The Heidenhain variant of Creutzfeldt-Jakob disease

Muniz, Bernardo Carvalho; Makita, Lana Sayuri; Ribeiro, Bruno Niemeyer de Freitas; Marchiori, Edson

doi:10.1590/0100-3984.2017.0166

Cited by 10 publications

(12 citation statements)

References 9 publications

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“…CJD can be classified as sporadic, which is the most common form, accounting for 85% of cases; inherited, which is caused by various heritable mutations in the prion protein (PRNP) gene; iatrogenic, caused by inoculation of prions through contaminated materials; or a variant form (vCJD), which usually results from the transmission of bovine spongiform encephalopathy to humans, most commonly through the consumption of contaminated meat. 7 A limited number of conditions, all of which are relatively uncommon, produce a syndrome of rapidly progressive dementia that can be mistaken for CJD. Some are treatable; therefore, the evaluation should be thorough.…”

Section: Discussionmentioning

confidence: 99%

“…The classic clinical manifestation of HvCJD is cortical blindness, due to involvement of the parieto-occipital cortex. 7 Isolated visual symptoms including poor vision, disturbed perception of colors or structures, visual defects, hemianopsia, visual agnosia, abnormal color/spatial perception and optical distortions, as well as optical hallucinations without any ocular disease, may also occur. 1,11 At disease onset, patients apparently not fully demented typically give up reading or watching television due to visual impairment, with visual field restriction, blurred vision, vision loss, or even total blindness.…”

Section: Discussionmentioning

confidence: 99%

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Heidenhain Variant of Creutzfeldt-Jakob Disease in Brazil: A Case Report

Mendonça¹,

Saffi²,

Louzada³

et al. 2020

Geriatr., Gerontol. Aging

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A doença de Creutzfeldt-Jakob (DCJ) é uma encefalopatia rara caracterizada por rápida progressão neurodegenerativa, causada pelo enovelamento incorreto da proteína priônica celular (PrP), conhecido como PrPSc. O quadro clínico da DCJ esporádica inclui um amplo espectro de sinais neurológicos de origens cortical, subcortical ou cerebelar, seja de forma isolada, seja combinada. Por causa da sua apresentação clínica variável, a DCJ esporádica deve ser distinguida de outras demências. Neste relato de caso, discutimos a variante Heidenhain da DCJ (vHDCJ), uma variante rara caracterizada por sintomas visuais precoces e características específicas no exame de imagem. Nossa paciente apresentou demência rapidamente progressiva e histórico de alucinações visuais. Assim como para as demais doenças priônicas, apenas o tratamento sintomático está disponível para a vHDCJ. Trinta anos de investigação clínica de pacientes com doença priônica têm resultado em pouco progresso, seja definindo os potenciais tratamentos, seja avaliando-os.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Heidenhain Variant of Creutzfeldt-Jakob Disease in Brazil: A Case Report

Mendonça¹,

Saffi²,

Louzada³

et al. 2020

Geriatr., Gerontol. Aging

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“…Recent studies conducted in Brazil have highlighted the importance of imaging methods in the evaluation of diseases affecting the central nervous system (24)(25)(26)(27)(28)(29) . Spectroscopy in the posterior cingulate gyrus is extensively studied in Alzheimer's disease, in which previous studies showed lower NAA/Cr ratio in patients with Alzheimer's disease than in individuals with mild cognitive impairment and controls.…”

Section: A B Cmentioning

confidence: 99%

Posterior cingulate gyri metabolic alterations in HIV-positive patients with and without memory deficits

et al. 2020

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Objective: We aimed to evaluate whether human immunodeficiency virus (HIV)-positive patients with and without clinically significant memory deficits and healthy control participants differ on in vivo hydrogen-1 magnetic resonance spectroscopy (H-MRS) in the posterior cingulate gyri. Materials and Methods: In total, 21 HIV-positive patients with memory deficit (HIV+wMD) were compared with 15 HIV-positive patients without memory deficit (HIV+wOMD) and 22 sex-, age-, and education-matched control participants. Memory impairments were classified based on the participants’ performance on the Rey Auditory Verbal Learning Test. Short echo time (30 ms), single-voxel H-MRS was performed using a 1.5-T magnetic resonance scanner. Results: The HIV+wMD and HIV+wOMD groups had higher choline/creatine ratio in the posterior cingulate gyri than the control group. There were no significant metabolite ratio differences between the HIV+wMD and HIV+wOMD groups. Conclusion: HIV-positive patients with and without memory deficits had significantly higher choline/creatine ratios than controls in the posterior cingulate gyri, which may reflect cerebral inflammation, altered cell membrane metabolism, microgliosis, and/or astrocytosis.

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“…A series of recent studies conducted in Brazil have highlighted the importance of MRI in the assessment of diseases of the nervous system ( 2 - 7 ) . In this pictorial essay, we illustrate the main causes of non-neoplastic intracranial cystic lesions, discussing their possible differential diagnoses as well as their most relevant imaging aspects.…”

Section: Introductionmentioning

confidence: 99%

Non-neoplastic intracranial cystic lesions: not everything is an arachnoid cyst

Pereira¹,

Ribeiro

Lima³

et al. 2021

Radiol Bras

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Intracranial cystic lesions are common findings on neuroimaging examinations, arachnoid cysts being the most common type of such lesions. However, various lesions of congenital, infectious, or vascular origin can present with cysts. In this pictorial essay, we illustrate the main causes of non-neoplastic intracranial cystic lesions, discussing their possible differential diagnoses as well as their most relevant imaging aspects.

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The Heidenhain variant of Creutzfeldt-Jakob disease

Cited by 10 publications

References 9 publications

Heidenhain Variant of Creutzfeldt-Jakob Disease in Brazil: A Case Report

Heidenhain Variant of Creutzfeldt-Jakob Disease in Brazil: A Case Report

Posterior cingulate gyri metabolic alterations in HIV-positive patients with and without memory deficits

Non-neoplastic intracranial cystic lesions: not everything is an arachnoid cyst

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