Creutzfeldt-Jakob disease with initial right hemiparesis masquerading as a stroke.

The early stage of sporadic Creutzfeldt-Jakob disease is generally characterised by progressive changes in behaviour and intellectual function. While only a few patients have stroke-like onset, Creutzfeldt-Jakob disease with initial monoparesis has been described. In this report, a patient with an unusual sporadic Creutzfeldt-Jakob disease with typical magnetic resonance imaging findings, positive cerebrospinal fluid 14-3-3 brain protein, sharp-wave complexes in electroencephalogram, and initial right hemiparesis is reported.

The contribution of a comprehensive stroke unit to the outcome of Chinese stroke patients.

INTRODUCTION: There are significant differences in stroke patterns and risk factors for cerebrovascular disease between Chinese subjects and Caucasians. The outcome of stroke unit care in a Chinese population has not been described in the medical literature. The present study aims to evaluate the outcome of stroke unit care in Chinese subjects. METHODS: By prospective comparative research, Chinese patients treated in the stroke unit were group-matched with those treated in the general medical ward by age, gender, premorbid functional status (by the Barthel Index), National Institute of Health Stroke Scale score, and stroke types. From April 2001 to April 2002, a total of 188 patients in the stroke unit group and 177 patients in the general ward group were recruited in the study. The main outcome measures included mortality at 28 and 120 days, and the length of inpatient stay. RESULTS: Stroke unit care significantly reduced mortality of patients with acute stroke after 28 and 120 days. After 28 days, mortality was 3.3 percent and 17.2 percent for the stroke unit group and general ward group, respectively (p-value is equal to or less than 0.01); whereas after 120 days, mortality was 5.0 percent and 24.7 percent for the stroke unit group and general ward group, respectively (p-value is equal to or less than 0.001). The stroke unit care was demonstrated, by logistic regression analysis, to have contributed to the reduction of mortality at 120 days (p-value is 0.014). At 28 days, there was only a trend for stroke unit care to contribute to the reduction of mortality by logistic regression analysis (p-value is 0.067). By Kaplan-Meier survival curves (log rank statistic is 10.46, p-value is 0.001) and a Cox regression (hazard ratio 0.253, 95 percent confidence interval 0.085 to 0.754, p-value is 0.014), the stroke unit care was further found to reduce mortality significantly. The mean length of inpatient stay of the stroke unit group was 37.1 days, while that of the general medical ward group was 69.3 days (p-value is equal to or less than 0.001). CONCLUSION: Chinese subjects receiving comprehensive stroke unit care are associated with less mortality and shorter length of hospital stay than those having conventional care in general medical wards.

A case of isolated pure trigeminal motor neuropathy.

Isolated trigeminal neuropathy after cranial trauma is exceptional. A 40-year-old woman was found to have unilateral trigeminal motor paralysis with muscle atrophy, unaccompanied by sensory involvement and without affecting other cranial nerves. She developed this deficit immediately after head trauma.

Computed tomography of the superior mesenteric artery syndrome.

An acute presentation of the superior mesenteric artery syndrome was diagnosed in a 13-year-old Chinese boy in whom no predisposing cause was found at surgery. Duodenogram combined with dynamic computed tomography, including 2-dimensional multiplanar reconstructions, was useful in confirming the diagnosis prior to definitive surgery.

Treatment of dystonia with botulinum A toxin: a retrospective study of 170 patients.

