Triphasic waves in a patient with tuberculous meningitis.

We report on the case of a 32-year-old woman with tuberculous meningitis (TBM) with electroencephalogram (EEG) output displaying triphasic waves (TWs). The EEG on day 8 revealed generalized slowing, frontal bilateral TWs, a background of 2Hz delta waves, and no epileptiform activity. The patient's condition improved slowly with antituberculosis chemotherapy treatment. A follow-up EEG on day 34 showed marked improvement, with no TWs, background activity improved to a 12Hz symmetric alpha wave pattern, and no epileptiform activity, as before. To our knowledge, this is the first report of TWs observed in a TBM case.

Autoimmune polyglandular syndrome type 2 with myasthenia gravis crisis.

We describe a rare case of autoimmune polyglandular syndrome type 2 initially presenting as Addison disease and autoimmune thyroid disease, with subsequent development of autoimmune hepatitis and myasthenia gravis (MG) crisis in a Japanese woman. MG improved with oral prednisolone followed by plasmapheresis for immunoadsorption; thymectomy was not performed. Conventional treatment for MG was effective and safe in this case, in which there was positivity for human leukocyte antigen A23, B52, B62, DR11, and DR15.

Familial Creutzfeldt-Jakob Disease with a codon 200 mutation presenting as thalamic syndrome: diagnosis by single photon emission computed tomography using (99m)Tc-ethyl cysteinate dimer.

The clinical features of familial Creutzfeldt-Jakob disease with a codon 200 point mutation [fCJD (E200K)] are similar to those of sporadic CJD (sCJD). MRI diffusion-weighted imaging (MRI-DWI) has been reported to be useful for the early diagnosis of CJD. We describe a Japanese fCJD (E200K) case in which thalamic symptoms were the initial manifestations. On admission, electroencephalography (ECG) showed no periodic synchronous discharge (PSD), and MRI showed no abnormalities. However, single photon emission computed tomography (SPECT) using (99m)Tc-ethyl cysteinate dimer ((99m)Tc-ECD) revealed hypoperfusion in the right thalamus. We conclude that the thalamic form of CJD tends to show no high-intensity area (HIA) by MRI-DWI, and that SPECT may be more useful for visualizing the affected area responsible for the thalamic symptoms at an early stage.

Myasthenia gravis with concomitant severe paraspinal muscle degeneration and mitochondrial DNA4977 deletion.

Recent reports have discussed the many causes of dropped head syndrome and bent spine syndrome. We described a case of myasthenia gravis with concomitant severe degeneration of spinal muscle, mitochondrial DNA4977 deletion and sensorineural deafness. These associations were thought to be independent, however this is an important case to consider the etiology of bent spine syndrome.

Myasthenia gravis and diabetes mellitus: a 35-year retrospective study.

BACKGROUND: The most common treatment of myasthenia gravis is high-dose prednisolone administration and thymectomy. A well-known adverse effect of prednisolone is hyperglycemia, however, to date there is no such detailed report. PATIENTS AND METHODS: We treated 325 myasthenia gravis patients in a recent 35 years period, and found 11 patients with diabetes mellitus. We compared these 11 diabetic patients with previously-reported cases. RESULTS: These 11 patients did not have any antibody against beta-cells in the pancreas such as anti-glutamic acid decarboxylase antibody. In 10 of 11 patients diabetes mellitus was controlled with oral medications. CONCLUSION: Myasthenic patients with diabetes mellitus could be classified into 2 groups, one group with positive organ-specific autoantibodies to many organs (with type 1 diabetes mellitus), and the other group with diabetes mellitus onset during prednisolone administration (with type 2 diabetes mellitus).

Histological and immunohistological changes of the skeletal muscles in older SJL/J mice.

SJL/J mice have been studied as the model animals for autoimmunological diseases. Recently it was clarified that SJL/J mice have a defect of dysferlin. Human limb girdle muscular dystrophy 2B and Miyoshi myopathy also have a defect of dysferlin. In this study we present the histological and immunohistological changes in the natural course. Histological study revealed that SJL/J mice had inflammatory, degenerative changes, and neurogenic changes in later ages. As for interstitial inflammatory cells, the macrophages were dominant in any age, and in the T cell subset, the CD4+ T cells were more abundant than the CD8+ T cells, and few B cells were seen. The laboratory data showed a high level of creatine kinase in all ages. It is suspected that the inflammatory changes were induced by the primary immunological abnormality or by the defect of dysferlin in SJL/J mice.

[A case of primary HIV infection presenting as mononeuritis multiplex].

