Normative values of the Brief International Cognitive Assessment for Multiple Sclerosis (BICAMS) in an Arab population: Corrected for age, sex and education.

Background Cognitive impairment is common in multiple sclerosis (MS) patients with 43-70% of patients being affected. The international cognitive assessment for MS BICAMS is a new battery for cognitive assessment in MS patients. It was suggested in 2012 that international validation efforts be undertaken. Objective To utilize regression to provide normative values for the BICAMS while correcting for age, gender and education, as well as assess test-retest reliability, in an Arab population. Methods Healthy subjects were recruited from different areas in Dubai. Corrections for demographics were calculated using multiple linear regression. Test-retest reliability was assessed using the Pearson correlation coefficient. Results Two hundred eighty-one healthy subjects were enrolled (174 women, mean age 30±10.3 years, mean education 17.8±3.7 years). Test re-test results showed significant correlation between baseline and retest scores. Regression-based equations were derived for the BICAMS. Conclusion This large-scale validation study provides evidence to support the facilitation of international implementation of the BICAMS assessment by focusing on the Arab population. Next steps include validation efforts in an Arab MS patient population to further support the use of the BICAMS in day to day practice and for longitudinal patient assessment.

Unusual presentation of idiopathic intracranial hypertension.

Idiopathic intracranial hypertension typically presents with holocephalic headache associated with nausea, vomiting and bilateral papilledema. Involvement of the sixth cranial nerve is relatively common. The involvement of other cranial nerves, however, is rare in this disorder. We describe a patient with idiopathic intracranial hypertension who presented with episodic unilateral retro-orbital pain and multiple cranial nerve abnormalities without papilledema. Imaging studies excluded alternate diagnoses, and the immediate resolution of symptoms after lumbar puncture confirmed that these symptoms were due to intracranial hypertension. Atypical presentations of such a disabling yet treatable disorder is very important to recognise and address.

CT pattern of Infarct location and not infarct volume determines outcome after decompressive hemicraniectomy for Malignant Middle Cerebral Artery Stroke.

Malignant middle cerebral artery [MMCA] infarction has a different topographic distribution that might confound the relationship between lesion volume and outcome. Retrospective study to determine the multivariable relationship between computerized tomographic [CT] infarct location, volume and outcomes in decompressive hemicraniectomy [DHC] for MMCA infarction. The MCA infarctions were classified into four subgroups by CT, subtotal, complete MCA [co-MCA], Subtotal MCA with additional infarction [Subtotal MCAAI] and co-MCA with additional infarction [Co-MCAAI]. Maximum infarct volume [MIV] was measured on the pre-operative CT. Functional outcome was measured by the modified Rankin Scale [mRS] dichotomized as favourable 0-3 and unfavourable ≥4, at three months. In 137 patients, from least favourable to favourable outcome were co-MCAAI, subtotal MCAAI, co-MCA and subtotal MCA infarction. Co-MCAAI had the worst outcome, 56/57 patients with additional infarction had mRS ≥ 4. Multiple comparisons Scheffe test showed no significant difference in MIV of subtotal infarction, co-MCA, Subtotal MCAAI but the outcome was significantly different. Multivariate analysis confirmed MCAAI [7.027 (2.56-19.28), p = 0.000] as the most significant predictor of poor outcomes whereas MIV was not significant [OR, 0.99 (0.99-01.00), p = 0.594]. Other significant independent predictors were age ≥ 55 years 12.14 (2.60-56.02), p = 0.001 and uncal herniation 4.98(1.53-16.19), p = 0.007]. Our data shows the contribution of CT infarction location in determining the functional outcome after DHC. Subgroups of patients undergoing DHC had different outcomes despite comparable infarction volumes.

Rescue treatment in patients with poorly responsive Guillain-Barre syndrome.

OBJECTIVES: To evaluate the effectiveness of rescue treatment (intravenous immunoglobulin or plasma exchange) in patients with Guillain-Barre syndrome who did not respond or deteriorated after the initial management with intravenous immunoglobulin. METHODS: We performed a retrospective review of the medical records of patients who responded poorly or did not respond to intravenous immunoglobulin treatment. The disability parameters of those who received second-line treatment with intravenous immunoglobulin or plasma exchange (20 patients) were compared with those who did not receive second-line treatment (19 patients). RESULTS: There was a statistically significant improvement in disability scores at 1 month in the patients who received the rescue treatment (p = 0.033). However, there was no significant difference in the disability scores at 3 and 6 months, or in length of intensive care unit stay. CONCLUSION: Our study showed that a second course of treatment to carefully selected patients may be beneficial.

Iatrogenic transverse myelitis in a patient with rheumatoid arthritis.

