Beneficial effect of interferon-beta treatment in patients with multiple sclerosis is associated with transient increase in serum IL-6 level in response to interferon-beta injection.

In order to predict the clinical benefit of interferon-beta (IFN-beta) to patients with multiple sclerosis (MS), the following markers were investigated; (1) chronological change of cytokines (IFN-gamma, TNF-alpha, IL-6, IL-10, and TGF-beta) after administration of IFN-beta, (2) untoward effects of IFN-beta such as headache and arthralgia, (3) backgrounds of the patients such as age and relapse rate, (4) efficacy of IFN-beta therapy assessed by the change of relapse rate and progression of disability. Chronological blood sampling was performed 0, 10, and 24 h after injection of IFN-beta. The increase of serum IL-6 level in response to IFN-beta administration was associated with headache, arthralgia, relapse rate before treatment, and disability score at the initiation of the therapy. Significant association of change of serum TNF-alpha with age and headache was also observed. The important finding in this study was that patients with a transient increase in IL-6 in response to IFN-beta showed a slow disease progression. This result suggests that this transient increase in the serum IL-6 predicts favorable response to IFN-beta treatment.

Clinical features, effectiveness of drug-based treatment, and prognosis of new daily persistent headache (NDPH): 30 cases in Japan.

Although new daily persistent headache (NDPH) is considered to be one of the most refractory headaches to pharmacological treatment, the effectiveness of drug-based treatment, and the prognosis of NDPH have not been well studied. The purpose of this study is to evaluate the effectiveness of pharmacological treatment of NDPH. Seventeen men and 13 women who met the IHS diagnostic criteria for NDPH, were treated for five years from November 1997 to October 2002, and whose headache conditions were entirely available for reference as of October 2003 were investigated in Toyonaka Municipal Hospital. Mean age at onset 35.0 years (range 13-73 years). The onset of headache occurred in relation to a stressful life event in six (20%) patients and any precipitating events could not be identified in 24 (80%) patients. For treatment, muscle relaxants were first administered and if no effect was observed, tricyclic antidepressants, selective serotonin reuptake inhibitors, and antiepleptic drugs were subsequently administered. Results of the drug-based treatments in the 30 cases were 'very effective' for eight (27%) cases, 'moderately effective' for one (3%) case, 'mildly effective' for six (20%) cases, and 'not effective' for 15 (50%) cases. According to the survey on the phone for the patients who did not improve to the level of 'mildly effective' or better and did not come back to our institution as of October 2003, there were no cases found to have spontaneously improved to the level of 'mildly effective' or better. These results suggest that NDPH is highly recalcitrant to the treatments with poor prognosis and better treatments are needed.

[An autopsy case of malignant lymphoma who showed hearing loss and cavernous sinus syndrome in the first stage and hemophagocytic syndrome].

A 48-year-old man was admitted to our hospital due to multiple cranial neuropathy such as vertigo, hearing loss of the left ear, visual loss of the left eye, left ptosis and total ophthalmoplegia of the left eye. Brain MRI revealed no abnormalities. After 10 days, he suddenly showed hemophagocytic syndrome with high fever, liver dysfunction, jaundice and pancytopenia. In spite of the treatment, he died of multiple organ failure after 26 days. At autopsy, malignant lymphoma cells of T cell origin directly invaded into the liver, spleen, intraperitoneal lymph nodes and other organs. Reports that direct invasion of lymphoma cells could be detected at the cranial nerves in the cavernous sinus are very rare.

[A case of musicogenic epilepsy induced by listening to an American pop music].

This is the second report of musicogenic epilepsy in Japan. A 23-year-old woman was admitted to our hospital due to the musicogenic epilepsy. She had four generalized tonic clonic seizures at 18 and 19 years old. Since 19, she had had complex partial seizures lasting for about 20 seconds which was easily evoked by listening to an American pops particularly "Dreamlover" song by Mariah Carey. Brian MRI and interictal 99mTc HMPAO-SPECT showed no abnormalities. In the ictal EEG recording, three minutes after listening to the song, seizure activities were recorded from the right temporal region with 11Hz rhythmic epileptiform activities over the right temporal region and subsequent delta activities over the right hemisphere. The song that induced the seizure had a specific meaning that might evoke her emotion as had been pointed out in the previous reports.

