Postural orthostatic tachycardia syndrome: an underrecognized disorder.

BACKGROUND: Postural orthostatic tachycardia syndrome (POTS), a clinical syndrome of orthostatic intolerance characterized by excessive tachycardia and symptoms of cerebral hypoperfusion on standing, is not well recognized in Australia. The aim was to study the clinical symptomatology, results of autonomic testing and outcome in patients with POTS. METHODS: Sixteen subjects from a tertiary referral centre who met the criteria for POTS were studied between January 2003 and January 2006. Ten of these patients consented to be interviewed using a validated autonomic symptom questionnaire. Heart rate responses to deep breathing and the Valsalva manoeuvre were measured using Colin BP-508 machine (WR Medical Electronic Co., Stillwater, MN, USA). Tilt studies were carried out for 10 min to 80 degrees of head-up tilting. Patient outcome was assessed as functionally normal, able to stand 30 min without symptoms, able to work and carry out recreational activities or worse on follow up. RESULTS: The mean age of 10 subjects was 24.9 +/- 6.8 years, six being women. The mean duration of symptoms was 70.7 months (range 3-228 months). The common presenting orthostatic symptoms were light-headedness (100%), palpitations (90%), pallor (90%), weakness (80%) and clammy skin (80%). The mean heart rate increment during the tilt study was 51.7 +/- 14.3 b.p.m. The mean duration of follow up was 8.9 months (range 1-16 months). Only five patients were functioning normally at the follow-up visit. CONCLUSION: POTS is an underrecognized but persistent autonomic disorder in young patients with a variety of symptoms and variable outcome.

Iatrogenic meningitis after lumbar puncture-a preventable health hazard.

Iatrogenic meningitis (IM) is a rare complication of diagnostic and therapeutic lumbar puncture (LP). This study includes cases of IM managed in the Departments of Neurology, of two referral hospitals, in India between January 1984 and April 2002. The diagnosis of IM was made when symptoms of meningitis occurred 24 h to 21 days after LP. All the procedures were performed in the peripheral hospitals before they were referred to the two centres. There were 17(63%) women and 10(37%) men. The age range was 19-50 years with a mean age of 31. The precipitating event was spinal anaesthesia for pelvic and intra-abdominal surgeries (Caesarean section 11 cases, hysterectomy three cases, herniorraphy two cases, appendicectomy two cases, anal fissurectomy one case, varicocelectomy one case and hydrocelectomy one case) laminectomy in two and diagnostic myelogram in four patients. The cerebrospinal fluid (CSF) culture was positive in six (22%) patients. The organisms were Pseudomonas aeruginosa in one case, Staphylococcus aureus in three cases, Acinetobacter spp. in one case and Mycobacterium tuberculosis in one case. In five individuals, mycotic aneurysms with subarachnoid haemorrhage due to invasive aspergillosis was documented at autopsy. The mean follow-up was 10.6 months (range 1-18). Seventeen (63%) patients received conventional antibiotics alone, while 10 patients received antibiotics and anti-tuberculous drugs when the meningitis became chronic. The mortality was 36%. The poor prognostic factors were women who underwent Caesarean section (P < 0.04) presence of hemiplegia (P < 0.04) and altered mental status (P < 0.0004). This study shows high morbidity and mortality of IM after LP. Simple aseptic precautions under- taken before the procedure can prevent IM. The urgent need for increasing the awareness among medical personnel in peripheral hospitals of developing countries cannot be over emphasized.

Non-convulsive status epilepticus: a rare presentation of juvenile myoclonic epilepsy.

We present a case of a boy with juvenile myoclonic epilepsy (JME) who presented with features of non-convulsive status epilepticus (NCSE). This case highlights the fact that NCSE, even though not a common occurrence in JME, should be kept in mind when a patient with previous history of seizures presents with subtle changes in sensorium with no obvious cause.

Epilepsia partialis continua--a clinical and electroencephalography study.

Epilepsia partialis continua (EPC) is a rare type of localization-related motor epilepsy. Clinical spectrum, electroencephalography (EEG) characteristics and various prognostic factors in EPC were studied in 20 patients. Patients who fulfilled the criteria for EPC between the years 1985 and 1999 were included in this retrospective and prospective study. The mean age was 18 years (range 5 months-70 years). Eleven patients (55%) had Type 1 EPC and in the remaining nine (45%) patients there were features of Type 2 EPC. Among children Rasmussen's encephalitis and viral encephalitis were the commonest cause for EPC. Encephalitis and vascular aetiology were frequently observed in adults. Tuberculous meningitis and tuberculomas occurred evenly in both the groups. The cause was unknown in two cases. Focal EEG abnormalities commonly consisted of discrete spikes, sharp waves (or) slow wave activity and periodic lateralized epileptiform discharges. The mean duration of follow up was 9.6 months with a range between 1 month and 4 years. Cognitive decline, motor deficits and pharmacoresistance to drugs were significantly seen among children with Type 2 EPC. Patients with Type 1 EPC had mild impairment of functional status with good response to treatment. The long-term prognosis depends upon the underlying cause.

Neurocysticercosis: a clinical and radiological appraisal from Kerala State, South India.

