Sleep medicine: a deserted field in Pakistan

Sleep is an important and essential aspect of life and we spend about one third of our lives sleeping. Sleep disorders often manifest with insomnia and excessive daytime sleepiness. Large population based studies have shown that people who sleep longer and lesser have shorter life span1,2. Other symptoms of the slep disorders include snoring, abnormal motor behaviors etc

Cramp fasciculation syndrome: a peripheral nerve hyperexcitability disorder

Cramp fasciculation syndrome is mildest among all the peripheral nerve hyperexcitability disorders, which typically presents with cramps, body ache and fasciculations. The diagnosis is based on clinical grounds supported by electrodiagnostic study. We report a case of young male with two months’ history of body ache, rippling, movements over calves and other body parts, and occasional cramps. His metabolic workup was suggestive of impaired fasting glucose, radiologic work up (chest X-ray and ultrasound abdomen) was normal, and electrodiagnostic study was significant for fasciculation and myokymic discharges. He was started on pregablin and analgesics. To the best of our knowledge this is report first of cramp fasciculation syndrome from Pakistan

Motor neuronopathy associated with Adenocarcinoma of Esophagus

Neurologic paraneoplastic syndromes (NPS) are remote neurologic effects, except metastasis, of systemic cancers. These are a rare group of disorders, commonly associated with small cell lung carcinoma (SCLC).1 Various NPS have been described, including motor neuronopathy. The motor neuronopathy has commonly been described with small cell carcinoma of lung, breast cancer and lymphoproliferative disorders.2,3 We report a case of motor neuronopathy in association with large cell adenocarcinoma of esophagus. To our knowledge this is the first reported case of motor neuronopathy associated with adenocarcinoma of the esophagus

An Adult with Polyneuropathy and Hypogonadism due to Poems Syndrome

POEMS (acronym for polyneuropathy, organomegaly, endocrinopathy, M protein myeloma and skin changes), is a rare disease which occurs in the setting of plasma cell dyscrasias. We describe a case of an adult lady who presented with gradual onset weakness of all four limbs and multisystem involvement characterized by pedal edema, ascites, hyperpigmentation and hypogonadism. Nerve conduction study showed severe sensorimotor polyneuropathy. Serum immunofixation showed lambda light chain restricted monoclonal gammopathy. Bone marrow biopsy consistent with plasma cell dyscrasia. Hormonal assay showed decreased FSH, LH and estradiol levels which led us to diagnosis of hypogonadotrophic hypogonadism. The patient responded well to combination therapy of thalidomide, melphalan and dexamethasone. Eight months after the therapy, she noted decreased paresthesias and increased strength. She had reduced edema and ascites

Post stroke seizures: descriptive study from a tertiary care centre in Pakistan

OBJECTIVE: To determine the frequency, mode of onset and outcome of post stroke seizures in a local setting. METHODS: The retrospective (Aug 1999 to July 2001) and prospective (August 2001 to July 2002) data of patients aged 14 years and above, with post stroke seizures was collected from a tertiary care centre in a metropolitan city. The demographic, clinical, radiological, laboratory, neurophysiologic and outcome data was recorded and analyzed. RESULTS: During the three year period 1548 patients with stroke were admitted to the hospital. Four hundred thirty one (28%) had intracerebral haemorrhage (ICH) and 1117 (72%) had ischaemic stroke. One hundred seventeen (8%) of the 1548 had seizures. Their mean age was 63 +/- 12 (range; 15-70) years. Fifty-nine (50%) were men and 58 (50%) were women. Twenty of 431(5%) patients with ICH and 97/1117 (9%) with ischaemic stroke, developed seizures. Nine of 117 (8%) developed a seizure within 14 days of stroke and 108/117 (92%) developed seizures after 14 days of stroke. Twenty-six (22%) had partial and 91 (78%) had generalized seizures. Two patients expired early in the course. Twenty four (21%) of these continued to have seizures at one year follow up. Fifteen of 37 (40%) patients who had systemic infections early in the course continued to have seizures at one year as compared to 9/80 (11%) who did not (p = 0.001). CONCLUSIONS: Frequency of post stroke seizures is the same as reported in western literature. Post stroke seizures are more common in patients with ischaemic stroke. Generalized seizures are more common than partial seizures. Systemic infections early in the course are associated with recurrence of seizures at one year

