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Neck clipping of basilar trunk aneurysms, particularly those of a large size, is challenging because of its location. Here, we report a case of a basilar artery aneurysm successfully treated with neck clipping using rapid ventricular pacing(RVP). A 67-year-old woman was referred to our hospital for treatment of a large basilar artery aneurysm. Although coiling was considered, we performed neck clipping of this aneurysm because of the expected radical therapeutic effect. The patient was positioned in the right park-bench position, and right suboccipital craniotomy was performed. The aneurysm was mainly approached via the right supracerebellar route. RVP softened the aneurysm for easy dissection and insertion of multiple clips. The postoperative course was uneventful, and she was discharged 1 week later without neurological deficits. RVP should be considered for the treatment of complex aneurysms as adjunctive techniques.

A 43-year-old man with no past history of underlying disease was admitted to an affiliated hospital, complaining of high fever and severe headache. Polynuclear dominant pleocytosis in the cerebrospinal fluid (CSF) suggested bacterial meningitis. He was immediately treated with several antibiotics, then his clinical symptoms improved, although intractable headache relapsed after withdrawal of the initial therapy. Thereafter he consulted our hospital for a second opinion. CSF examination in our hospital demonstrated mononuclear dominant pleocytosis with normal sugar value. However, bacterial culture of the CSF specimen yielded a gram-negative bacillus that was finally identified as a Campylobacter fetus on Skirrow's culture. Treatment with meropenem and other antibiotics improved both the clinical symptoms and CSF findings. Thus, meningitis with a Campylobacter fetus could manifest a chronic and atypical clinical course, careful attention should be paid to establish an early diagnosis.

Background: Pergolide mesilate is widely used to treat Parkinson's disease in both the USA and Japan, but the maintenance dose is distinctly different between the USA (usually more than 1.5 mg/day) and Japan (usually less than 1.5 mg/day). Although several reports from the USA have suggested that mitral, aortic, and tricuspid valvular lesions were caused by pergolide, it is unclear whether low-dose pergolide therapy causes such valvular lesions., Objectives: The effects of low-dose pergolide therapy on cardiac valves were studied in Japanese patients with Parkinson's disease., Methods: One hundred and five consecutive patients with Parkinson's disease approved for our protocol were enrolled in this study. Forty patients were treated with low-dose pergolide (0.05-1.5 mg/day for 2-115 months), and were included in the pergolide group (mean age 71 +/- 6 years). The other 44 patients received no ergot-derived dopamine receptor agonists, and 32 patients acted as age-matched controls (mean age 71 +/- 7 years). Both groups of patients underwent echocardiographic examination to detect organic lesions in cardiac valves such as thickening of the leaflet, annular calcification, restriction of valve motion and valvular tenting, and valvular regurgitation greater than 2 + on the 4-point scale., Results: No significant difference was observed in the incidence of aortic, mitral and pulmonic valve lesions between the pergolide group and the control group. Although no organic lesions were detected in the tricuspid valve, the incidence of tricuspid regurgitation was significantly higher in the pergolide group than in the control group (p < 0.05)., Conclusions: Although low-dose pergolide of less than 1.5 mg/day does not cause serious damage in the left-sided valves, it may induce tricuspid regurgitation.

A 49-year-old woman with seronegative myasthenia gravis (SNMG) was admitted to our hospital with severe respiratory failure, proximal muscle weakness and bulbar palsy. Permanent tracheostomy and continuous mechanical ventilation were performed. At a previous hospital, she was diagnosed as SNMG on the basis of the positive waning during 3 Hz repetitive stimulation of the ulnar nerve, although no acetylcholine receptor antibodies (Ab) were detected by serological examination. Before admission to our hospital, she was treated with corticosteroids, intravenous immunoglobulin and tryptophan column immuno-adsorption therapy without clinical improvement. At our hospital, serological examination detected muscle-specific receptor tyrosine kinase (MuSK) Ab and plasma exchange was performed as treatment. Plasma exchange and subsequent immunomodulating therapy with corticosteroids and tacrolimus showed a dramatic clinical improvement with a marked decline of MuSK Ab level in the serum. These results suggested that plasma exchange should be considered as first choice to treat patients with refractory MuSK Ab-positive MG.

