Adult Onset Alexander’s Disease (AOAD) is caused by mutation of the glial fibrillary acidic protein (GFAP) gene, leading to astrocyte dysfunction. The disease is often associated with the classic MRI findings of medullary and cervical cord atrophy with corresponding T2 hyperintense signal changes with or without enhancement. We describe a 67-year-old woman who presented with progressive neurolo...

The porphyrias are diseases characterised by accumulation of porphyrins and porphyrin precursors owing to enzymatic deficiencies of the haem synthetic pathway. In the acute hepatic porphyrias accumulation of porphyrin precursors, in particular delta-aminolaevulinic acid (ALA), cause dysfunction of the central, peripheral and autonomic nervous systems. This leads to the characteristic clinical f...

Transient abnormalities in ECGs, echocardiograms, and cardiac enzymes have been described in the acute setting of subarachnoid haemorrhage. In addition, left ventricular dysfunction has been reported at the time of brain death. A patient with an acute subarachnoid haemorrhage who presented with raised troponin I (TnI) concentrations and diffuse left ventricular dysfunction is described. After d...

A 25-year-old man, a field operator in a petroleum refinery was found unconscious. He was exposed to hydrogen sulfide and presented with Glasgow Coma Score of 5, severe hypoxemia on arterial blood gas analysis, normal chest radiography, and normal blood pressure. On hospital day 7, his mental state became clear, and neurologic examination showed quadriparesis, profound spasticity, increased ten...

Cerebral dysfunction and injury in the ICU presents as focal neurologic deficits, seizures, coma, and delirium. These syndromes may result from a primary brain insult, such as stroke or trauma, but commonly are a complication of a systemic insult, such as cardiac arrest, hypoxemia, sepsis, metabolic derangements, and pharmacologic exposures. Many survivors of critical illness have cognitive imp...

It is estimated that hereditary hearing loss accounts for 60% of deafness in the developed countries. About 30% of hereditary hearing impairment is syndromic which involves other presenting abnormalities along with deafness. There are more than 400 syndromes which include various degrees of hearing impairment with different phenotypes. (Barlow Stewart et al., 2007; Berrettini et al., 2008). Abn...

Transient abnormalities in ECGs, echocardiograms, and cardiac enzymes have been described in the acute setting of subarachnoid haemorrhage. In addition, left ventricular dysfunction has been reported at the time of brain death. A patient with an acute subarachnoid haemorrhage who presented with raised troponin I (TnI) concentrations and diVuse left ventricular dysfunction is described. After de...

Cardiac arrest (CA) is a leading cause of mortality worldwide. The majority of the associated mortalities are caused by post‑CA syndrome, which includes symptoms, such as neurologic damage, myocardial dysfunction and systemic inflammation. Following CA, return of spontaneous circulation (ROSC) leads to a brain reperfusion injury, which subsequently causes adverse neurologic outcomes or mortalit...

BACKGROUND There is paucity of research to quantify the severity and to predict the mortality risk of severe falciparum malaria even if it affects multiple organ systems during the course of the disease. Therefore, the aim of the present study is to develop a severity score for assessment of disease severity and risk prediction in adult patients of severe falciparum malaria on the first day of ...

Intraoperative nerve dysfunction has been difficult to investigate because of its rarity and unpredictable occurrence. The diagnostic test attributes function monitors have not clearly defined. This proof-of-concept study aimed assess the feasibility using brachial plexus blockade (BPB) in awake patients as an experimental model for characterize somatosensory evoked potentials (SSEPs). We obtai...