Decompressive craniectomy (DC) is a surgical option in managing uncontrolled raised intracranial pressure refractory to medical therapy. The authors evaluate the addition of zygomatic arch (ZA) resection with standard DC and analyze the resulting increase in brain volume using three-dimensional volumetric CT scans. Measurements of brain expansion dimension morphometrics from CT images were also analyzed. Eighteen patients were selected and underwent DC with ZA resection. The pre- and post-operative CT images were analyzed for volume and dimensional changes. CT images of 29 patients previously operated on at the same center were retrieved from the picture archiving and communication system (PACS) and were similarly studied. The findings obtained from the two groups were compared and analyzed. Analysis from three-dimensional CT volumetric techniques revealed an significant increase of 27.97ml (95% confidence interval [CI]: 39.98-180.36; p=0.048) when compared with standard DC. Brain expansion analysis of maximum hemicraniectomy diameter revealed a mean difference of 0.82cm (95% CI: 0.25-1.38; p=0.006). Analysis of the ratio of maximum hemicraniectomy diameter to maximum anteroposterior diameter gave a mean difference of 0.04 (95% CI: 0.05-0.07; p=0.026). The addition of ZA resection to standard DC may prove valuable in terms of absolute brain volume gain. This technique is comparable to other maneuvers used to provide maximum brain expansion in the immediate post-operative period.
In neurosurgery and ear, nose and throat surgery the application of computerised navigation systems for guiding operations has been expanding rapidly. However, suitable models to train surgeons in using navigation systems are not yet available. We have developed a technique using an industrial, rapid prototyping process from which accurate spatial models of the cranium, its contents and pathology can be reproduced for teaching. We were able to register, validate and navigate using these models with common available navigation systems such as the Medtronic StealthStation S7®.
Recent studies associated the HLA-B 1502 allele with carbamazepine (CBZ)-induced Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) in patients from China, Thailand and Malaysia. No association has been found in patients from Europe or Japan. Linkage summary reports from East and South-east Asia predict a highly significant odds ratio (OR) of 84.75 (95% confidence interval [CI]=42.53-168.91; p=8.96×10[-15]) with sensitivity and negative predictive values of 92% and 98%, respectively. The higher prevalence of HLA-B 1502 allele among certain Asian populations (10-15%) compared to Caucasians (1-2%) may explain a 10-fold to 25-fold higher incidence of CBZ-SJS/TEN in patients from Asia. Screening for HLA-B 1502 before using CBZ can prevent SJS/TEN in certain populations, but screening may be less beneficial in populations with low HLA-B 1502 allele frequency and in patients exposed to CBZ for more than 2 months. A retrospective study demonstrated that the costs of HLA-B 1502 screening were less than those of SJS treatment. This article reviews possible benefits and concerns of HLA-B 1502 screening in clinical practice.
Paroxysmal kinesigenic dyskinesia is a rare disorder, and there are few reports of Asian patients with this condition. We reviewed the clinical features of all patients with idiopathic paroxysmal kinesigenic dyskinesia (PKD) seen at a major neurological centre in Malaysia. The charts of 11 patients with idiopathic PKD seen between 1995 and 2008 were reviewed retrospectively. The male:female ratio was 9:2. Ten patients were of Chinese ethnicity, and one was Malay. Three patients (from two families) had a family history of PKD. The involuntary movement was dystonia in 73% of patients. In one patient, attacks were precipitated by vestibular stimulation. One patient had generalized epilepsy. Another patient who did not have epilepsy demonstrated epileptiform discharges. Only slightly over one-quarter of patients had a positive family history. Males, and people of Chinese ancestry, seem to be affected more frequently by PKD in certain Asian populations.
A 24-year-old male patient with refractory Tourette syndrome was treated with deep brain stimulation (DBS) and developed subsequent bilateral subcortical haematomas. Additional blood tests revealed abnormalities of plasma factor XIIIA and tryptophan levels, which may be associated with Tourette syndrome. Neurosurgeons who perform DBS surgery on patients with Tourette syndrome must be aware of possible disastrous complications resulting from factor XIIIA disorders of blood haemostasis. Routine screening for this condition is not typically performed prior to surgery in these patients.
We studied the efficacy of two surgical methods used for the treatment of intracranial subdural empyema (ISDE) at our centre. A cross-sectional study (1999-2005) of 90 patients with non-traumatic supratentorial ISDE revealed that the two surgical methods used for empyema removal were burr hole/s and drainage (50 patients, 55.6%) and a cranial bone opening procedure (CBOP) (40 patients, 44.4%). Patients in the CBOP group had a better result in terms of clinical improvement (chi-squared analysis, p=0.006) and clearance of empyema on brain CT scan (chi-squared analysis, p<0.001). Reoperation was more frequent among patients who had undergone burr hole surgery (multiple logistic regression, p<0.001). The outcome and morbidity of ISDE survivors were not related to the surgical method used (p>0.05). The only factor that significantly affected the morbidity of ISDE was level of consciousness at the time of surgery (multiple logistic regression, p<0.001). We conclude that CBOP and evacuation of the empyema is a better surgical method for ISDE than burr hole/s and drainage. Wide cranial opening and empyema evacuation improves neurological status, gives better clearance of the empyema and reduces the need for reoperation. Level of consciousness at the time of presentation is a predictor of the morbidity of ISDE. Thus, aggressive surgical treatment should occur as early as possible, before the patient deteriorates.
