test. Laboratory tests including CSF exami-nation and brain MRI were normal but MR venography and following 4-vessel angiography showed thrombosis in the left transverse and sigmoid sinuses and suspicious thrombosis in the right lateral sinus. She was treated with heparin and proceeded to get well.

Reversible MRI Findings in Metronidazole-induced Cerebellar Dysfunction

Metronidazole is widely used for the treatment of trichomoniasis, giardiasis, amebiasis, and anaerobic infections. It pro-duces a number of neurological side effects including peripheral neuropathy, encephalopathy, cerebellar dysfunction, and seizures. A 66-year-old male patient was admitted to our department because of a tingling sensation in both toes as well as ataxia with dizziness which had developed five prior to admission. He had had been diagnosed with hepatoma and had an operation in 1993. One month before admission, he felt a febrile sensation and was diagnosed as having abdominal abscess by an ultrasonography, and took about 30g of metronidazole until visiting our emergency room. A nerve conduc-tion test revealed sensorimotor polyneuropathy in the four extremities. A brain MRI showed symmetrically increased signal intensities without enhancement in the supratentorial white matter and the dentate nucleus of the cerebellum. After stopping metronidazole, his neurological symptoms began to improve and on the following MRI taken seven weeks later, the symmetrically increased signal intensities in the dentate nucleus of the cerebellum were nearly disappeared.

Reversible MRI Findings in Metronidazole-induced Cerebellar Dysfunction

Metronidazole is widely used for the treatment of trichomoniasis, giardiasis, amebiasis, and anaerobic infections. It pro-duces a number of neurological side effects including peripheral neuropathy, encephalopathy, cerebellar dysfunction, and seizures. A 66-year-old male patient was admitted to our department because of a tingling sensation in both toes as well as ataxia with dizziness which had developed five prior to admission. He had had been diagnosed with hepatoma and had an operation in 1993. One month before admission, he felt a febrile sensation and was diagnosed as having abdominal abscess by an ultrasonography, and took about 30g of metronidazole until visiting our emergency room. A nerve conduc-tion test revealed sensorimotor polyneuropathy in the four extremities. A brain MRI showed symmetrically increased signal intensities without enhancement in the supratentorial white matter and the dentate nucleus of the cerebellum. After stopping metronidazole, his neurological symptoms began to improve and on the following MRI taken seven weeks later, the symmetrically increased signal intensities in the dentate nucleus of the cerebellum were nearly disappeared.

Sympatheic Skin Response and Nerve Conduction Studies in Diabetic Neuropathy

Fifty eight diabetic patients and 20 normal controls were studied by the method of nerve conduction studies(NCS) and sudomotor skin reponse(SSR), and the following results were obtained. 1. SSR was easily obtainable in all normal control. 2. The patients with abnormal NCV showed more numbers of abnormal SSR than the pat ients with normal NCV. 3. The patients with abnormal SSR were significantly older than the patient with normal SSR, but duration of disease was not different between the patients with normal SSR and with abnormal SSR. 4. The levels of sugar at fasting and postprandial 2 hours, and level of HbAlC in serum were not different between the patients of norrnal SSR and of abnormal SSR. 5. The incidence of autonomic symptoms, such as syncope, sweating abnormality, impotence in male, urinary disturbance, postural hypotension, was not different between the patient of norrnal SSR and of abnormal SSR. 6. Among the patients of diabets, the number of abnormal nerves in terminal latency motor and sensory nerve condcution velocity, and amplitude of compound motor and nerve action potentiaL were significantly correlated with the number of abnormal nerves in SSR. 7. The absolute values of motor terminal latency, motor and sensory conduction velocity and amplitude of compound motor and sensory action potential in the patient of abnormal SSR were significanly decreased thai. The patients with norrnal SSR. The values of terminal latency in abnormal SSR were significanly prolonged than the patients with normal SSR We concluded that the SSR test is a valuabe one for early detection of sympathetic nerve invasion in the diabetes' patients.

A Case Report of Neuronal Type of Charcot-Marie-Tooth Disease

We report a familial case of neuronal type of Charcot-Marie-Tooth disease diagnosed by sural nerve biopsy, nerve conduction study, and electromyography.