Objective: To report on the incidence, and the clinical and laboratory features of patients seen at the University of Malaya Medical Centre with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Methods: The charts of all patients admitted to the adult neurology ward with encephalitis over an 18- month period from January 2010 to June 2011 were reviewed. Diagnosis of anti-NMDAR encephalitis was based on the presence of encephalitis plus antibody against the NMDAR. Two other paediatric patients with anti-NMDAR encephalitis seen over the same period were also included in this report. Results: There was a total of 10 patients with anti-NMDAR encephalitis seen over the study period. The mean age was 18.1 years (range 9-29 years). Eight patients were female, two male. Five were Malay and fi ve were Chinese. All patients had prominent psychiatric symptoms, followed by epileptic seizures. Nine patients had a movement disorder, orofacial dyskinesia being the commonest, and all had autonomic involvement. None had an underlying tumour. Treatments consisted of corticosteroid, plasma exchange and intravenous immunoglobulin (IVIG). The clinical outcome was variable, with full recovery (2), substantial recovery (3), partial recovery (4), and mortality (1) seen. Remarkably, the eight adult cases of anti-NMDAR encephalitis accounted for 50% of the 16 cases of encephalitis seen during the study period. Conclusion: Anti-NMDAR encephalitis may be a relatively common cause of adult encephalitis among certain Asian groups. None of our cases was paraneoplastic in origin.

Accidental carbon monoxide poisoning in countries with cold climates is commonly related to indoor heating. This condition appears to be relatively uncommon in tropical Asian countries and therefore the diagnosis may be unsuspected. We report a case of a Malaysian patient who presented with a severe, and ultimately fatal, delayed (biphasic) neuropsychiatric syndrome due to carbon monoxide poisoning. The diagnosis was made only when a history compatible with carbon monoxide poisoning subsequently surfaced, and neuroimaging demonstrated the typical pallidal lesions, associated with marked leukoencephalopathy, seen in this condition. Our case is unique because the poisoning occurred in the setting of indoor operation of a portable electricity generator in a karaoke centre because of power failure. Karaoke is a highly popular form of entertainment in many parts of Asia and we suggest that a high index of suspicion of carbon monoxide poisoning is required in this setting.

There are 50-100 million dengue infections each year, but dengue encephalitis is relatively uncommon. The aetiology of neuronal injury is proposed to be due to direct viral neurotropism or host immune response-mediated inflammation causing neuronal damage. We report a case of severe dengue encephalitis, presenting during the acute viraemic phase of the disease. This was associated with inflammation and haemorrhage of the internal medullary lamina of both thalami which, to our knowledge, has not yet been reported in other infections of the central nervous system.
Dengue

Purposeless groaning has been reported in advanced progressive supranuclear palsy. We present a case of purposeless groaning occurring as a primary complaint in a patient with advanced Parkinson's disease. Purposeless groaning is thought to be a manifestation of disinhibition and perseveration due to frontal-subcortical dysfunction. Proper recognition of this phenomenon will help clinicians to avoid unnecessary investigations and treatment (e.g., prescription of opioid medications).
Humans ; Parkinson Disease ; Prescriptions ; Supranuclear Palsy, Progressive

Ayurveda is a traditional medical system used widely in India and increasingly worldwide. Here, we report on a patient with Parkinson’s disease (PD) who developed marked striatal hand with antiparkinsonian medication withdrawal during Ayurvedic medicine treatment for her PD. Although a direct role for the Ayurvedic medicines in inducing the hand deformity cannot be excluded, we propose that severe dopaminergic defi ciency (due to prolonged withdrawal of antiparkinsonian medications in the context of long-standing PD) was probably the main culprit in our patient.

Thrombocytopenia is a common laboratory finding in dengue infection. However, it usually resolves as the patient recovers from the infection. Persistent thrombocytopenia following dengue infection requires further investigation. Here, we present a case of immune thrombocytopenic purpura (ITP) following dengue infection complicated by intracranial bleeding.

Hyponatraemia with rapid correction of serum sodium may cause an osmotic demyelination syndrome (ODS) with damage to pontine and/or extrapontine areas of the brain. The prognosis of ODS can range from complete recovery to death; at present, our ability to predict clinical outcome is very limited. We describe here a patient with ODS and increased signal intensity in the striatum on diffusion-weighted MRI, with corresponding low apparent diffusion coeffi cient values (indicating restricted water diffusion). This case provides a further example of the typical MRI appearance of extrapontine ODS and suggests the potential value of diffusion-weighted MRI in predicting prognosis in ODS.

No abstract available.
Neuroimaging

Background: Mutations in glucocerebrosidase (GBA) have been associated with the risk of developing Parkinson’s disease (PD) in different ethnic populations. The prevalence of GBA mutations among Malay PD patients is unknown. Thus, the aim of this study was to determine the frequency of GBA mutations among Malay PD patients, focusing on early (EOPD) and late-onset (LOPD) patients. Methods:EOPD (n = 50) and LOPD (n = 50) patients along with 50 ethnically and age-matched control wererecruited. The GBA exons of these patients were sequenced using the Ion Torrent PGMTM System. Results: Five heterozygous mutations exclusive to EOPD patients were identified; c.-203A>G,p.S146L, p.R159Q, p.L483P and p.L483R+c.-145G>A. In LOPD patients, c.543C>T(p.(F181=)), c.28-10C>A and p.R202Q were identified in which this p.R202Q was also present in a control subject. In addition, c.259C>A(p.(R87=)) and c.-145G>A were identified in two control subjects. In summary, we observed GBA mutations in 8% and 6% of Malay PD cases and control subject, respectively. The prevalence of GBA mutations was higher in EOPD (10%) than LOPD (6%). However, these differences were not statistically significant; [PD vs. controls: OR = 1.36, 95%CI 0.35-5.38, p = 0.752] and [EOPD vs. LOPD: OR = 1.74, 95%CI 0.39-7.71, p = 0.715]. Conclusion: We identified five exclusive heterozygous GBA mutations in EOPD patients which might predict the increase susceptibility of Malays to develop PD at young age. These findings could add knowledge into the existing evidences linking genetic alterations in GBA and PD.