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Showing posts from August, 2023

Normal Pressure Hydrocephalus

Normal Pressure Hydrocephalus Overview Normal pressure hydrocephalus is a condition that is not due to a block within the ventricular system. Still, there is an obstruction in the subarachnoid space, preventing cerebrospinal fluid (CSF) from flowing over the ventricles. This condition is more common in the elderly. Aetiology A history of subarachnoid hemorrhage (SAH), head injury, or meningitis may be associated with the development of normal pressure hydrocephalus. Clinical Features The clinical features of normal pressure hydrocephalus include progressive memory impairment and dementia, slowness, marked unsteadiness of gait, and urinary incontinence. Investigations The removal of 50 ml of CSF can lead to a temporary improvement in cognition. CT or MRI can show dilatation of the ventricular system and relatively normal sulci. Periventricular lucencies can also be found on MRI. Management The treatment for normal pressure hydrocephalus involves the insertion of a shunt to improve the c

Creutzfeldt-Jakob Disease

Overview Creutzfeldt-Jakob Disease is a rapidly progressive degenerative disease of the nervous system that can be transmitted by blood or tissues between human beings. Epidemiology It is estimated that 50-100 cases occur each year in the UK, with an equal sex ratio and onset typically occurring between the ages of 40 and 60. Aetiology The disease is caused by the accumulation of an abnormal prion protein in the brain, which is encoded on chromosome 20. Those with the E4 allele of apolipoprotein are at a higher risk of developing the disease, and the familial form, known as Gerstmann-Straussler-Scheinker, accounts for 10% of patients and principally affects the cerebellum. It is also autosomal dominant. Clinical Features Clinical features of the disease can include personality changes, seizures, intellectual deterioration, and neurological deficits such as cerebellar ataxia, spasticity, extrapyramidal signs, and myoclonus sensitivity to noise or touch. In new-variant CJD (nvCJD), initi