OBJECTIVES: Little has been published on Neurocysticercosis (NCC) in the State of Kerala, South India. This study was conducted to describe the clinical and radiological features of NCC in Kerala and also to study the frequency of NCC as seen in a tertiary referral setting. MATERIALS AND METHODS: We evaluated retrospectively 11 patients who were admitted with a diagnosis of NCC from 1986 to 1998. A pre-abstracted proforma containing detailed demographic data, dietary habits, clinical features and history of travel outside Kerala and neuroimaging findings were obtained from patient records and the data was tabulated and analysed. Follow up assessment was made three months after treatment. RESULTS: There were a total of 11 patients, including nine males and two females in the age range of 24 to 62 years and a mean age of 35.2 years. All were nonvegans, only 36% were pork eaters while 18% claimed consumption of salads and uncooked vegetables. 55% of patients were migrants. Migrants were defined as those who lived outside the state of Kerala for more than six months. Seizure was the most common presenting complaint and occurred in all patients (100%). Multiple ring enhancing lesions were seen on computerised tomography (CT) and magnetic resonance imaging (MRI) scans in 60%. Calcified lesions were noted in two patients. An isolated instance of miliary or disseminated cysticercosis with subcutaneous nodules and multiple brain lesions in MRI scan was observed. All patients received anticysticercal therapy besides anticonvulsants. CONCLUSIONS: It appears that NCC is rather uncommon in Kerala. Better socioeconomic status, high literacy rate, improved sanitation and health care in the state of Kerala could be reasons for this observation. A prospective case-control study of NCC in Kerala is needed to study these factors.

Brainstem abscess complicating tetralogy of Fallot successfully treated with antibiotics alone.

Medically treated brainstem abscess in a 11 year old boy with tetralogy of Fallot is reported. There was a complete resolution of the lesion without any neurologic sequelae during parenteral antibiotic therapy with crystalline penicillin, chloramphenicol and metronidazole. The pathogenesis and management of cardiogenic brain abscesses in general and brainstem abscess in particular has been reviewed.

Prevalence, knowledge, attitude, and practice of epilepsy in Kerala, South India.

PURPOSE: To ascertain the prevalence and pattern of epilepsy and to characterize and quantify knowledge, attitude, and practice (KAP) toward epilepsy among the people of the state of Kerala, which is distinguished from the rest of India by a high level of literacy and health awareness of its population. METHODS: We conducted a door-to-door survey covering the entire population of 238,102 people residing in 43,681 households in a semiurban area of central Kerala. The screening questionnaire administered by medical social workers had a sensitivity of 100% for identifying persons with epilepsy. Neurologists examined all the individuals suspected of having epilepsy. We evaluated KAP toward epilepsy among 1,118 subjects (439 males and 679 females; mean age, 33.3 years; age range, 15-85 years) from households without epilepsy in the study area. RESULTS: Through a three-phased survey, we ascertained 1,175 cases (616 males and 559 females) with active epilepsy, providing a crude point prevalence ratio of 4.9 cases per 1,000 people and an age-adjusted prevalence ratio of 4.7 cases per 1,000 population. The highest age-specific prevalence rate of 6.5 per 1,000 occurred in the 10- to 19-year-old age group. Sex-specific prevalence rates did not significantly differ. The proportion of generalized and localization-related epilepsies was 58.8% and 30.6%, respectively. Ninety-nine percent of the KAP respondents had read or heard about epilepsy. Thirty-one percent and 27% thought epilepsy was a hereditary disorder and a form of insanity, respectively. About 40% of the respondents felt that individuals with epilepsy could not be properly educated or employed. Eleven percent would object to their children having contact with epileptic children. CONCLUSIONS: The prevalence and pattern of epilepsy in central Kerala, South India, do not differ from that of developed countries. Although the awareness of epilepsy among the people of Kerala was comparable to that of developed countries, the attitudes were much more negative. The need for educating the people of Kerala on epilepsy and for incorporating an adequate knowledge of epilepsy in the school curricula cannot be overemphasized.

Fulminant cerebral infarction in a patient with nephrotic syndrome.

Fulminant cerebral infarction secondary to arterial thrombosis in adults with nephrotic syndrome is rare. We report a 42 year old male with fulminant right anterior cerebral and middle cerebral artery infarction. Minimal change disease of the kidney was documented by renal biopsy. The possible pathogenesis is discussed and pertinent literature reviewed.

Myasthenia gravis, motor neuron syndrome and thymoma.

An adult male with thymomatous myasthenia gravis (MG) and a motor neuron syndrome simulating amyotrophic lateral sclerosis is reported. After thymectomy and corticosteroid therapy, the MG remitted. During 4 years of follow-up, the lower motor neuronsigns in the upper limbs and upper motor neuron signs in the lower limbs remained unchanged. Literature concerning paraneoplastic neurological syndromes associated with thymoma has been reviewed.

Dermatomal somato sensory evoked potentials in cervical spondylosis.

Dermatomal somatosensory evoked potentials (DSSEP) from C5-T1 dermatomes were recorded in 20 normal healthy adult volunteers as controls and 61 patients with neck pain. The patients were divided into three groups. Group I : symptomatic patients withclinical signs of C5-T1 radiculopathy and radiological evidence of cervical spondylosis (21 patients). Group II : symptomatic patients without clinical signs of radiculopathy but with radiological evidence of cervical spondylosis (20 patients). Group III: patients with persistent neck pain without any clinical radiculopathy or radiological evidence of cervical spondylosis (20 patients). A complete neurological examination and nerve conduction study of median and ulnar nerves were done to rule out peripheral neuropathy. Normative data was established for DSSEP from C5-T1 dermatomes using the control group and the values obtained were comparable with western normative data. In the group I, DSSEP was abnormal in all the cases. It was possible to preciselylocate the involvement of C5 root in 75 , C6 root in 88.88, C7 root in 92.30 , C8 root in 76.92, and T1 root in 100. The DSSEP was abnormal in 11 patients in Group II, but was normal in all the patients of Gruoup III. DSSEP of C5-T1 dermatomes correlated well with the clinical findings in Group I patients. It was possible to detect subclinical radioculopathy in a significant number of patients in Group II. DSSEP is a simple non invasive method for evaluation of cervical radiculopathy.