Stroke in Pakistan

Stroke is the most common cause of disability and a leading cause of mortality world wide. Though the incidence is falling in West but probably is rising in Asia. The burden of stroke risk factors in Pakistan is enormous e.g. by 2020 Pakistan will be 4th most populous country in terms of diabetic patients. Similarly every 3rd person above age of 45 years has hypertension. Ironically a great majority of these patients are unaware of their diagnosis. This is further complicated by the fact that most of diagnosed patients have uncontrolled hypertension, as a result of poor compliance on patients part and poor updated knowledge on physicians part. Data on stroke incidence and prevalence from Pakistan is scarce, however, there are several reported case series in literature highlighting significant differences in terms of stroke epidemiology, risk factors and stroke subtypes/patterns. Considering a high population, absolute number of stroke in our country would be in millions. Its consequences are myriad ranging form physical disability to death, to psychologic, social and economic consequences. These consequences do not only affect the individual or his/her family but also society as a whole. We reviewed available literature on stroke and its risk factors from Pakistan

Prognostic Indicators in Patients with Primary Intraventricular Haemorrhage

Objectives: Primary intraventricular haemorrhage (PIVH) is a rare clinical entity. We sought to evaluate risk factors, clinical and radiological features, and outcome of patients with PIVH. Materials and Methods: Cases of PIVH were identified from cohort of patients with non traumatic intracerebral haemorrhage (ICH) by reviewing the radiological data. Their charts were reviewed and demographic, clinical, radiological and laboratory data was recorded and analyzed. Chi square and t test were used to determine predictors of poor outcome. Results: Fifteen of 677 (2%) patients with ICH had PIVH. Nine (60%) were men. Median age was 56 years. Predisposing factors included hypertension in twelve (80%), coagulopathy in five (33%) and vascular malformations in two (13%) patients. Eleven (73%) patients developed hydrocephalus. Two patients died. Univariate analysis identified diabetes mellitus, blood in all ventricles and coagulopathy as predictors of death during initial hospital stay and hydrocephalus as predictor of poor outcome (death and disability combined) (p\u3c0.05). Conclusion: Hypertension is most common associated risk factor for primary intraventricular haemorrhage followed by coagulopathy. Hydrocephalus is a common complication, associated with poor outcome. Diabetes mellitus, coagulopathy and panventricular blood predict early mortalit

Post-infarct cerebellar cognitive affective syndrome: a case report

Post Infarct cerebellar cognitive affective syndrome is a rare disorder, characterized by cognitive impairment in the domains of memory, language, visuo-spatial functioning and affect after cerebellar stroke. We report a case of young female who developed mood alteration and cognitive disturbance following isolated cerebellar infarct. We, therefore, advocate a potential role of cerebellum in regulation of cognition and behaviour in humans

Hypotension at presentation is an indicator of poor prognosis in acute intracerebral haemorrhage

OBJECTIVE: To identify frequency of hypotension in a large cohort of patients with intracerebral haemorrhage and its prognostic significance. METHODS: We retrospectively reviewed medical records of 920 patients with spontaneous intracerebral haemorrhage (ICH). Patients were divided in three groups based on Diastolic blood pressure (DBP); hypotensive group (DBP \u3c 70 mmHg), normotensive group (DBP; 71-90 mmHg) and hypertensive group (DBP \u3e 90 mmHg). RESULTS: Of the total patients with ICH, 7% (64) presented with hypotension, 13% (120) were normotensive and 80% (736) were hypertensive. In the hypotensive group, 37% (24) patients died as compared to 25%(30) in normotensive group and 25% (182) in hypertensive group (p = 0.03). Hypotension at presentation, thalamic and lobar haemorrhages were predictors of poor outcome. Patients with diastolic BP of less than 70 were significantly more likely to die than with DBP 71-90 (OR = 1.9, 95% CI; 1.1-2.9, p = 0.03). This relationship was still significant after adjusting for age, sex, history of presentation, coma at presentation and location of haemorrhage (OR = 1.45, 95% CI; 1.0-2.2, p = 0.045). CONCLUSION: Our findings suggest that hypotension at presentation is a predictor of poor outcome in patients with ICH. Patients with diastolic blood pressure less than 70 are more likely to have a fatal outcome as compared to those with normal blood pressure