An Adolescent Boy with Acquired Epileptic Aphasia –Landau Kleffner Syndrome: A rare case report.

A 13 year adolescent boy presented with complaints of progressive loss of speech and seizure disorder for the past 1 year. The boy was developmently normal before the onset of illness. There was history of subtle trauma to head. He started communicating with signs followed by paucity of speech progressed to complete aphasia and abnormal behavior in the form of burst of aggressiveness and hyperactivity. Boy had partial, generalized tonic-clonic seizures occurred during sleep. Mental status examination revealed abnormal behaviour, attention deficits, auditory agnosia and aphasia (both expressive and receptive). All relevant investigations including BERA were normal.EEG showed repetitive spikes and wave activity in bilateral parietooccipital regions during nonrapid eye movement. Clinical features and EEG led to a diagnosis of Landau Kleffner Syndrome . He was started on Lamotrigine, steroid and speech therapy. The boy started showing improvement of symptoms in form of understanding of short sentences.

Neuromuscular respiratory failure in Guillain-Barre Syndrome: evaluation of clinical and electrodiagnostic predictors.

Guillain-Barre Syndrome (GBS) has an unpredictable clinical course with up to 30% of patients requiring assisted ventilation during the course of their illness. Successful management mandates anticipation, prompt recognition and optimal treatment of neuromuscular respiratory failure in GBS. AIMS: To identify clinical and electrodiagnostic predictors of neuromuscular respiratory paralysis in GBS. MATERIALS AND METHODS: Forty six patients of GBS were studied over a 6 year period, the study being 2 year retrospective and 4 year prospective. Clinical and electrodiagnostic data were compared between ventilated (28) and non-ventilated (18) patients. The clinical parameters assessed were median age, gender, antecedent infection, prior lung disease, time to peak disability, bifacial weakness, upper limb weakness, bulbar paralysis, neck weakness and autonomic dysfunction. Electrodiagnostic studies included motor nerve conduction studies in 11 ventilated and 13 non-ventilated patients, done prior to maximum disability in each group. Multiple logistic regression analysis was used to compare the two groups. RESULTS: Comparing the clinical data in the ventilated and non-ventilated groups, 'early peak disability', autonomic dysfunction and bulbar weakness predicted the onset of respiratory paralysis. Age, gender, neck or bifacial weakness, upper limb paralysis, or preceding infection did not influence the development of neuromuscular respiratory weakness. Electrodiagnostic testing revealed abnormal H reflex and F waves to be the commonest abnormality in either group. Although data was not sufficient for statistical analysis, the presence of markedly attenuated Compound Muscle Action Potentials inexcitable motor nerves and denervation changes on the electromyography, was commoner in the ventilated group. Thirty six patients received treatment with either plasmapheresis (12) or intravenous immunoglobulin (24). Overall mortality was 5, all 5 patients being on assisted ventilation. CONCLUSION: Early progression to peak disability, bulbar dysfunction and autonomic instability predicted the development of neuromuscular respiratory paralysis in GBS. Early electrodiagnostic studies in this series suggest axonopathic GBS as a predictor of respiratory paralysis, a finding that needs to be evaluated with sufficient data to permit statistical analysis.

Geophagia leading to hypokalemic quadriparesis in a postpartum patient.

Pica is an eating disorder associated with ingestion of variety of non-food substances. A postpartum patient who presented with acute flaccid quadriparesis was detected to have severe hypokalemia. After extensive investigations for cause of hypokalemia, history of geophagia (clay-eating) was obtained. Approach to hypokalemia and health hazards of pica are discussed.

Presenile dementia--etiology, clinical profile and treatment response at four month follow up.

Dementia is the development of multiple cognitive deficits that includes memory impairment and at least one of the following--Aphasia, apraxia, agnosia or disturbances in executive functioning. The common causes of dementia among the elderly are Alzheimer's disease, vascular dementia, mixed dementia and Lewy body disease. The concept of reversible dementia was introduced in 1980 when a task force sponsored by National Institute of Ageing found 10-12% of dementia cases in older group to have reversible causes such as metabolic-nutritional, drugs, infections, psychiatric disorders etc. In our series of 76 patients in the presenile age group (<65 years), 34.21% (26/76) had a reversible condition underlying the dementia. 43.42% (33/76) had vascular dementia, 13.15% (10/76) had Alzheimer's disease and 9.21% (7/76) had mixed dementia. Hypertension, hyperlipidemia and diabetes mellitus were commoner in the vascular dementia group as compared to the Alzheimer's group. Evaluation of MRI as a tool in diagnosis of dementia showed increased sensitivity of MRI towards detecting lacunes. The potentially reversible dementias comprised infections 14.47% (11/76), metabolic-nutritional 14.47% (11/76) and autoimmune diseases 3.94% (3/76). These were characterized by a subcortical dementia. Four month follow up of MMSE in this group showed significant and sustained improvement in the metabolic nutritional group.

ARDS in a case of vesicular mole with secondary hyperthyroidism.

Vesicular mole is best regarded as a benign neoplasia of the chorion with malignant potentials. In India, the prevalence is one in four hundred pregnancies.We present one such case who presented with thyrotoxicosis and pulmonary edema.

Epidemic of leptospirosis: an ICU experience.