We report the case of a 59-year-old Arab woman who was presented with acute onset of neck pain followed by quadriparesis, paraesthesias of lower limbs and incontinence of urine. Examination revealed asymmetric sensorimotor quadriparesis with sensory level at T1, establishing a clinical diagnosis of transverse myelitis. Cervical and thoracic spinal MRI showed enhancing T2/fluid attenuated inversion recovery (FLAIR) hyperintense lesion extending from C4 to C7 level in addition to long-segment lesion extending the whole of the spinal cord. She was known to have rheumatoid arthritis for the past 20 years and has been on etanercept for the past 8 years and methotrexate since past 3 years. Etanercept was stopped and she was treated with methylprednisolone followed by oral steroids and physiotherapy with which she had near complete recovery.

Delayed presentation of neck arteries dissection, caused by water slide activity.

A 16-year-old Korean boy presented with acute onset vertigo, dysphagia and gait ataxia of 16 hours duration. He had history of headache and neck pain along with transient vertigo during a water slide ride 12 days before presentation. CT brain showed left cerebellar and left lateral medullary infarcts. CT angiography showed left vertebral dissection with occlusion of left posterior inferior cerebellar artery. A 52-year-old Indian man, presented with acute onset global aphasia and right hemiparesis within 3 hours of onset of symptoms. He received intravenous tissue plasminogen activator (tPA) with partial improvement in his symptoms. He had headache and neck pain since 2 weeks, ever since he had a water slide ride. CT brain was normal, while the CT angiogram showed left carotid dissection. Cervical artery dissection has been reported with roller coaster rides and rarely with delayed presentations. Delayed presentation of cervical artery dissection after water rides have not been reported.

Factors that Can Help Select the Timing for Decompressive Hemicraniectomy for Malignant MCA Stroke.

In patients with malignant middle cerebral artery (MMCA) stroke, a vital clinically relevant question is determination of the speed with which infarction evolves to select the time for decompressive hemicraniectomy [DHC]. A retrospective, multicenter cross-sectional study of patients referred for DHC, based on the criteria of randomized controlled trials, was undertaken to identify factors for selecting the timing of DHC in MMCA stroke, stratified by time [< 48, 48-72, > 72 h]. Infarction volume and infarct growth rate [IGR] were measured on all CT scans. One hundred eighty-two patients [135 underwent DHC and 47 survived without DHC] were included in the analysis. After multivariate adjustment, factors showing the strongest independent association with DHC were patients < 55 years of age, septum pellucidum deviation, temporal lobe involvement, MCA with additional infarcts, and IGR on second CT. Of the five factors identified, different combinations of determining factors were observed in each subgroup. Both first and second IGRs were highest in the < 48, 48-< 72, and > 72 h [p < 0.001]. Patients who survived without surgery had the slowest IGRs. There was no association between time to DHC and infarct volume, although infarct volume was lower in patients who survived without DHC compared to the DHC subgroups. We identify the major risk factors associated with DHC in time-stratified subgroups of patients with MMCA. Evaluation of IGRs between the first and second scan and when possible second and third scan can help in selecting the timing of hemicraniectomy.

Mycoplasma pneumoniae infection presenting as stroke and meningoencephalitis with aortic and subclavian aneurysms without pulmonary involvement.

A 39-year-old Philipino man presented with acute onset fever and headache. Neurological examination was normal except for neck stiffness. There was no history of chest pain, cough or breathlessness. Cerebrospinal fluid (CSF) showed a mild increase in protein with normal sugar and lymphocytic pleocytosis. CSF PCR for herpes simplex and varicella zoster virus was negative. He developed acute right haemiplegia a week after hospitalisation. MRI showed acute infarct in the left centrum semiovale. His angiogram showed aneurysm in the left subclavian artery and aortic arch. The mycoplasma antibody test came positive with very high titres, while rest of the workup was negative. He was treated with azithromycin and his symptoms improved completely.He was asymptomatic on follow-up after a month. His repeat immunoglobulin G mycoplasma antibody titre showed elevation. Mycoplasma infection is a treatable cause of meningoencephalitis and stroke secondary to vasculitis. Arterial aneurysms are known to occur with mycoplasma infection although rare.

Prediction of infarction volume and infarction growth rate in acute ischemic stroke.

The prediction of infarction volume after stroke onset depends on the shape of the growth dynamics of the infarction. To understand growth patterns that predict lesion volume changes, we studied currently available models described in literature and compared the models with Adaptive Neuro-Fuzzy Inference System [ANFIS], a method previously unused in the prediction of infarction growth and infarction volume (IV). We included 67 patients with malignant middle cerebral artery [MMCA] stroke who underwent decompressive hemicraniectomy. All patients had at least three cranial CT scans prior to the surgery. The rate of growth and volume of infarction measured on the third CT was predicted with ANFIS without statistically significant difference compared to the ground truth [P = 0.489]. This was not possible with linear, logarithmic or exponential methods. ANFIS was able to predict infarction volume [IV3] over a wide range of volume [163.7-600 cm3] and time [22-110 hours]. The cross correlation [CRR] indicated similarity between the ANFIS-predicted IV3 and original data of 82% for ANFIS, followed by logarithmic 70%, exponential 63% and linear 48% respectively. Our study shows that ANFIS is superior to previously defined methods in the prediction of infarction growth rate (IGR) with reasonable accuracy, over wide time and volume range.

