Spontaneous dural tear leading to intracranial hypotension and tonsillar herniation in Marfan syndrome: a case report.

BACKGROUND: We describe the case of a 38 year old male with Marfan syndrome who presented with orthostatic headaches and seizures. CASE PRESENTATION: The patient was diagnosed with Spontaneous Intracranial Hypotension secondary to CSF leaks, objectively demonstrated by MR myelogram with intrathecal contrast. Epidural autologous blood patch was administered at the leakage site leading to significant improvement. CONCLUSION: Our literature search shows that this is the second reported case of a Marfan patient presenting with symptomatic spontaneous CSF leaks along with tonsillar herniation.

Parkinson's Disease in the Middle East, North Africa, and South Asia: Consensus from the International Parkinson and Movement Disorder Society Task Force for the Middle East.

BACKGROUND: Understanding the regional needs and available healthcare resources to treat Parkinson's disease (PD) is essential to plan appropriate future priorities. The International Parkinson and Movement Disorder Society (MDS) Task Force for the Middle East was established to raise awareness and promote education across the region on PD and other movement disorders. Broadly, the task force encompasses the countries of the Middle East but has included North Africa and South Asia as well (MENASA). OBJECTIVE: To create a list of needs and priorities in the advancement of PD in MENASA countries based on consensuses generated by the MDS task force for the Middle East. METHODS: A Strengths Weaknesses-Opportunities-Threats (SWOT) analysis was conducted by the task force members to generate consensus about PD care this region. RESULTS: Eight overarching principles emerged for the consensus statement on current needs: more movement disorders specialists, multidisciplinary care, accurate epidemiologic data, educational programs, availability of drugs, and availability of more advanced therapy, enhanced health care resources and infrastructure, and greater levels of awareness within the general population and among health care professionals. CONCLUSION: This pilot study sheds light on unmet needs for providing care to people with PD in the MENASA region. These data offer directions on priorities to increase awareness of PD, to develop better infrastructure for research and management of PD, to foster healthcare policy discussions for PD and to provide educational opportunities within these countries.

A Case Report of Seronegative Limbic Encephalitis.

Most patients with autoimmune encephalitis do not present with well-described symptoms. Demographic data and information regarding co-morbidities could help in diagnosing the underlying disorder, but a definitive diagnosis is made by the result of autoimmune antibodies. Limbic encephalitis (LE), a variant of autoimmune encephalitis, is the inflammation of the limbic system of the brain. The disorder presents with the rapid development of confusion, working memory impairment, mood changes, and often seizures. LE could have paraneoplastic or non-paraneoplastic etiology. We present the case of a 15-year-old girl with seronegative LE, who presented with cognitive decline and seizures. This condition is rare, and therefore poses a great challenge in diagnosis at an early stage.

Neuroleptic malignant syndrome: need for early diagnosis and therapy.

BACKGROUND: Neuroleptic Malignant Syndrome (NMS) is a medical entity that has received little attention in the clinical settings in Pakistan. The aim of our study was to review the predisposing factors, outcomes and characteristics of in-patients diagnosed with NMS. METHODS: We performed a retrospective chart review of all cases (age >15 years) at a tertiary care center in Karachi between January 01, 1990 and November 30, 2001, diagnosed using ICD 10 coding. Data was collected using a standardized data entry form and statistical analysis was performed using Epi Info 6, Version 6.02. RESULTS: There were a total of 20 patients diagnosed with NMS (11 male and 9 female) in our study with a mean age of 46.6 +/- 15.9 years. Haloperidol was the most frequently responsible neuroleptic. Of the 18 patients on a neuroleptic, most developed NMS after 8 weeks of therapy. There were 5 mortalities all of which were associated with septic shock. Fourteen patients recovered completely from the episode and did not have any neurologic sequelae. CONCLUSIONS: NMS is an important preventable clinical entity. Early diagnosis and judicious use of antipsychotics is warranted to prevent mortality and heightened morbidity.

Autonomic nervous system dysfunction predicts poor prognosis in patients with mild to moderate tetanus.

BACKGROUND: Autonomic nervous system (ANS) dysfunction is present in up to one third of patients with tetanus. The prognostic value of ANS dysfunction is known in severe tetanus but its value is not well established in mild to moderate tetanus. METHODS: Medical records of all patients admitted with tetanus at two academic tertiary care centers in Karachi, Pakistan were reviewed. The demographic, clinical and laboratory data was recorded and analyzed. ANS dysfunction was defined as presence of labile or persistent hypertension or hypotension and sinus tachycardia, tachyarrythmia or bradycardia on EKG. Patients were divided into two groups based on presence of ANS dysfunction (ANS group and non ANS group). Tetanus severity was classified on the basis of Ablett criteria. RESULTS: Ninety six (64 males; 32 females) patients were admitted with the diagnosis over a period of 10 years. ANS group had 31 (32%) patients while non ANS group comprised of 65 (68%) patients. Both groups matched for age, gender, symptom severity, use of tetanus immunoglobulin and antibiotics. Twelve patients in ANS group had mild to moderate tetanus (Ablett I and II) and 19 patients had severe/very severe tetanus (Ablett III and IV). Fifteen (50%) patients in ANS group required ventilation as compared to 28 (45%) in non-ANS group (p = 0.09). Fourteen (47%) patients died in ANS group as compared to 10 (15%) in non ANS group (p= 0.002). Out of those 14 patients died in ANS group, six patients had mild to moderate tetanus and eight patients had severe/very severe tetanus. Major cause of death was cardiac arrhythmias (13/14; 93%) in ANS group and respiratory arrest (7/10; 70%) in non ANS group. Ten (33%) patients had complete recovery in ANS group while in non ANS group 35(48%) patients had complete recovery (p= 0.05). CONCLUSIONS: ANS dysfunction was present in one third of our tetanus population. 40% patients with ANS dysfunction had only mild to moderate tetanus. ANS dysfunction, irrespective of the need of mechanical ventilation or severity of tetanus, predicted poor outcome.

Kleine-Levin syndrome.

Kleine-Levin Syndrome (KLS) is a rare disorder of uncertain etiology, characterized by recurring episodes of undue sleepiness lasting for days. We report a case of young female presenting with episodes of undue sleep along with hypersexuality and excessive food intake, who improved significantly on lithium and valproate. KLS should be considered in young patients, who present with episodes of undue somnolence.

Predictors of ischemic versus hemorrhagic strokes in hypertensive patients.

OBJECTIVE: To identify the factors that predispose to ischemic versus hemorrhagic stroke in hypertensive patients. DESIGN: Cohort study. PLACE AND DURATION OF STUDY: The study was conducted at the Aga Khan University Hospital (AKUH), Karachi, from August 1999 to May 2001. MATERIALS AND METHODS: All the hypertensive patients, who were registered in AKUH acute stroke outcome data base, over a period of 22 months, were identified and from this cohort the patients with first ever stroke were selected. The data regarding demographics, stroke type (ischemic vs. hemorrhagic), pre-existing medical problems, laboratory and radiological investigations was recorded and analyzed. RESULTS: Five hundred and nineteen patients with either ischemic stroke or parenchymal hemorrhage were registered over a period of 22 months. Three hundred and forty-eight patients (67%) had hypertension and of these, 250 had first ever stroke at the time of admission. Presence of diabetes mellitus (OR: 3.76; CI:1.67-8.46) and ischemic heart disease (OR: 6.97; CI:1.57-30.98) were found to be independent predictors of ischemic strokes. CONCLUSION: Presence of diabetes mellitus and ischemic heart disease predict ischemic stroke in a patient with hypertension.