A patient with Facial diplegia and paresthesias, a rare regional variant of Guillain-Barré syndrome (GBS), is described. A 37-year-old woman developed paresthesias in the distal limbs and subsequently bifacial weakness. She had had a preceding episode of laryngitis. Neurological examination showed severe facial diplegia with loss of taste sensation on the tip of the tongue. Limb muscle power was preserved. Deep tendon reflexes were generally absent. She complained of paresthesias in the distal limbs, but sensory examination was normal. Cerebrospinal fluid showed albuminocytological dissociation. Electrophysiological studies revealed severe facial nerve involvements and demyelinative findings in her limbs. Intravenous immunoglobulin rapidly improved the abnormal sensation in her limbs. Although facial diplegia gradually lessened after the therapy, mild residual weakness was noted 6 months after the neurological onset. To diagnose facial diplegia and paresthesias, it is important to clarify the findings common to typical GBS such as the antecedent illness, acute and monophasic course, distal paresthesias, areflexia, cerebrospinal fluid albuminocytological dissociation, and demyelinative conduction abnormalities in the limbs.

A 65-year-old man with previous history of congestive heart failure and genetically proven Becker muscular dystrophy (BMD) was suddenly suffered from aphasia and right hemiplegia. Physical examination showed severe motor aphasia, right hemiplegia, and signs of left heart failure. An echocardiogram before the onset of aphasia showed markedly dilated left ventricle and decreased ventricular contraction. Intracardiac thrombus was not detected. Although his electrocardiogram on admission showed sinus rhythm, atrial fibrillation was noted at the time of neurological deterioration. MRI of the brain revealed acute infarction in the territory of the left middle cerebral artery and the left anterior inferior cerebellar artery. MR angiography showed vascular occlusion at the left M2 segment. Cerebral embolism due to atrial fibrillation associated with BMD-related DCM was diagnosed. While an administration of anti-coagulant, diuretics, and dopamine relieved his respiratory distress and right hemiplegia, severe motor aphasia persisted. Cerebral embolism may be a notable complication in patients with BMD presenting with late-life expression of skeletal muscular weakness and antecedent cardiac involvement.

A 65-year-old male was admitted with the chief complaint of voiding difficulty on 28 October, 2001. He was in the state of urinary retention. Urological investigation including cystosocopy, urethrocystography, and urodynamic study revealed an areflex-type bladder, according to the new International Continence Society (ICS) classification with no prostatic urethral obstruction. He was diagnosed with neurogenic bladder although the cause of detrusor areflex was unknown. Because all medication was ineffective, transurethral resection of prostate was performed on 11 January, 2002, but urinary retention could not be relieved. After operation, he complained of muscle weakness of upper extremities and poor control of bowels. He was diagnosed with Lambert-Eaton myasthenic syndrome associated with small cell lung carcinoma. His myasthenic symptoms, including autonomic symptoms were relieved after chemotherapy and radiation for small cell lung carcinoma.

We described a patient with ataxic Guillain-Barré syndrome who subsequently developed facial diplegia. A 38-year-old man developed ataxia, distal limb paresthesias, mild dysphagia, urinary retention and orthostatic hypotension a week after an episode of laryngitis. He had high titers of serum anti-GQ1b, anti-GD1b, anti-GM1b, anti-GT1a, and anti-GD1a IgG antibodies during the acute phase. Although the initial symptoms markedly improved by intravenous immunoglobulin therapy, asymmetric facial diplegia subsequently occurred and remained longer than ataxia. Similar course of facial nerve palsy has been reported in patients with Fisher syndrome. Common pathophysiological mechanism may function in the development of delayed facial diplegia in Fisher syndrome and ataxic Guillain-Barre syndrome.

In June 2000, there was a large-scale outbreak of food poisoning after consumption of Snow Brand low fat milk. In the evening of a day the incident made public, some cartons of low fat milk were brought to our laboratory for examination. Next day, we detected only staphylococcal enterotoxin (SE) A gene among SE (A-E) genes by PCR in left-over milk samples or samples from the same lots that patients had consumed. We presumed that the outbreak was caused by the intake of SEA. We subsequently confirmed the presence of SEA in these samples. To investigate the existence of SE (A-E) genes in milk, we examined 100 samples of commercial low fat milk and milk by PCR, but none of the genes was detected. We estimated the detection limit of SEA gene in low fat milk by PCR. Four strains of SEA-producing Staphylococcus aureus cultures were serially diluted in low fat milk. The SEA gene was detected at levels of 5.5 x 10(2) to 1.6 x 10(4) cfu/mL of S. aureus. These amounts of S. aureus are higher than the values in raw milk reported previously. Therefore we consider that SE genes in low fat milk should usually be undetectable by our PCR. This study shows that quick detection of SE genes by PCR is very helpful to analyze outbreaks, especially if no significant bacterium can be cultured.