Hypoglossal neurinoma extending intra- and extracranially: case report.

BACKGROUND: Hypoglossal neurinoma is very rare; our case is the 46th case. CASE DESCRIPTION: We report a 59-year-old woman with hypoglossal neurinoma. Her equilibrium state was disturbed due to vestibular dysfunction and she exhibited right hypoglossal palsy with glossal hemiatrophy. Computed tomography (CT) revealed a large bony erosion of the right hypoglossal canal. Magnetic resonance imaging (MRI) revealed intra- and extracranial tumor extension. There was an intracranial mass attached to the enlarged hypoglossal vertebral and posterior inferior cerebellar artery medially. The extracranial part of the mass extended along the course of the hypoglossal nerve through the enlarged hypoglossal canal. The intracranial part of the tumor was totally removed via the transcondylar approach. The diameter of the origin of the posterior interior cerebellar artery was narrow before tumor removal; it increased after surgery due to decompression. A histological diagnosis of neurilemoma was made. Her symptoms due to vestibular dysfunction disappeared postoperatively. CONCLUSIONS: Our patient's difficulty with balance was thought to be an ischemic symptom due to circulation disturbance of the posterior inferior cerebellar artery. It disappeared after tumor removal. Surgery may be necessary later if further growth of the extracranial part of the neurinoma becomes evident.

Eyelid "apraxia" in patients with motor neuron disease.

Three patients with motor neuron disease had eyelid "apraxia" with impaired voluntary but preserved involuntary eyelid movements. Attempts were made to localise the lesions responsible with neuroimaging and neuropathological examination.

[A case of pituitary abscess caused by infection of Rathke's cleft cyst].

Pituitary abscess is relatively rare. Only about 80 cases have been reported. Preexisting lesions in pituitary fossa, such as pituitary adenoma, craniopharyngioma and Rathke's cleft cyst, are inclined to be complicated by infection more than the normal pituitary glands are. We reported a case of pituitary abscess caused by infection of Rathke's cleft cyst. A 67-year-old male had general fatigue and loss of appetite 4 months before admission. On admission he was found to have diabetes mellitus, diabetes insipidus, and hypernatremia. These defects were controlled by medication but he gradually became comatose and febrile. CT and MRI revealed an intrasellar lesion with ring enhancement. Lumbar puncture demonstrated an increase of mononuclear cells and protein. Blood chemistry revealed a marked increase of CRP. He was operated on via the transsphenoidal approach, which revealed sphenoid sinusitis and abscess formation in the pituitary gland. Histological examination of the surgical specimen revealed infection of Rathke's cleft cyst but the fluid in the cyst was sterile. By the drainage of the cyst and the use of antibiotics the patient became alert and signs of infection disappeared. He was discharged with a slight hypopituitarism and returned to normal life. Mortality rate of pituitary abscess is decreasing but is still high because of hypopituitarism and severe infection. Accurate diagnosis and operation are necessary. Transsphenoidal surgery is preferable for postoperative drainage of the abscess.

Glutamate metabolism of leukocytes and skin fibroblasts in spinocerebellar degeneration with lowered glutamate dehydrogenase activity.

From 21 patients with spinocerebellar degeneration 5 had markedly decreased glutamate dehydrogenase (GDH) activities and high values of serum plasma glutamate level after oral glutamate loading tests. Skin fibroblasts from patients with GHD deficiency showed intracellularly higher glutamate and lower glutathione contents than those from controls and showed significantly decreased viability in L-glutamate-containing medium. These data suggest that glutamate toxicity may at least play a part in this degeneration process.

Mitochondrial encephalomyopathy with sleep apnea.

A rare case with mitochondrial encephalomyopathy, in association with cerebellar ataxia, peripheral neuropathy, mental retardation and alveolar hypoventilation syndrome with sleep apnea, as demonstrated by polysomnography, was encountered. This combination has not been described previously. From a prognostic point of view, alveolar hypoventilation syndrome with sleep apnea is an important clinical feature is this disease entity. Neither ataxia nor the abnormality of pyruvate metabolism was alleviated after 6 months of therapy with coenzyme Q10.