AIMS: To study the clinical profile and outcome of critically ill patients suffering from leptospirosis with organ dysfunction and correlate mortality with individual risk factors. METHODS: A study of critically ill patients suffering from leptospirosis was carried out in the Medical Intensive Care Unit of a tertiary centre of a metropolitan city between 1st June 2002 and 31st May 2003. All the patients in whom diagnosis was confirmed by ELISA IgM antibody testing underwent thorough clinical examination and necessary biochemical investigations. They received standard antimicrobial therapy and extensive supportive therapy as required. Mortality was correlated with individual risk factors. RESULTS: Out of 834 total admissions in this period, 60 (7.2%) patients suffered from leptospirosis. There were 48 males and 12 females with age ranging from 12 to 60 years, mean age being 40 years. The clinical manifestations varied from fever (58 patients), jaundice (38), subconjunctival haemorrhages (24), to altered sensorium (22). All the patients had evidence of severe sepsis. Forty six patients had multiple organ dysfunction syndrome (MODS) and 26 required ventilatory support. The total mortality in leptospirosis patients was 52% which was much higher compared to the total MICU mortality (31.4%) in the same period. CONCLUSION: Leptospirosis is an important infection associated with high mortality when associated with organ dysfunction. The poor prognostic factors are preponderance of male sex, alcohol dependence, age group > 50 years, MODS, acute respiratory distress syndrome (ARDS), presence of acidosis and need for mechanical ventilation. However, timelyintervention and intensive therapy can save many young lives.

Use of mobile phones in ICU--why not ban?

Due to the rapid growth of mobile telecommunications it is predicted that by 2005 there will be 1.6 billion mobile phone users worldwide. The usage of cellphones in Intensive Care Units carries with it a high incidence of interference with a number of medical devices like implantable defibrillators, cardioverters, pacemakers, monitors and other important devices like ventilators. It is in this context that this article will throw a light on complications of cellphones use in the Intensive Care Units and various strategies that can be taken to restrict their use in the Intensive Care Units.

Evaluation of efficacy and tolerability of Losartan and Ramipril combination in the management of hypertensive patients with associated diabetes mellitus in India (LORD Trial).

AIM: The study was conducted to evaluate efficacy and tolerability of fixed dose combination (FDC) of Losartan and Ramipril in the management of mild to moderate hypertensive Native Asian Indian patients with associated diabetes mellitus. The secondary objective was to evaluate the efficacy of the combination in reducing microalbuminuria. MATERIAL AND METHODS: The study was an open, non-comparative, multicentric clinical trial conducted in seven Indian centres in 315 eligible patients. All the patients were treated with Losartan 50 mg + Ramipril 2.5 mg or Losartan 50 mg + Ramipril 5 mg once a day in 12 weeks and consisted of a total of eight visits. RESULTS: The mean age of patients was 52.93 years (range 45 - 60 years). Of the total patients, 62.86% were males and 37.14% were females. The mean prestudy systolic blood pressure was 160.56 +/- 14.44 which was significantly reduced to 126.85 +/- 9.78 at the end of 12 weeks (P < 0.001). Similarly the mean diastolic blood pressure was 98.91 +/- 8.33 at baseline (stage I) which was significantly reduced to 79.82 +/- 5.42 at the end of 12 weeks (P < 0.001). A mean fall of 33.72 mmHg in systolic blood pressure and the mean fall of 19.10 mmHg was observed in systolic and diastolic blood pressure respectively at the end of the treatment which was statistically highly significant (P < 0.001). The JNC-VII goal of blood pressure < 130/80 was achieved in 79.05% patients after the treatment which losartan and ramipril combination only. Microalbuminuria (urinary albumin excretion > 30 but < 300 mg/day) was seen in 83/250 (33.2%) patients and 135 (54%) patients had clinical proteinuria (albuminuria) at baseline. At the end of the therapy 20.8% patients achieved normoalbuminuria. Good to excellent efficacy response was reported in 98.09% patients and 98.41% patients reported good to excellent tolerability to the treatment. CONCLUSION: The fixed dose combination of Losartan and Ramipril showed good to excellent efficacy response in 98.10% patients and achieved a target blood pressure of 130/80 mmHg in 79.05% patients in 12 weeks. The combination reduced the urinary albumin excretion in majority of the patients with microalbuminuria and proteinuria (the major marker of nephropathy).

Olivopontocerebellar atrophy presenting with stridor.

The spectrum of degenerative ataxia includes the symptomatic degenerative ataxias and the primary degenerative ataxias. The later may be sporadic and idiopathic or hereditary, being genetically determined. When an individual ataxic patient presents with an adult-onset degenerative ataxia and has a negative family history, the physician is faced with a diagnosis of pure idiopathic sporadic degenerative ataxia or one of the hereditary ataxias. The clinical spectrum of olivopontocerebellar atrophy (OPCA) usually consists of pancerebellar signs with pyramidal and abnormal eye movements. Although Stridor is more commonly found in multisystem atrophy, it is rarely seen in OPCA. We, here report a case of third decade onset of ataxia presenting with stridor.

An outbreak of leptospirosis in Mumbai.

Following prolonged water logging due to heavy rainfall in Mumbai during July 2000, a total of 102 patients clinically suspected of leptospirosis were admitted in our hospital. Blood samples were examined for the presence of leptospires by dark ground microscopy (DGM) and IgM antibodies were detected by ELISA. Out of 102 blood samples, 37 were positive by ELISA giving a positivity rate of 36.27%. Of these, only 24 were positive by DGM. Out of the positive cases, 37.83% had respiratory symptoms, 32.43% each had jaundice and conjunctival suffusion and 16.21% had renal involvement. Mortality amongst the positive cases was 10.81%. Apart from hepatic and renal involvement, respiratory symptoms due to leptospirosis are on the rise.