Interêt de l\'electroencephalogramme(EEG) dans le diagnostic de la Maladie de Creutzfeldt-Jakob (MCJ) en Afrique. Description de trois cas en Côte D\'Ivoire

Aka-Diarre, E; Sonan-Douayoua, Thérèse; Assi, B.; Kouame-Assouan, Ange-Eric; Doumbia, Moussa; Datié, A.; Yapo, Félix Boa; Kouassi, EB

doi:10.4314/ajns.v26i2.7600

Cited by 3 publications

(3 citation statements)

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“…The annual incidence of sCJD is approximately 1 per million population, but the epidemiology of sCJD is poorly reported outside Europe, Australia and North America. 5 From the African continent, there are only a few reported cases from North, 6 West, 7 and East 8 Africa, with one iatrogenic case from South Africa. 9 Out of the 18 published cases from Africa, only 2 had MRI brain imaging, of which none showed pathognomonic changes, and none of the patients were reported to present with the Heidenhain variant of sCJD.…”

Section: Introductionmentioning

confidence: 99%

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

Sokhi¹,

Yakub²,

Sharma³

et al. 2021

IMCRJ

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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.

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Section: Introductionmentioning

confidence: 99%

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

Sokhi¹,

Yakub²,

Sharma³

et al. 2021

IMCRJ

View full text Add to dashboard Cite

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“…It is a relatively infrequent and universally fatal disease with an annual incidence of approximately 1–2 per million worldwide but is on the increase 4. Only 23 cases, mostly of sporadic CJD, have been reported from Africa,5–12 although genetic CJD has been reported historically in migrant populations coming from North Africa 13. Readers need little reminding of the dangers of transmissible prion disease, with the outbreak of bovine spongiform encephalopathy in the UK in the 1990s 14.…”

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confidence: 99%

“…The occurrence of CJD is likely to be underestimated in Africa. Apparent limitations are evidenced by the relatively small number of histologically proven cases9 10 12 and just a single recent case report using the RT-QuIC test, accessed abroad 5 6…”

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confidence: 99%