OBJECTIVE: Botulinum A toxin has been reported to provide excellent symptomatic relief for patients with dystonia. To analyse the treatment, complications, and outcome of patients receiving botulinum A toxin injection, the case records of 170 patients attending the Botox Clinic at the Kwong Wah Hospital from 1 December 1992 to 31 December 1996 were reviewed. Of these 170 patients, 130 (76.5%) had idiopathic hemifacial spasm, 18 (10.6%) had blepharospasm, 18 (10.6%) had spasmodic torticollis, and 4 (2.4%) had generalised or focal limb dystonia. One hundred and sixty-six (97.6%) patients were Chinese. The average dose of botulinum A toxin required for an optimal response was 14.54 U for those with hemifacial spasm, 49.64 U for those blepharospasm, and 137 U for those with spasmodic torticollis. Among patients with hemifacial spasm, 103 (81.7%) gave a good response, 21 (16.7%) gave a partial response, and there was no response in two (1.6%) patients. The corresponding figures for patients with blepharospasm were 7 (38.9%), 10 (55.6%), and 1 (5.6%), respectively, and for those with spasmodic torticollis, the figure were 6 (37.5%), 6 (37.5%), and 4 (2.5%), respectively. Complications from botulinum A toxin injection were rare (less than 10%), minor, transient, and usually dose-related. In conclusion, idiopathic hemifacial spasm was the most common type of movement disorder encountered in our Botox Clinic and botulinum A toxin injection was safe and effective in the majority of patients.

Cerebrotendinous xanthomatosis in three siblings from a Chinese family.

Cerebrotendinous xanthomatosis (CTX) is exceptionally rare in the Chinese population. We reported a 31-year-old Chinese male in Hong Kong, who has the characteristic features of cerebrotendinous xanthomatosis including the multiple xanthomas of tendons, mental retardation, bilateral cataracts, cerebellar ataxia and spasticity of the left arm, high concentrations of plasma phytosterols and abnormal MR of brain. On screening the family, two other siblings of 27 and 29 respectively, have tendon xanthomas and high plasma phytosterols. An extensive search of the international medical literature, including the Medline, has revealed only one other case report of cerebrotendinous xanthomatosis in Taiwan. CTX is a potentially treatable disease. It is hoped by alertness, early diagnosis and treatment can be made, and hence prevent further progression of the disease.

Isolated hypoglossal nerve palsy in Behçet's disease.

Isolated hypoglossal nerve paresis as a manifestation of Behcet's disease has not been reported. We describe a 42-year-old man with isolated unilateral hypoglossal nerve paresis, who had had a five-year history of recurrent orogenital aphthae and relapsing arthritic manifestations. It is suggested that the nerve disorder represents a form of mononeuritis that may occur in Behcet's disease.

The role of evoked potential and MR imaging in assessing multiple sclerosis: a comparative study.

INTRODUCTION: The role of evoked potential (EP) in evaluating multiple sclerosis (MS) has changed with the advent of magnetic resonance (MR) imaging. Given the possibly varied nature and distribution of pathologic lesions in Asian MS, this study compared the diagnostic usefulness of EPs to that of MR imaging among Chinese subjects. METHODS: This was a retrospective study of MS patients treated at the Kwong Wah Hospital, Hong Kong, between June 2004 and June 2009. The visual (VEPs), brainstem auditory (BAEPs), somatosensory (SEPs) and trigeminal (TSEPs) EPs were compared with MR imaging for correlation and usefulness using the chi-square test. Sensitivities and specificities were calculated. RESULTS: The results showed that abnormalities were detected in the three modalities of EP among the 17 patients studied (VEP 82 percent, median and tibial SEP 65 percent, BAEP 47 percent). Compared with MR imaging, VEP was far more useful at detecting optic nerve lesions, while SEP was less sensitive at detecting cord lesions. BAEP was able to localise lesions along the auditory pathways at a rate that was almost similar to that of MR imaging (non-inferiority). Both TSEP and MR imaging for trigeminal nerves were negative in the two patients with trigeminal neuralgia. In some instances, EPs yielded abnormalities that were undetected by conventional MR imaging, and the sensitivity increased with the number of EP modalities. CONCLUSION: EP may be considered in clinical situations in which MR imaging is negative or cannot be performed. They may also be performed when evaluating treatment response, long-term prognosis and nonspecific changes on MR imaging.