We report on two patients with intra-operative rupture of cerebral aneurysms that were managed by microsuturing. This is one of only a few reports of successful direct repair using suturing. We found that stitching remains an option to repair a tear of a saccular part of an aneurysm and a torn neck of a blister-like aneurysm, and thus this technique can be considered before sacrificing the artery.
Despite technological advances in imaging, multiple sclerosis (MS) remains a clinical diagnosis that is supported, but not replaced, by laboratory or imaging findings. However, imaging is essential in the current diagnostic criteria of MS, for prediction of the likelihood of MS for patients with clinically isolated syndromes, correlation with lesion pathology and assessment of treatment outcome. This article gives an overview of imaging in MS with particular emphasis on the role of MRI in various diagnostic imaging criteria. Novel imaging for MS using 3 Tesla field strengths, magnetization transfer imaging, diffusion tensor imaging, magnetic resonance spectroscopy and cell-specific contrast will be reviewed.
Neurenteric cysts are rare congenital spinal masses that result from the dysgenesis of the endoderm tissue during development. We report a 4-year-old girl who presented with an insidious onset of lower limb paraparesis. An MRI scan revealed a cervicothoracic intradural extramedullary neurenteric cyst at the thoracic T1/T2 level, with marked spinal cord compression. No associated spinal dysraphism was noted. The patient underwent laminotomy and excision of the cyst. She recovered her neurological functions completely post-operatively, and at her six-month follow-up she was asymptomatic without any neurological deficits. We will discuss the pathogenesis, clinical presentation, and neuroradiological findings. We emphasize the value of early surgical intervention and long-term follow-up when this type of lesion is only partially excised.
Burkholderia pseudomallei infection of the central nervous system (CNS) is rare with less than 50 cases reported over the last 30 years. The retrospective melioidosis study at University Malaya Medical Centre has documented three cases of CNS melioidosis out of more than 160 cases of melioidosis since 1978. There were two patients with brain abscess and one with spinal epidural abscess. The predisposing factors were: one patient was an aboriginal farmer and the other two were diabetic. Their age ranged from 17 to 45 years. Prominent neurological features were limb weakness, cranial nerve palsy (6th and 7th) and visual disturbance. CT brain scan and MRI spine showed abscess formation, subdural collection, and spinal epidural collection, osteomyelitis of vertebra and occipital bone and also sagital sinus thrombosis. All these patients underwent surgical drainage leading to bacteriological diagnosis as well as appropriate long-term antibiotic therapy. All had good recovery at 6 months after completion of treatment.
Universiti Sains Malaysia is the only institution in Malaysia which incorporates all fields of the neurosciences under one roof. The integration of basic and clinical neurosciences has made it possible for this institution to become an excellent academic and research centre. This article describes the history, academic contributions and scientific progress of neurosciences at Universiti Sains Malaysia.
Minor head injury in a developing country like Malaysia is managed by primary care physicians and/or medical assistants in district hospitals. These patients are seen initially in their local hospitals, which have at least an X-ray machine for the purpose of screening. This study aimed to guide these physicians to manage these patients at a primary care level. A cross-sectional study was conducted and the study revealed significant predictors of significant computed tomographic (CT) findings. The presence of a Glasgow coma scale (GCS) score of 14 or 13 was associated with the risk of developing significant CT findings compared to patients with a GCS of 15 (p<0.001). Thirty-seven out of 50 patients with GCS of 14 or 13 developed clinically significant brain injury on CT scan. Similarly, the presence of skull fracture was associated with the risk of developing CT abnormalities (p<0.001). Forty-two out of 51 patients with skull fracture developed clinically significant CT findings. Vomiting was associated with developing CT abnormalities (p=0.017). Twenty-seven out of 40 patients with vomiting showed significant CT findings. Soft tissue injury was also found to be associated with developing CT abnormalities (p=0.007). Therefore, we propose reclassifying minor head injury based on the GCS score. Patients with a GCS score of 15 were classified as having mild head injury, while patients with a GCS score of 13 or 14 were at higher risk of developing brain injury and therefore categorized as high risk mild head injury. This group requires emergency CT scan examination, especially when associated with non-motor vehicle accidents, abnormal central nervous system (CNS) examination, craniofacial injuries or skull fractures. They should be referred to a general surgical unit which can treat head injuries or a neurosurgical tertiary centre.
