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.

Keywords: Degenerations; immune-mediated; infections; nutritional; rapidly progressive dementia.

Figure 1

Distribution of various diseases among patients with rapidly progressive dementia

Figure 2

A case of familial Creutzfeldt-Jakob disease with trichotillomania

Figure 3

Cortical ribboning in diffusion-weighted images of a patient with Creutzfeldt-Jakob disease

Figure 4

Magnetic resonance imaging showing multiple neurocysticercosis

Figure 5

T2 fluid-attenuated inversion recovery images showing hyperintensity in the temporal lobe left more than right in a patient with herpes simplex encephalitis

Figure 6

T2 fluid-attenuated inversion recovery showing hyperintensities in thalamus, insula and both temporal lobes

Figure 7

Tuberculosis meningitis with hydrocephalus

Figure 8

Puckered skin lesions of Whipple disease

Figure 9

Angiosarcoma scalp deposit with anti-Ri antibody positive dementia

Figure 10

Susceptibility weighted sequence of MRI showing small and large bleeds in a case of vasculitis

Figure 11

Subacute sclerosing panencephalitis patient with classical periodic complexes

Figure 12

Sagittal brain section showing a large frontal mixed density lesion with a satellite lesion in the posterior frontoparietal region