A 34-year-old man presented with gait disturbance. He had a low grade fever and diarrhea for a few days prior to admission. Two weeks after he started to have diarrhea, he developed gait disturbance with a left foot drop. The cerebrospinal fluid obtained on admission showed pleocytosis (30/microl) and increased protein. The motor nerve conduction velocities (MCV) of the left peroneal nerve and the tibial nerve were slow, but the right peroneal and tibial MCVs were within normal limits. A test for human immunodeficiency virus (HIV) antibody was positive. The Western blot was positive with bands of gp160 and p24, confirming HIV infection. Seroconversion-related neuropathy of HIV was diagnosed. Acute HIV infection should be included in the differential diagnosis of mononeuritis multiplex. Before full-fledged AIDS development, it is important to find early stage of HIV infection in patients, so that we can treat them more effectively by means of anti-HIV drugs.

Subacute combined degeneration of the spinal cord concomitant with gastric cancer.

We report a rare case of subacute combined degeneration of the spinal cord concomitant with gastric cancer. A 67-year-old man was admitted because of posterior column symptoms, pyramidal tract sign and peripheral neuropathy with severe hyperchromic anemia. He was treated with mecobalamin 1 mg IM, after which his anemia and neurological signs recovered. He was diagnosed as having subacute combined degeneration with pernicious anemia. Subsequent stomach biopsy revealed gastric cancer, and the patient underwent gastrectomy. It is a well known association that chronic atrophic gastritis is associated with gastric cancer or subacute combined degeneration. Our findings suggest that in this case subacute combined degeneration and gastric cancer are independent of each other; rather, both resulted from chronic atrophic gastritis.

[Postgraduate education for neurologists in hospitals authorized by the Japanese Society of Neurology].

To evaluate postgraduate education for neurologists, we performed a questionnaire-based investigation in hospitals authorized by the Japanese Society of Neurology. The response rate to the questionnaire was 91%. The number of board-certified neurologists is usually less than 5, even in the authorized teaching hospitals. Most respondents thought that the postgraduate training programs already existing in many teaching hospitals are insufficient, but nevertheless useful. More staff may be needed for postgraduate education in neurology. The respondents considered that neurological education has generally functioned well, except in certain areas such as brain death judgment. On the other hand, the rotation of training in neurosurgery, psychiatry and child neurology is still inadequate. The problem of devising a suitable training rotation system in postgraduate education for neurologists has not yet been solved.

New classification and treatment for myotonic disorders.

Myotonia is repetitive firing of muscle action potentials causing prolonged muscle contractions even after mechanical stimulations to the muscles have ceased. Most common myotonic disorder is myotonic dystrophy which is now termed DM1, myotonic dystrophy type 1. In Japan, proximal myotonic myopathy, which is now called DM2 has not been reported. Both DM1 and DM2 have Cl channel abnormality which causes myotonia. Less commonly we encounter Thomsen's disease, and autosomal recessive generalized myotonia (Becker type) which also have a Cl channel abnormality. There are other myotonic disorders related to Na channelopathy which include three disorders: paramyotonia congenita, adynamia episodica hereditaria, and myotonia fluctuans. Myotonia has been treated by various Na channel blockers, mexiletine, phenytoin, and carbamazepine, but they were originally developed for cardiac arrhythmia, or seizure disorders and they have undesirable side effects, weakness. Comprehensive treatment includes myotonia control without reducing the strength, and care for systemic manifestations of DM1.

[A case of Brown-Vialetto-van Laere (BVVL) syndrome in Japan].

Here we report a sixty-year-old woman of Brown-Vialetto-van Laere (BVVL) syndrome in Japan. She had sensorineural deafness, weakness and atrophy of her extremities from 15 years of age. Her neurological symptoms slowly progressed. She first visited our hospital in 1993 when she was 49 years old. At that time, she had distal muscle weakness and atrophy of the four extremities and bulbar palsy. Deep tendon reflexes were absent and the plantar toe reflex was flexor. EMG revealed neurogenic changes and the nerve conduction studied were normal. The vital capacity was marked decreased. On August 10, 2003, she was admitted to our hospital because of CO2 narcosis. She had III, VII, X, XI, XII cranial nerve palsy, distal muscle weakness and atrophy of the four extremities. From her neurological symptoms and signs, we made a diagnosis of BVVL syndrome. MRI revealed no high signal in pyramidal tract by FLAIR image. ABR showed no response, and VEP demonstrated delay of the P100. She was intubated, and was attached to a respirator to improve her CO2 narcosis. After treatment she improved and did not need to be assisted by a respirator during daytime. During night time, she had apnea, and her blood gas showed the retention of CO2, and she still required the respiratory assistance during her sleep. This is the first report of BVVL syndrome in Japanese literature.