Cerebral amyloid angiopathy (CAA) accounts for approximately 10–20% of spontaneous intracerebral haemorrhage (ICH). This figure is thought to be higher in the elderly population. With the increasing life expectancy of our population, we anticipate that the prevalence of CAA- related ICH will increase in tandem. Although CAA-related ICH and hypertension-related ICH are distinct entities based on histopathology and imaging, the clinical presentation of the two conditions is similar. The use of brain computed tomography (CT) scans remain the ICH imaging modality of choice in Malaysia due to its availability, cost, and sensitivity in detecting acute bleeds. On the other hand, the use of brain magnetic resonance imaging (MRI) with susceptibility-weighted imaging (SWI) sequencing enables the clinician to determine the presence of chronic blood products in the brain, especially clinically silent microbleeds associated with CAA. However, the use of brain MRI scans in our country is limited and leads to a blurring of lines when differentiating between hypertension-related ICH and CAA-related ICH. How this misrepresentation affects the management of these conditions is unclear. In this study, we present two cases of ICH to illustrate this point and to serve as a springboard to question current practice and promote discussion.

Traumatic middle meningeal arteriovenous fistula with sphenoparietal sinus involvement is very rare. It is usually associated with skull fracture and intracranial hemorrhage. Ocular manifestation as sole presentation is extremely rare and only a few cases have been reported. We report a case of traumatic middle meningeal arteriovenous fistula with sphenoparietal sinus involvement 2 months after head trauma, presenting with left eye redness and dilated corkscrew episcleral vessel. CT scan showed venous out flow disturbance of the cavernous sinus with dilated ophthalmic veins. Transarterial endovascular embolisation of the fistula was done successfully; clinical and 6 months MRA resolution was noted. In conclusion, patient with traumatic middle meningeal arteriovenous fistula into the sphenoparietal sinus can present solely with subtle ocular symptoms. Transarterial endovascular embolisation is an effective procedure to close the fistula as seen in this case
Arteriovenous Fistula

A young man was admitted with sudden onset of right-sided weakness. He was assessed in the emergency department, and an immediate computed tomography (CT) perfusion study of the brain was arranged, which showed a left middle cerebral artery territory infarct with occlusion of the M1 segment. There was a significant penumbra measuring approximately 50% of the arterial territory. By the time his assessment was completed, it was 5.5 hours from the onset of symptoms. He was nonetheless administered intravenous recombinant tissue plasminogen activator (rtPA) based on the significant penumbra. He was discharged from the hospital after one week with significant residual deficit. At 2 months clinic follow-up, he showed almost complete recovery with a Modified Rankin Score of 1. We hope to demonstrate that a significant penumbra is an important determinant for good neurological recovery and outcome following stroke thrombolysis, even when patients present outside the 4.5 hours onset-to-treatment time window.

Brain ; pathology ; Demyelinating Diseases ; complications ; diagnosis ; pathology ; Encephalomyelitis ; complications ; diagnosis ; pathology ; Fatal Outcome ; Humans ; Leukoencephalitis, Acute Hemorrhagic ; complications ; diagnosis ; pathology ; Magnetic Resonance Imaging ; Male ; Young Adult

Background and Objective: Intravenous thrombolysis service for stroke was introduced at the Universiti Kebangsaan Malaysia Medical Centre (UKMMC) in 2009, based on the recommendations of a multidisciplinary team of clinicians. We report the experience at our center in establishing a stroke protocol incorporating computed tomography perfusion (CTP) of the brain, to assess the feasibility of incorporating CTP in the stroke protocol. Methods: A retrospective review of all patients who had a CTP between January 2010 and December 2011 was performed. Results: Of 272 patients who were admitted with acute ischemic stroke, 44 (16.2%) arrived within 4.5 hours from symptom onset and had a CTP performed with the intention to treat. The median time for symptom-to-door, symptom-to-scan and door-to-scan was 90.0 minutes (62.5 – 146.3), 211.0 minutes (165.5 – 273.5) and 85.0 minutes (48.0 – 144.8) respectively. Eight patients (2.9%) were thrombolysed of whom five received IV thrombolysis and three underwent mechanical thrombolysis. The median symptom-to-needle and door-to-needle times were 290.5 minutes (261.3 – 405.0) and 225.0 minutes (172.5 – 316.8) respectively. Four patients were thrombolysed despite being outside the window of treatment based on the CTP findings. Six of the thrombolysed patients had a Modified Rankin Score (MRS) of 1-2 at 5 months post procedure. Conclusions: CTP provides a benefit to management decisions and subsequent patient outcome. It is feasible to incorporate CTP as a standard imaging modality in a stroke protocol. The delays in the time-dependent pathways are due to our work flow and organisational process rather than performing the CTP per se.

Intravascular lymphoma (IVL) is a rare subtype of extranodal diffuse large cell lymphoma, characterized by intravascular proliferation of B or T lymphocytes within small blood vessels; which may lead to occlusive symptoms, its neurological involvement has been said to be uncommon among Asians.1 We describe a Malaysian with central nervous system IVL, to demonstrate that IVL is an important differential diagnosis in diffuse brain pathology also among Asians.

Labrune’s syndrome, or leukoencephalopathy with brain calcifications and cysts (LCC), is a rare genetic syndrome with variable neurological presentations. Psychiatric manifestations and involuntary movements are uncommonly reported. We report the case of a 19-year-old female, initially diagnosed with Fahr’s syndrome, who presented to us with acute psychosis, abnormal behavior and involuntary movements. Her brain computed tomography showed extensive bilateral intracranial calcifications without cysts. Genetic testing detected two compound heterozygous variants, NR_033294.1 n.*9C>T and n.24C>T, in the SNORD118 gene, confirming the diagnosis of LCC. We discuss the expanding phenotypic spectrum of LCC and provide a literature review on the current diagnosis and management of this rare syndrome.

Objective: The basal ganglia (BG) are susceptible to fluctuations in blood urea levels, sometimes resulting in movement disorders. We described patients with end-stage kidney disease (ESKD) presenting with movement disorders associated with bilateral BG lesions on imaging. Methods: We report four patients and systematically reviewed all published cases of ESKD presenting with movement disorders and bilateral BG lesions (EBSCOhost and Ovid). Results: Of the 72 patients identified, 55 (76.4%) were on regular dialysis. Parkinsonism was the most common movement disorder (n = 39; 54.2%), followed by chorea (n = 24; 33.3%). Diabetes mellitus (n = 51; 70.8%) and hypertension (n = 16; 22.2%) were the most common risk factors. Forty-three (59.7%) were of Asian ethnicity. Complete clinical resolution was reported in 17 (30.9%) patients, while 38 (69.1%) had incomplete clinical resolution with relapse. Complete radiological resolution occurred in 14 (34.1%) patients. Conclusion Movement disorders associated with BG lesions should be recognized as a rare and potentially reversible metabolic movement disorder in patients with ESKD.