R Lee

Cerebral infarcts complicating tuberculous meningitis.

Cerebral infarction (CI) is a serious complication of tuberculous meningitis (TBM). It can be asymptomatic or symptomatic, causing stroke. We studied 40 TBM patients. All had initial CT brain scan, CT/MRI brain scan 3 months later and urgent CT brain scan for deterioration. CI was classified into lacunar infarction (LI) or large artery infarction (LAI). Twelve (30%) had CI, in 9 (23%) it was symptomatic and in 3 (8%) silent. Seven (58%) had LAI ± LI. Eight (67%) had multiple CI. Two died from brainstem CI and 6 were dependent at 1 year. Patients with LAI might develop posterior circulation CI more frequently than those with LI only. CI is a common complication of TBM locally, with LAI and multiple CI being common. Two thirds of TBM patients complicated by CI had poor prognosis despite adjunctive dexamethasone treatment.

Abnormal diffusion tensor in nonsymptomatic familial amyotrophic lateral sclerosis with a causative superoxide dismutase 1 mutation

To determine whether diffusion abnormalities can be observed in nonsymptomatic family members with a known causative Cu/Zn superoxide dismutase mutation (asymptomatic familial amyotrophic lateral sclerosis; AFALS+SOD1) in a family with autosomal dominant familial amyotrophic lateral sclerosis (ALS) using diffusion tensor imaging (DTI).

Neuromyelitis optica-IgG in idiopathic inflammatory demyelinating disorders amongst Hong Kong Chinese

Background:  Idiopathic inflammatory demyelinating disorders (IIDD) affect the central nervous system. In classical multiple sclerosis (CMS), brain, optic nerves [optic neuritis (ON)] and spinal cord [acute transverse myelitis (ATM)] are affected. In neuromyelitis optica (NMO), optic nerves and spinal cord are predominantly affected. NMO‐IgG, an autoantibody targeting aquaporin‐4, is a marker for NMO. We studied the frequency and clinical relevance of NMO‐IgG seropositivity in IIDD patients.

Warfarin associated intracerebral hemorrhage in Hong Kong Chinese

Abstract Objectives: Warfarin-associated intracerebral hemorrhage (WICH) is a serious neurological condition associated with significant mortality and morbidity. We aimed to study the clinical features and factors that predict clinical outcome of Chinese patients with WICH. Methods: Medical records of patients with spontaneous intracerebral hemorrhage (ICH) admitted to our hospital between July 2001 and June 2010 were reviewed and those with WICH were studied in detail retrospectively. Results: Fifty-one patients with WICH were studied. The mean age was 74·3 ± 10·5 years and 52·9% of the patients were female. The mean international normalized ratio (INR) on presentation was 2·9 ± 1·0. The median ICH volume was 23·3 (10·4–59·3) ml. The mortality rate at 3–6 months for WICH was 62·0%. Multivariate logistic analysis revealed that an initial ICH volume of > 20 ml (OR 34·4, P  =  0·037) and presence of intraventricular hemorrhage (OR 22·9, P  =  0·046) were independently associated with poor outcome. Supratherapeutic INR (INR > 3·0) on admission (P  =  0·724) and complete correction of INR within 24 hours after admission (P  =  0·486) were not independent predictors of poor outcome. The median ICH volumes did not differ between INR groups (18·2 (9·4–61·1) ml for INR ≤ 3 vs 27·3 (13·7–58·5) ml for INR > 3, P  =  0·718). Neurological deterioration (ND) was documented in 19 (63·3%) of the 30 patients included in a smaller sub-cohort, and was associated with poor neurological outcome (OR 20·7, P  =  0·027). Warfarin was resumed in 7 of the 20 survivors. There were two episodes of recurrent WICH and one episode of ischemic stroke during a mean follow-up duration of 5·4 years. In survivors who were not resumed on warfarin, there were two episodes of recurrent ICH and 12 episodes of ischemic vascular events (nine ischemic strokes) during a mean follow-up duration of 2·6 years. Conclusion: Warfarin-associated intracerebral hemorrhage is a very serious complication of warfarin therapy with high mortality and morbidity. Initial ICH volume, presence of intraventricular hemorrhage, and ND are independent predictors of clinical outcome.

