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

Asian Cerebral venous thrombosis registry: study protocolV

Introduction: Cerebral venous thrombosis (CVT) is a well known but poorly reported entity. Most of the studies and registries related to CVT are reported from European countries. No large multi-center or multi- national data base or registry has been reported from Asian countries. CVT is not uncommon in Asia especially in south Asian subcontinent including India, Pakistan and Bangladesh. One study reported from India that CVT accounted for half of all strokes in the young and 40% of strokes in women. Review of CVT cases from Asian countries is suggestive of differences in risk factors profile and outcome in these patients as compared with European studies. These findings from multi- center data base in Asian countries will be extremely important in identifying risk factors for CVT in these countries. Study design: This is a prospective observational study. We plan to enroll more than 1000 patients from at least ten Asian countries (about 40-50 centers). Patients will be enrolled prospectively and followed for six months. Primary outcome would be death or dependence as assessed by modified Rankin scale (mRS). Data will be collected on a pre-defined data form. There will not be any laboratory test, investigation or treatment specified by the study. Only results of routinely performed studies and treatments will be recorded. Patient (aged 16 or above) will only be included in study if they have diagnosis of CVT proven by magnetic resonance imaging (MRI), magnetic resonance venography (MRV), computed tomography (CT) venography and cerebral venography according to established criteria. Follow up visits will be performed at 6 months, 12 months, and yearly thereafter, preferably by direct interview and observations by the local investigators. Outcome: Primary outcome is death or dependence (mRS \u3e2) at the end of the follow-up period. Secondary outcomes are death and dependence at 6 months. Patients will be enrolled from January 2009 to June 201

Review of various intraoperative neurophysiologic monitoring techniques

IONM is use to monitoring nervous tissues (including brain, spinal cord, cranial nerves and peripheral nerves) in real-time during surgeries, alert neurological injuries and corrective measures and prevent disability. There are various IONM monitoring techniques including evoke potentials (SSEP, BAEP, MEP), EMG (Free-running and triggered), NAP (Nerve action potential) and Electroencephalography (EEG) to monitor the functional integrity of neural structures. SSEP evaluates integrity of posterior column-medial lemniscus pathway. SSEP is clinical use in spinal cord surgeries, vascular surgeries (carotid endarterectomy, cerebral aneurysm surgery etc), and localization of sensor motor cortex. BAEP evaluates integrity of peripheral and central auditory pathway. BAEP is clinical use in CP angle tumors surgery (acoustic neuroma ,meningioma), microvascular decompression of CN-VII for hemifacial spasm, CN-V for trigeminal neuralgia, CN-IX for glossopharyngeal neuralgia, skull base surgery, Suboccipital decompression (e.g. fractures/dislocation C-1vertebra, chiari malformation). MEP evaluates integrity of motor pathway. MEP is sensitive to neuromuscular blocker anesthetic medications. Clinical utility of MEP including any surgery risking motor pathway injury include tumor near the motor cortex or corticospinal tract, intracranial aneurysm clipping, posterior fossa surgery, tethered cord or cauda equina surgeries, spinal deformity or fracture surgery, vertebral tumor resections, and anterior cervical discectomy, descending aortic procedures, spinal arteriovenous malformation interventions and carotid endarterectomy. EMG (free running and triggered) evaluates integrity of innervating nerves and electrical activity of muscles. Clinical utility of facial and other cranial nerve monitoring in posterior fossa surgery (eg, acoustic neuroma), selective dorsal rhizotomy, tethered cord release , Pedicle screw placement and Anal or urinary sphincter function monitoring

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

Obstetric cerebral venous thrombosis

Pregnancy and puerperium are most prevalent prothrombotic states leading to cerebral venous thrombosis. Likelihood of stroke to be of venous origin is greater in stroke associated with pregnancy compared to stroke unrelated to pregnancy. Pregnancy induces several changes in coagulation system, which persists at least during early puerperium, rendering it a prothrombotic state. Hypercoaguability worsens further after delivery as a result of volume depletion and trauma. During puerperium additional risk factors include infection and instrumental delivery or Caesarean section. The management follows general rules as for the venous thrombosis unrelated to pregnancy, however the prognosis is different