A case of very rapid progressive ataxia in rehabilitation setting

Gialanella, Bernardo; Prometti, Paola; Ferlucci, Cristina; Bertolinelli, Maurizio

doi:10.1007/bf03325165

Cited by 5 publications

(9 citation statements)

References 17 publications

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“…In a sCJD case, reported by Gialanella et al [3], the patient did not show any functional improvement despite rehabilitation. Similarly, functional improvement was not observed after rehabilitation in our case.…”

Section: Discussionmentioning

confidence: 84%

“…The median age of onset is 65 years, and the median duration of survival is four months [1]. It is due to the action of neurolytic pathogen proteins, called prions, which gradually damage central nervous system cells resulting in brain damage with distinct pathologic features [3]. The well-known clinical manifestations of sCJD are rapidly progressive dementia, myoclonus, and ataxia.…”

Section: Discussionmentioning

confidence: 99%

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Rapidly progressive tetraplegia and cognitive deterioration during rehabilitation: A case of neurodegenerative disease

Afşar¹,

Yemişçi²

2019

Journal of Surgery and Medicine

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How to cite / Atıf için: Afşar Sİ, Yemişçi OÜ. Rapidly progressive tetraplegia and cognitive deterioration during rehabilitation: A case of neurodegenerative disease.This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-NoDerivatives License 4.0 (CC BY-NC-ND 4.0) where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal. AbstractHuman prion diseases are fatal, progressive neurodegenerative disorders caused by neurolytic pathogen proteins, called prions. The most common human prion disease is sporadic Creutzfeldt-Jakob disease, with an approximate annual prevalence of 0.5-1 per million. The symptoms and signs include rapidly progressive dementia, ataxia, myoclonic seizures, akinetic mutism and other neurological and neurobehavioral disorders. The clinical spectrum of Creutzfeldt-Jakob disease is highly variable; therefore it can be difficult to diagnose premortem. This article describes a 78-yearold woman who initially presented with difficulty walking and balance disorder. As a result of the evaluation, the patient was transferred to rehabilitation clinic, with a diagnosis of cervical spinal stenosis. During hospitalization, she showed progressive decline in gait and balance and deteriorated rapidly. The patient was considered to be probable sporadic Creutzfeldt-Jakob disease after further investigations. Özİnsan prion hastalıkları, prionlar olarak adlandırılan nörolitik patojen proteinlerin neden olduğu ilerleyici nörodejeneratif hastalıklardır. En yaygın insan prion hastalığı sporadik Creutzfeldt-Jakob hastalığı olup, yıllık prevalansı yaklaşık milyonda 0.5-1'dir. Semptomlar ve bulgular; hızla ilerleyen demans, ataksi, miyoklonik nöbetler, akinetik mutizm ve diğer nörolojik ve nörodavranışsal bozuklukları içerir. Creutzfeldt-Jakob hastalığının klinik spektrumu oldukça değişkendir, bu nedenle premortem teşhis etmek zor olabilir. Bu makalede, başlangıçta yürüme zorluğu ve denge bozukluğu ile başvuran 78 yaşında bir kadın hasta anlatılmaktadır. Yapılan değerlendirme sonucunda hasta servikal spinal stenoz tanısı ile rehabilitasyon kliniğine transfer edildi. Hastanede yatışı sırasında, yürüme ve dengesi progresif olarak kötüye giderek bozuldu. İleri tetkiklerden sonra hastanın olası sporadik Creutzfeldt-Jakob hastalığı olduğu düşünülmüştür. Anahtar kelimeler: Nörodejeneratif hastalık, Creutzfeldt-Jakob hastalığı, Rehabilitasyon

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Section: Discussionmentioning

confidence: 84%

Section: Discussionmentioning

confidence: 99%

Rapidly progressive tetraplegia and cognitive deterioration during rehabilitation: A case of neurodegenerative disease

Afşar¹,

Yemişçi²

2019

Journal of Surgery and Medicine

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“…Our patient's MRI findings were typical with cortical ribbon hyperintensities, a highly sensitive finding in sCJD [ 9 ]. Also, S100B protein is a biomarker that has 65–90% sensitivity and specificity as high as 90% in diagnosing sCJD; however, it has been proposed to have greater diagnostic utility when combined with typical proteins, such as 14-3-3 [ 3 , 4 ]. S100B is not typically used alone for a diagnosis [ 10 ].…”

Section: Resultsmentioning

confidence: 99%

“…Delays in diagnosis and misdiagnosis are not uncommon since sCJD mimics other neurologic disorders, including autoimmune encephalitis, infectious encephalitis, and stroke. There is no proven effective therapy, but an early diagnosis allows patients and their families to prepare for the predictable clinical progression and avoid inappropriate interventions [ 3 ]. We present a case of sCJD diagnosed clinically as a stroke months before presenting with end-stage disease.…”

Section: Introductionmentioning

confidence: 99%

Rapidly Progressing Sporadic Creutzfeldt-Jakob Disease Presenting as a Stroke

et al. 2018

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Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, fatal human prion disease that is characterized by progressive dementia and neurologic degeneration. It can mimic multiple other neurological disorders, and a high index of clinical suspicion is necessary to make a diagnosis. A 74-year-old woman with a 3-month history of a stroke and progressive neurologic deterioration was found to have sCJD. She expired within a week of her diagnosis. Autopsy revealed spongiform encephalopathy consistent with prion disease, and genetic analysis revealed 129 polymorphism and no pathologic mutation, confirming the diagnosis of nonfamilial human prion disease. No pathologic evidence of a stroke was found. Awareness of the disease by clinicians is important not only at the time of initial presentation but also during the following months. Since there is no treatment, invasive medical procedures should be limited to only those that are required for either diagnosis or hospice care.

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“…Furthermore, the variability of initial symptoms and rapid progression of the diseases means patients are often misdiagnosed or identified in the later stages. 5 Reports in the literature have previously described the misdiagnosis of CJD with a number of conditions, one of which includes stroke. 5,6 In these situations, misdiagnosis can result in detrimental affects on the patient's remaining quality of life.…”

Section: Introductionmentioning

confidence: 99%

When is a stroke not a stroke? An unusual mimic presenting to AMU

et al. 2016

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Introduction: Creutzfeldt-Jakob disease (CJD) is a rare prion disease classically manifesting with rapidly progressive dementia, abnormal movements and typical electroencephalographic (EEG) changes. Case Report: A 74 year-old Caucasian man was recently discharged from another centre diagnosed with a stroke. He re-presented to our acute medical unit with worsening symptoms, and stroke remained the working diagnosis. Collateral history revealed a progressive cognitive decline and unilateral myoclonus. Further investigations supported the diagnosis of probable CJD, confirmed by the national CJD surveillance centre. Discussion: Signs and symptoms atypical of stroke should raise the possibility of alternative diagnoses, including prion disease. CJD can present with unilateral symptoms, EEG and MRI changes. Early diagnosis prevents unnecessary investigations and treatments, allowing early palliative care input, where appropriate.

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A case of very rapid progressive ataxia in rehabilitation setting

Abstract: Our case report suggests that sporadic CJD must be hypothesized when ataxia worsens rapidly and severely despite rehabilitation.

Cited by 5 publications

References 17 publications

Rapidly progressive tetraplegia and cognitive deterioration during rehabilitation: A case of neurodegenerative disease

Rapidly progressive tetraplegia and cognitive deterioration during rehabilitation: A case of neurodegenerative disease

Rapidly Progressing Sporadic Creutzfeldt-Jakob Disease Presenting as a Stroke

When is a stroke not a stroke? An unusual mimic presenting to AMU

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