A case of acute encephalitis with refractory,repetitive partial seizures (AERRPS) showing transient disappearance of the seizure with occurrence of choreo-ballistic movement

We present here a 5-year-old girl with acute encephalities with refractory, repetitive partial seizures (AERRPS), a new clinical entity defined by the following five criteria: 1. acute encephalitis with a prolonged acute phase of more than 2 weeks, 2. persistent partial seizures with identical phenotype both in the acute and recovery phase, 3. seizures frequently evolving into convulsive status especially during the acute phase, 4. extremely intractable, and 5. no causative lesion or agent is identified. Interestingly, her seizures had completely diminished from the fifty-sixth day of her illness with concomitant appearance of choreo-ballistic involuntary movements. After the 120th day of the illness, seizures evolved again, though the involuntary movements persisted. This transient disappearance of intractable seizures might provide a clue to the pathophysiology of seizures in AERRPS.     

Chemiluminescent detection of induced reactive oxygen metabolite production of human polymorphonuclear leucocytes by anthophyllite asbestos

Incidences of lung cancer and pleural plaque have been reported in relation to exposure to anthophyllite asbestos. To investigate the pathogenic mechanisms of anthophyllite, chemiluminescence (CL) detection of reactive oxygen metabolite (ROM) generation of human polymorphonuclear leucocytes (PMN) stimulated by anthophyllite asbestos was determined and compared with that of other asbestos and mineral fiber samples. When anthophyllite fiber sample was mixed with the luminol-primed PMN, high levels of CL which exhibited a specific time course characterized by two separate peaks were induced. The CL induced by anthophyllite sample was greater than that induced by chrysotile, crocidolite, and amosite asbestos. We further investigated the two peaks of CL using specific inhibitors of signal transduction mechanisms. The two peaks of CL by anthophyllite sample were different in sensitivity to cytochalasin B and genistein; the former relates to the cytoskeleton-dependent mechanism and the latter has been shown to inhibit tyrosine kinase, which resides in the pathway to cause PMN activation. The strong ROM reaction of PMN by anthophyllite suggests that the surface characteristics of the fiber may participate in the pathogenic mechanisms of anthophyllite asbestos.     

Primary Sjögren's syndrome initially manifested by optic neuritis: MRI findings

We herein describe the MRI findings in a patient clinically diagnosed with primary Sj?gren's syndrome (SjS) initially manifested by retrobulbar optic neuritis. A 63-year-old woman suddenly had left ocular pain and progressive visual disturbance. MR T2-weighted images revealed hyperintensity in the left optic nerve, with swelling. Contrast-enhanced T1-weighted images showed no abnormal enhancement. Follow-up MRI 6 months after admission revealed no significant changes in the affected optic nerve. To our knowledge, optic neuritis as a complication of SjS has been reported in ten patients [1, 2, 3, 4, 5, 6] and MRI findings in only one of them [6]. We thought MR images were useful for visualizing optic nerve involvement in SjS and observing its course.     

A case of large carcinoma of the tongue and mouth floor,in which chemotherapy using a combination of nedaplatin and 5-FU was effective

We encountered a case of extensive squamous cell carcinoma ranging from the tongue to the mouth floor, in which chemotherapy using a combination of nedaplatin and 5-FU was effective. The patient was a 46-year-old male, who noticed a small mass in the mouth floor in September 2000, and visited the department of oral and maxillofacial surgery at a hospital on October 12, 2000. A 27 x 15 mm tumor with erosion was noted on the mouth floor, which was diagnosed as squamous cell carcinoma by biopsy, and the patient was referred to our department for treatment on November 16, 2000. Intra-oral findings included a healthy-colored induration with a bulge in the area ranging from the apex of the tongue posteriorly for 30 mm, and from the right lateral edge of the tongue to the left side exceeding the median area of the tongue by approximately 7 mm. MRI detected a tumor of approximately 2 cm in diameter in the area ranging from the median area of the tongue to the right ventral side of the tongue, which protruded on the mouth floor side. Two courses of combination chemotherapy using nedaplatin and 5-FU were performed. Nedaplatin (90 mg/m2, total 143 mg) and 5-FU (700 mg/m2, total 1,113 mg) were administered in a first course during December 15-19, 2000, and in the same amounts in a second course during January 23-27, 2001. Adverse effects of gastralgia and stomatitis occurred, but they gradually disappeared with time. The tumor with erosion in the tongue and mouth floor and induration disappeared 2 weeks after administration. Tongue was recovered, so that tongue movement was almost normal. Postoperative MR showed no abnormal signals in T2-weighed images, suggesting that the tumor in the right mouth floor had almost disappeared. External irradiation (40 Gy/20 times/28 days, 2 Gy/day, opposing bilateral portal irradiation) between March 13 and April 9, 2001, and micro-selection high dose fractionated interstitial irradiation (42 Gy/7 times/6 days, 6 Gy/1 time) April 18-23, 2001 were performed as booster therapy. Outpatient observation has shown a good clinical course without recurrence.     

Rapid eye movement sleep in breath holders

One-night polysomnography was performed on seven subjects suffering from breath-holding spells, including one whose death was suggested to be a consequence of a breath-holding spell. The fatal case showed no rapid eye movements (REMs) during REM sleep, although he exhibited REMs during wakefulness. The average numbers of both REMs and bursts of REMs in REM sleep in the other six breath holders were significantly lower than those in age-matched controls. The breath holders showed no airway obstruction, desaturation, or sleep fragmentation. Since the rapid ocular activity in REM sleep is generated in the brain stem, we hypothesized that a functional brainstem disturbance is involved in the occurrence of breath-holding spells.     

Sibling cases of a degenerative neurological disease associated with hypocupraemia and hypobetalipoproteinaemia

We describe two siblings, a boy and his younger sister, with degenerative neurological disturbances, hypocupraemia and hypobetalipoproteinaemia. The neurological features in both cases were developmental delay, dysarthria, hyperkinetics with an attention deficit, dysdiadochokinesis, night blindness, myoclonic jerks and convulsions. Their serum cooper levels did not increase despite administration of copper sulphate both orally or intravenously. The copper contents of the cultured fibroblasts in the patients were 1.5-fold that of controls.Although neurological disorders associated with abnormal copper metabolism and inherited in an X-linked manner have been previously reported, this is the first report of a neurodegenerative disease concurrent with abnormal copper metabolism and hypobetalipoproteinaemia.     

Three cases of malakoplakia of prostate

Three cases that were confirmed as malakoplakia of the prostate by means of prostatic biopsy are reported here. All of the patients had a previous history of urinary tract infection with Escherichia coli. These are cases that had not been found in the literature in Japan, and they are deemed the eighth, ninth, and tenth cases of prostatic malakoplakia reported in the world literature.