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Heidenhain Variant of Sporadic Creutzfeldt-Jakob Disease: First Reported Case from East Africa

Authors Sokhi D, Yakub F, Sharma K, Waa S, Mativo P

Received 17 October 2020

Accepted for publication 19 December 2020

Published 2 February 2021 Volume 2021:14 Pages 39—44


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Professor Ronald Prineas

Dilraj Sokhi,1 Fazal Yakub,1 Karishma Sharma,1 Sheila Waa,2 Peter Mativo1

1Department of Medicine, Aga Khan University Hospital, Aga Khan University Medical College of East Africa, Nairobi, Kenya; 2Department of Radiology, Aga Khan University Hospital, Aga Khan University Medical College of East Africa, Nairobi, Kenya

Correspondence: Dilraj Sokhi
Department of Medicine, Aga Khan University Hospital, Aga Khan University Medical College of East Africa, Room 405, 4th Floor East Tower Block, Third Avenue Parklands, P.O. Box 30270, Nairobi, 00100 GPO, Kenya
Tel +254 710 559 541

Abstract: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare prion disease that causes rapidly progressive fatal neurodegeneration. The rarer Heidenhain variant of sCJD presents with visual symptoms and is rarely reported in the literature from sub-Saharan Africa. We report the case of a 57-year-old male with a three-week history of losing direction when driving home and visual hallucinations described as seeing rainbows. Magnetic resonance imaging (MRI) of the brain revealed unilateral parieto-occipital sulcal hyperintensities with restriction on diffusion-weighted imaging (DWI), and electroencephalography (EEG) showed right para-central slowing leading to an initial diagnosis of non-convulsive status epilepticus. He was treated with anti-epileptic medication but was re-admitted less than a month later with worsening spatial memory, aggression, ataxia, dysarthria, myoclonic jerks and a positive startle response, later developing generalised tonic-clonic seizures. Repeat MRI brain scan showed widespread posterior-predominant sulcal DWI restriction in a cortical ribboning pattern pathognomonic for sCJD. EEG showed diffuse slowing, and cerebrospinal fluid was analyzed for abnormal prion protein using real-time quaking-induced conversion but was inconclusive due to suboptimal sample collection. The patient fulfilled the diagnostic criteria for probable sCJD, Heidenhain variant; the family declined brain biopsy for definitive diagnosis. He was subsequently palliated at a local hospice where he died approximately three months after the onset of symptoms. Our case highlights the presence of a rare form of sCJD, and the diagnostic challenges faced in our resource-limited setting.

Keywords: Creutzfeldt-Jakob disease, prion, Heidenhain variant, sub-Saharan Africa

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