Type 1 intramedullary high SI on T2-weighted MR images indicates a better surgical outcome than does type 2.

Objective-To identify clinical characteristics of various forms of Guillain-Barré syndrome in Taiwan. Methods-The clinical and electrophysiological data of 167 consecutive patients with Guillain-Barré syndrome admitted to Chang Gung Memorial Hospital, a general paediatric and adult hospital in Taiwan, were reviewed. Results-Analysis of age distribution disclosed a high incidence (21%) among patients under the age of 10 years. Seasonal preponderance in Spring (March to May) was found. Utilising clinical and electrophysiological data, these 167 patients with Guillain-Barré syndrome were subclassified; 82 (49%) had acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 32 (19%) had Fisher syndrome (FS), and six (4%) had axonal forms of Guillain-Barré syndrome. The remaining 47 (28%) patients were unclassified. Patients with AIDP and FS had many common clinical features, including seasonal distribution, history of preceding illness, sensory abnormalities, cranial nerve involvement except for extraocular motor nerves, and albuminocytological dissociation on examination of CSF. Follow up study on 145 patients disclosed that 127 (87%) recovered satisfactorily, 14 (10%) were persistently disabled, and four (3%) died during admission to hospital. Clinical features associated with poor outcome (persistent disability or death) were requirement for mechanical ventilation, a low mean compound muscle action potential amplitude (< 10% of the lower limit of normal), and age greater than 40 years. Conclusion-Guillain-Barré syndrome in Taiwan showed a peculiar age and seasonal distribution and a high frequency of FS not seen in other series. Given that patients with AIDP and FS had many common clinical features, AIDP and FS may have similar underlying pathological mechanisms.

Bacterial meningitis, a world-wide disease, has to be reviewed periodically because the specific micro-organisms responsible for the infection vary with time, geography and patient age. To determine its incidence and clinical features in Taiwan, we reviewed the microbiological records for cerebrospinal fluid (CSF) and blood cultures, and the medical records of patients with bacterial meningitis admitted between 1981 and mid-1998. Bacterial micro-organisms were demonstrated in the CSF and/or blood in 395 patients with 418 episodes of bacterial meningitis. Streptococcus species were the most common causative micro-organism group, at 23. 21% of all episodes. Its prevalence rate significantly decreased from the first 7 years of study (41.9%) to the last 10.5 years (19. 2%). However, Klebsiella meningitis and Staphylococcal meningitis were more frequently noted after 1987. More than 70% of patients had at least one underlying disease or condition. Poor prognostic factors indicated by univariable analysis were: age >60 years; diabetes mellitus; severe neurological deficits on the first day of treatment; infection with Gram-negative bacilli; CSF WBC count >5000x10(6)/l; malignancy; seizure; and bacteraemia. The overall mortality rate was 29.4%, 29.7% in the first 7 years of study and 29. 4% in the last 10.5 years. The use of new antibiotics has not reduced the mortality rate in our patients with bacterial meningitis.

The purpose of this study was to review the clinical manifestations of 40 patients who fulfilled the clinical criteria for Hirayama disease (juvenile muscular atrophy of distal upper extremities), identified in our neuromuscular clinic between February 1995 and December 2005. Of these 40 patients, 87.5% were male. The mean age at onset was 16.8 years, which was 4.5 years later than the peak age of the normal growth curve. Progressive muscle weakness and wasting were characteristic and occurred predominantly in the distal part of the right upper limb. Neurogenic symptoms ceased to progress within 5 years in most patients (92.5%). About one third of patients had participated frequently in heavy physical activity before onset of muscle symptoms. Reduced amplitude of the compound muscle action potential of the ulnar nerve was the most prominent finding in nerve conduction studies. Electromyography showed acute or chronic neurogenic changes, most frequently in muscles supplied by the C7-T1 segments. Magnetic resonance imaging showed anterior shifting of the posterior dura and engorged posterior venous plexus at the cervical level in 95% of patients. Our results support the belief that Hirayama disease is a self-limited, focal lower motor neuron disease involving the lower cervical segments. Disproportionate growth between the vertebral column and the contents of the spinal canal may be the underlying cause, and strenuous physical activity may be a precipitating factor.