Decompressive Hemicraniectomy for Malignant Middle Cerebral Artery Stroke: South Asian Experience.

BACKGROUND: The randomized trials showed improved outcome and reduced mortality in malignant middle cerebral artery (MMCA) undergoing Decompressive hemicraniectomy (DHC) within 48 hours of stroke onset. Despite high prevalence of stroke, especially in younger individuals, high and short-term mortality from stroke in South Asian and Middle East, there is little published data on DHC in patients with MMCA stroke. METHODS: This is a retrospective, multicenter cross-sectional study to measure outcome following DHC using the modified Rankin Scale (mRS) and dichotomized as favorable (mRS ≤ 4) or unfavorable (mRS > 4), at 3 months. RESULTS: In total, 137 patients underwent DHC. At 90 days, mortality was 16.8%; 61.3% of patients survived with an mRS of 4 or less and 38.7% had an mRS greater than 4. Age (55 years), diabetes (P = .004), hypertension (P = .021), pupillary abnormality (P = .048), uncal herniation (P = .007), temporal lobe involvement (P = .016), additional infarction (MCA + anterior cerebral artery, posterior cerebral artery) (P = .001), and infarction growth rates (P = .025) were significantly higher in patients with unfavorable prognosis in univariate analysis. Multivariate analysis showed age, additional infarction, septum pellucidum deviation greater than 1 cm, and uncal herniation to be associated with a significantly poor prognosis. Time to surgery had no impact on outcome (P = .109). CONCLUSIONS: Similar to the results of the studies from the West, DHC Improves functional outcome in predominantly South Asian patients with MMCA Stroke.

Revisiting Hemicraniectomy: Late Decompressive Hemicraniectomy for Malignant Middle Cerebral Artery Stroke and the Role of Infarct Growth Rate.

Objective and Methods. The outcome in late decompressive hemicraniectomy in malignant middle cerebral artery stroke and the optimal timings of surgery has not been addressed by the randomized trials and pooled analysis. Retrospective, multicenter, cross-sectional study to measure outcome following DHC under 48 or over 48 hours using the modified Rankin scale [mRS] and dichotomized as favorable ≤4 or unfavorable >4 at three months. Results. In total, 137 patients underwent DHC. Functional outcome analyzed as mRS 0-4 versus mRS 5-6 showed no difference in this split between early and late operated on patients [P = 0.140] and mortality [P = 0.975]. Multivariate analysis showed that age ≥ 55 years, MCA with additional infarction, septum pellucidum deviation ≥1 cm, and uncal herniation were independent predictors of poor functional outcome at three months. In the "best" multivariate model, second infarct growth rate [IGR2] >7.5 ml/hr, MCA with additional infarction, and patients with temporal lobe involvement were independently associated with surgery under 48 hours. Both first infarct growth rate [IGR1] and second infarct growth rate [IGR2] were nearly double [P < 0.001] in patients with early surgery [under 48 hours]. Conclusions. The outcome and mortality in malignant middle cerebral artery stroke patients operated on over 48 hours of stroke onset were comparable to those of patients operated on less than 48 hours after stroke onset. Our data identifies IGR, temporal lobe involvement, and middle cerebral artery with additional infarct as independent predictors for early surgery.

Cerebral venous thrombosis presenting with subdural haematoma as first presentation for systemic lupus erythematosus with negative antiphospholipid antibodies.

We report the case of a 30-year-old woman, without any previous comorbidities presenting with acute onset headache, altered sensorium and unsteadiness of gait. Neurological evaluation revealed a drowsy patient with papilloedema, bilateral lateral rectus palsy, generalised hyper-reflexia and up going plantar responses. Urgent imaging performed showed extensive cortical venous sinus thrombosis. Workup for secondary causes of cortical venous sinus thrombosis revealed very high titres of antinuclear antibody and anti-dsDNA, but negative antiphospholipid antibodies (APLA). In hospital she started developing other complications of systemic lupus erythematosus (SLE). Urine evaluation revealed proteinuria and granular casts suggestive of glomerulonephritis. Cardiac evaluation revealed moderate pericardial effusion. We have discussed neurolupus as initial presentation of SLE and the rare occurrence of major neurovascular complications without secondary APLA syndrome.