A 57-year-old woman developed muscular stiffness and painful cramps, which were relieved by administration of dantrolene sodium. Her serum level of antibodies to glutamic acid decarboxylase (GAD) was markedly elevated and continuous muscular activities were observed on resting surface EMG. These features were compatible with those in stiff-person syndrome (SPS). She was found to have thymoma on CT scan. Immediately after thymomectomy, which was histologically diagnosed as a benign hyperplasia, she developed head retraction reflex-like movements evoked by sensory stimulation to the face, which were followed by severe bulbar symptoms with dysphagia and respiratory arrest. Postoperative myasthenia gravis was excluded clinically. While somatosensory evoked EMG on splenius muscle initially showed biphasic responses with latency of 15 msec and 55 msec, respectively after oral angle non-painful electric stimulation, the late potential phase disappeared after the patient recovered from bulbar symptoms. This suggests that head retraction reflex-like movements of this patient reflected the attenuation of inhibitory potentials from the brainstem.

We report a 34-year-old woman with linear sebaceous nevus syndrome and dolichomegalic artery. The patient was admitted to our hospital for evaluation of a headache and fever. Neurological examination revealed no focal sign except neck stiffness. She had had sebaceous nevi on the left side of her head, cheek and neck since she was born. A cranial CT scan showed high signal intensity in the subarachnoid space. Cranial MRI showed a dolichomegalic artery. The patient's past history included many episodes of headache and fever since her first decade of life and she had been diagnosed five times with cerebrovascular disease. At the present admission, subarachnoid hemorrhage was diagnosed and treated. The patient improved and was discharged on the 21st day. Linear sebaceous nevi are associated with many types of anomalies, but we found no other reported cases of linear sebaceous nevus syndrome associated with a cerebrovascular anomaly. This case suggests that a patient with sebaceous nevi who presents with headache and fever should be examined with careful attention to the cerebrovascular system.

We report a patient who had generalized painful muscle cramps associated with isolated ACTH deficiency. A 68-year-old woman was hospitalized because of painful muscle cramps present for one year. Neurological examination revealed no abnormalities except for generalized painful muscle cramps. Serum electrolyte and CPK levels were normal. Serum ACTH and cortisol levels as well as urine 17-OHCS were low. An ACTH loading test employing insulin, TRH and LH-RH indicated isolated ACTH deficiency. Just after the muscle cramp, EMG revealed a low amplitude in the biceps muscle. Colon biopsy showed mild fibrosis and inflammatory cell infiltration in the lamina propria. Her muscle cramps improved markedly after two weeks of hydrocortisone replacement therapy and resolved after three weeks, suggesting that this symptom was closely related to isolated ACTH deficiency. Our case suggests that isolated ACTH deficiency may present with very similar clinical symptoms to Satoyoshi disease.

The present study was performed to examine the pyramidal tracts of the brain in both 51 normal subjects (21 male and 30 female subjects; mean age of 43.5 +/- 16.1 years) and 12 patients with motor neuron disease (6 male and 6 female patients; mean age of 57.4 +/- 7.9 years), using the magnetic resonance imaging (MRI). The 12 patients with motor neuron disease (MND) comprised 7 suffering from spinal progressive muscular atrophy (SPMA) and 5 from amyotrophic lateral sclerosis (ALS). The MRI used in this study was of both short spin echo and long spin echo sequence. Of the 52 normal subjects, 24 of them (47%) had the T2 prolonged small areas (high signal intensity areas) at the posterior limb of internal capsule. These findings were not found in the normal subjects over fifty years old. No similar finding was detected in the pyramidal tracts except the posterior limb of internal capsule. On the other hand, 8 patients with MND (67%) proved to have the high signal intensity areas in the pyramidal tracts. Moreover, these high intensity areas were extended from the crus cerebri to corona radiata in 7 patients (58%). In all patients with ALS, these areas were extended in whole areas of the pyramidal tracts, and the similar findings were also found in two patients with SPMA. These findings were demonstrated to be more extensive than those in the normal subjects. The results thus obtained warrant us to conclude that cranial MRI is useful to detect the degeneration of the pyramidal tracts of MND patients.