Syndromes of Rapidly Progressive Cognitive Decline-Our Experience

Abstract

Background:Dementias are fairly slowly progressive degenerative diseases of brain for which treatment options are very less and carry a lot of burden on family and society. A small percentage of them are rapidly progressive and mostly carry a different course outcome. However, there are no definite criteria other than the time line for these patients.Aims:The aim of this was to identify and categorize the causes and course of rapidly progressive dementias seen in our center.Settings and Design:Patients who presented with rapid deterioration of cognitive functions within weeks to 1 year between 2011 and December 2016 were evaluated.Patients and Methods:All patients underwent all mandatory tests for dementia including brain imaging. Complete vasculitis workup, autoimmune encephalitis profile including Voltage Gated Potassium Channel, N-methyl-D-aspartic acid receptor, glutamic acid-decarboxylase, thyroid-peroxidase antibody, cerebrospinal fluid, and other special tests such as duodenal biopsy and paraneoplastic workup were done based on clinical indications.Results and Conclusions:Out of 144 patients 42 had immune-mediated encephalopathy, 18 had Creutzfeldt-Jakob disease, 3 had Vitamin B12 deficiency, 63 had infection with neurocysticercosis, 7 had tuberculosis, 2 had HIV, 1 had herpes simplex encephalitis, 1 had neurosyphilis, 1 Whipples disease, 1 had Subacute Sclerosing Panencephalitis, 1 had Mass lesion, 3 had Frontotemporal dementia, and 3 had small vessel disease. Good majority of these patients have infective and immune-mediated causes and less number belong to degenerative group. Therefore, caution is needed to look for treatable cause as it carries a different treatment options and outcome.

Cognitive and behavioral profile of treatment-naïve children aged 6–14 years with neurocysticercosis from North India

“…Few authors believe in the complete workup of children and adults with cognitive and behavioral disturbances including autoimmune, paraneoplastic, and vasculitic workup. [ 17 ] These study findings could possibly allay the fear of epidemiologist who has attributed behavioral changes and suboptimal learning and intelligence of school-going children to the endemicity of T. solium .…”

Pearls & Oy-sters: Rapidly Reversible Dementia

“…B 12 deficiency constitutes a minority (1%-4%) of RPDs. 2 It is often challenging to identify potentially reversible B 12 -related dementia from the wide variety of disease conditions presenting as RPDs. The pattern of reversible cognitive impairment reported in B 12 deficiency is predominantly of frontotemporal type.…”

Rapid cognitive decline and myoclonus in a 52-year-old woman

“…These include Whipple disease (subacute dementia, ataxia, and myoclonus), human immunodefi ciency virus (HIV) encephalitis, tuberculosis, herpes simplex encephalitis, and subacute sclerosing panencephalitis. 1,2 While not strictly caused by infectious pathogens, spongiform encephalopathies (also called prion diseases) such as fatal familial insomnia, kuru, and Creutzfeldt-Jakob disease (CJD) can present with rapid cognitive decline. Autoimmune etiologies should also be considered, including antibodies to both extracellular antigens such as Nmethyl-d-aspartate receptor and intracellular antigens such as Hu antigens.…”