We report a previously well 10-month-old Somalian girl who acquired asymmetric lower limb weakness in July 2013 in Mogadishu, Banadir, before arriving in Malaysia at 12 months of age. In May 2013, there was a wild poliomyelitis outbreak in that area, as reported by the World Health Organization. Laboratory investigation, including cerebrospinal fluid, was unremarkable, and electrophysiological studies showed active axonal denervation in the left lower limb. The whole spine T2-weighted MRI revealed non-enhancing hyperintensities of the bilateral anterior horn cells, predominantly on the left side at T11-12. The viral isolations from two stool specimens at her presentation to our centre, 2 months after the onset of illness and 2 weeks apart, were negative. Despite lacking the acute virological evidence of poliomyelitis, in view of the girl's clinical, electrophysiological and classical spinal neuroradiological features, together with her temporal relationship with a World Health Organization reported wild poliomyelitis outbreak, we believe these findings are consistent with a diagnosis of imported poliomyelitis. A review at 30 months of age showed persistent left lower limb monoplegia with little recovery. Our patient reiterates the importance of maintaining awareness of wild polio importation, and keeping abreast of the latest news of global poliomyelitis outbreaks when treating patients with flaccid paralysis, even if they arrive from non-endemic poliomyelitis areas.
A 24-year-old woman presented with a 3.5-year history of paroxysmal dystonia that was precipitated by sudden movement, especially when she started to walk. It was characterised by shrugging of shoulders, flexion of the neck and thoracic spine, and stiffness of the right leg followed by falls. Each attack lasted for less than 5min. Inadequate sleep and stress were exacerbating factors. There was no similar family history. Physical examination and investigations were normal. The following manoeuvres that caused vestibular stimulation precipitated attacks: turning her head from side to side while standing still, sitting still on a rotating chair and an ice-water caloric test. She had partial responses to phenytoin and levodopa, and a good response to haloperidol. Vestibular stimulation as a precipitating factor in paroxysmal kinesigenic choreoathetosis has not been reported previously.
We report two patients with myopathic dropped head syndrome, a rare and interesting neuromuscular syndrome characterised by a predominant weakness of the neck extensor muscles. The first patient, a middle aged Chinese man, presented with progressive weakness of neck extension but his clinical course later stabilised despite a lack of response to corticosteroids. Muscle biopsy revealed a necrotising myopathy with no evidence of inflammation. This patient supports the existence of an idiopathic restricted non-inflammatory myopathy, a so called isolated neck extensor myopathy syndrome which is recognised to pursue a less progressive, more benign course. Our second patient had histopathological evidence for polymyositis; there was a favourable response to steroids. Our cases underscore the fact that there may be a spectrum of pathological processes associated with the myopathic dropped head syndrome ranging from non-inflammatory muscle necrosis to a full blown inflammatory myositis.
Sixty patients with brain abscess were treated at the Neurosurgical Unit of the Department of Surgery, Hospital Universiti Sains Malaysia between January 1990 and December 1996. A retrospective study was done and data were collected from the computerise d registry of the Record Unit of Hospital Universiti Sains Malaysia. Good results were achieved in patients who were both treated surgically and medically. There were only twelve deaths in this group. The main factor that influences morbidity and mortality of brain abscess is the clinical presentation on admission. The mortality was high in patients treated solely by medical means. Death was common in patients who presented with acute onset of symptoms of less than one week duration and those with poor mental status. Brain abscess is common in the East Coast population of peninsular Malaysia, probably due in part to lower socioeconomic status. Efforts should be directed towards prevention of infection and early recognition and management.
Malaysia had the second highest crude accident rate in the world until 1998. Most children who were involved in these road traffic accidents required intensive neurosurgical care management. We report a prospective study on 36 paediatric neurotrauma patients in rural North East West Malaysia who underwent uniform intensive therapy and were subsequently followed up over a period of 2 years. The modified paediatric Glasgow Coma Scale with support of the revised Wechlser Intelligence Scale for children was used to test the outcome of these children over a period of two years. All patients were managed aggressively in our intensive care as well as our high dependency units. Our results indicate that improvement in outcome is seen after a six month period. Midline shift, duration of coma and duration of transport were found to be significant variables associated with bad outcome. Other variables i.e. age, sex, Glasgow Coma Scale on admission and on site, and lesions of the dominant lobe were not found to be associated with good outcome in these patients.
A study of clinical, computerised tomography (CT) profile and outcome was made of 128 patients, diagnosed to have diffuse axonal injury based on CT criteria. The mean age was 26 years. A lucid interval was present in 37 patients and hypoxia and/or hypotension was present in 43 patients. The depth of "marker lesions" in CT correlated with Glasgow Coma Score (GCS) (p<0.02) and duration of coma (p<0.01). The presence of associated intracranial haematoma >25 ml portended an adverse outcome. A logistic regression analysis revealed age, GCS, state of perimesencephalic cisterns and pupillary dilation to be independent predictors of poor outcome. Residual sequelae (focal deficits, cognitive deficits, postconcussion syndrome) were evident in nearly half of patients with diffuse axonal injury (DAI) and a lucid interval.