Central nervous system inflammatory demyelinating disorders among Hong Kong Chinese

Classical multiple sclerosis (CMS) and neuromyelitis optica spectrum disorders (NMOSD) are distinct central nervous system inflammatory demyelinating disorders (CNS IDD). Early diagnosis of CNS IDD is important as appropriate immunotherapies to optimize prognosis. We studied the diagnoses of CNS IDD among Hong Kong Chinese in a hospital-based setting. Consecutive Chinese patients who presented to our hospital with clinically isolated syndrome and subsequently diagnosed to have CNS IDD from 1980 to 2010 were reviewed. Patients with known diagnosis of CNS IDD referred for further care were excluded. Serial sera were assayed for aquaporin-4 autoantibodies (AQP4 Ab), at least 3 assays within 2-5years. A total of 210 patients diagnosed to have CNS IDD with disease duration of at least 2years were studied. Among 198 patients with serial sera available, 40 (20.2%, 20 had NMO and 20 other NMOSD) were AQP4 Ab-positive. Four patients who were AQP4 Ab-negative on the initial assay converted to AQP4 Ab-positive on repeated assays. The diagnoses of 210 patients were CMS in 88 (41.9%), NMOSD 47 (22.4%, 27 NMO, 20 other NMOSD), single attack of myelitis 23 (11.0%), single attack of optic neuritis 21 (10.0%), relapsing myelitis 10 (4.8%), acute disseminated encephalomyelitis (ADEM) 9 (4.3%), relapsing optic neuritis in 6 (2.9%), opticospinal multiple sclerosis 3 (1.4%) and single attack of brainstem encephalitis 3 (1.4%). Compared to CMS, NMOSD patients had older onset age, lower frequencies of brain MRI abnormalities and CSF OCB, higher frequency of LETM, higher CNS inflammation attack frequency in the first 2years, worse clinical outcome with higher EDSS score and mortality rate. This hospital-based study suggests that CMS (41.9%) and NMOSD (22.4%) are the most common CNS IDD among Hong Kong Chinese. NMOSD has worse clinical outcome than CMS. Detection of AQP4 Ab facilitates early diagnosis and prompts immunotherapies of NMOSD.

Orbital Ectopic Lymphoid Follicles with Germinal Centers in Aquaporin-4-IgG-Positive Neuromyelitis Optica Spectrum Disorders

Neuromyelitis optica spectrum disorders (NMOSDs) are important autoimmune central nervous system (CNS) astrocytopathy causing acute myelitis, optic neuritis (ON), and encephalitis associated with significant morbidities and mortality. It is important to diagnose NMOSDs early as they are treatable. The majority of NMOSDs patients are seropositive for aquaporin-4 IgG (AQP4-IgG) autoantibodies, which target CNS aquaporin-4 (AQP4) expressed abundantly in astrocytic foot processes. We report the novel observation of orbital masses containing ectopic lymphoid follicles with germinal centres (GC) in two patients with AQP4-IgG-positive NMOSD. Both patients had severe extensive myelitis with symptomatic or asymptomatic ON, with the ectopic lymphoid structures detected on initial presentation. Histolopathological studies confirmed that the orbital masses contained reactive lymphoid follicles with GC containing B cells and plasma cells. Our observations support that AQP4-IgG positive NMOSDs patients have underlying AQP4 autoimmunity and suggest that ON (symptomatic or asymptomatic) may trigger formation of orbital ectopic GC contributing to development of high-affinity AQP4-specific memory B cells and plasma cells, which produce highly pathogenic AQP4-IgG.

Inability to start hemodialysis after a smooth temporary hemodialysis catheter insertion procedure

Cannulation of the central vein for placement of the temporary dual‐lumen catheter for hemodialysis can usually be safely and reliably performed under ultrasonographic guidance. Here, we report a case of aberrant catheter entry into the internal thoracic vein during an apparently smooth procedure. The value of sonographic guidance, together with fluoroscopy with or without venography, will be discussed.