Atypical subacute sclerosing panencephalitis with short onset latency

Sachan, Deepak

doi:10.1007/s13312-013-0242-6

Cited by 5 publications

(3 citation statements)

References 3 publications

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“…In the fourth case, an 8‐year‐old unimmunized child developed acute onset dystonia which evolved into status dystonicus over 2 weeks. In this patient, the MRI showed discrete hyperintensities involving bilateral occipital lobes and left globus pallidus 45 . In a report of a 9‐year‐old boy, after 5 months history of myoclonus, pathological laughter/crying, he presented with severe axial dystonic storm with flexion of upper limbs, opisthotonic posturing and extension of both lower limbs.…”

Section: Resultsmentioning

confidence: 69%

Movement Disorders in Patients with Subacute Sclerosing Panencephalitis: A Systematic Review

Garg,

Patel,

Sankhla

et al. 2024

Movement Disord Clin Pract

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BackgroundSubacute sclerosing panencephalitis (SSPE) is a complication of measles, occurring after a latency of 4–10 years. It continues to occur in developing countries although resurgence is being reported from developed countries. Characteristic features include progressive neuropsychiatric issues, myoclonus, seizures, movement disorders and visual impairment. Electroencephalography (EEG) typically shows periodic generalized discharges, and elevated CSF anti‐measles antibodies are diagnostic. Movement disorders are being increasingly recognized as part of the clinical spectrum, and range from hyperkinetic (chorea, dystonia, tremor, tics) to hypokinetic (parkinsonism) disorders and ataxia.ObjectivesThis article aims to comprehensively review the spectrum of movement disorders associated with SSPE.MethodsA literature search was conducted in PubMed and EMBASE databases in December 2023 and articles were identified for review.ResultsMovement disorders reported in SSPE included hyperkinetic (chorea, dystonia, tremor and tics), hypokinetic (parkinsonism), ataxia and extraocular movement disorders. Myoclonus, a core clinical feature, was the most frequent “abnormal movement.” Movement disorders were observed in all clinical stages, and could also be a presenting feature, even sans myoclonus. Hyperkinetic movement disorders were more common than hypokinetic movement disorders. An evolution of movement disorders was observed, with ataxia, chorea and dystonia occurring earlier, and parkinsonism later in the disease. Neuroradiological correlates of movement disorders remained unclear.ConclusionA wide spectrum of movement disorders was observed throughout the clinical stages of SSPE. Most data were derived from case reports and small case series. Multicentric longitudinal studies are required to better delineate the spectrum and evolution of movement disorders in SSPE.

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Section: Resultsmentioning

confidence: 69%

Movement Disorders in Patients with Subacute Sclerosing Panencephalitis: A Systematic Review

Garg,

Patel,

Sankhla

et al. 2024

Movement Disord Clin Pract

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“…Atypical subacute sclerosing panencephalitis (SSPE), a neurodegenerative disease related with MV, is not included in AMS. However, it differs from the classical form of SSPE by occurring in adults, by having a very short latency or showing atypical clinical or biologic features [54][55][56]. It is of note, that SSPE, is the only form of the measles complex in which a proven persistent MV infection has been determined.…”

Section: New Aspects Of the Researchmentioning

confidence: 99%

Relevance of the Measles Virus Expression in Cancer - an Update

Benharroch

Ariad

Tadmor

et al. 2016

Pathol. Oncol. Res.

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Evidence of an association between classical Hodgkin lymphoma and the measles virus has previously been presented by our group. Arguments held against our thesis were reevaluated. Substantiation of a relationship between the measles virus and additional solid tumors was submitted. Moreover, a pathogenic pathway was suggested to support a possible contribution of the measles virus to the development of classical Hodgkin lymphoma. We have chosen to exclude a discussion of measles virotherapy, since this carries distinct implications. We now add new evidence regarding the expression of the measles virus phosphoprotein in a few cancers. We also suggest a role in this context for atypical measles syndrome in malignant tumors. Last, we propose a collaboration which may make the best, on the one hand of our cohort of classical Hodgkin lymphoma, half of which carry the measles virus expression in their tumor cells. The planned study will also look into the patients vaccination records and into a previous history of the measles disease. On the other hand, cohorts of patients diagnosed with late onset measles will be assessed for the eventual diagnosis of atypical measles syndrome and will be followed up for the subsequent development of a malignant tumor.

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“…Many Indian SSPE children are lost to follow-up in the wake of being told the prognosis of the disease. With increasing awareness, more children with SSPE with atypical presentations are being seen [5][6][7]. Despite the high incidence of SSPE in India, an accurate and early diagnosis remains a challenge.…”

mentioning

confidence: 99%

Myoclonic-Atonic Epilepsy Masquerading as Subacute Sclerosing Panencephalitis: A Clinical Conundrum

et al. 2022

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Letter to the editorMyoclonic epilepsy of infancy, myoclonic-atonic epilepsy (MAE) with onset in early childhood, and later-onset syndromes such as juvenile myoclonic epilepsy, eyelid myoclonic epilepsy, and myoclonic absence epilepsy are all examples of childhood-onset myoclonic epilepsy syndromes. Degenerative brain disorders, subacute sclerosing panencephalitis (SSPE), autoimmune disorders, and a few mitochondrial abnormalities are among the other uncommon causes. There have been attempts to define hereditary myoclonic epilepsies that do not meet the recognised criteria for myoclonic epilepsy syndromes [1]. The cognitive outcomes of epilepsy syndromes vary, but in general, they have a good prognosis. Myoclonic-atonic epilepsy is classified as an epileptic encephalopathy, and the cognitive outcome can be normal to severely impaired [2]. SSPE, in contrast, is a debilitating neurological illness that has so far eluded treatment. It is characterised by cognitive decline, periodic myoclonus, ataxia, vision complaints, a vegetative state, and death [3]. Clinicians usually identify SSPE using a combination of classic clinical features paired with elevated anti-measles antibody titres in the cerebrospinal fluid (CSF). Thus, a problem arises when a clinician is faced with a case of SSPE presenting in its early stages or with atypical www.annchildneurol.org 208

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Atypical subacute sclerosing panencephalitis with short onset latency

Cited by 5 publications

References 3 publications

Movement Disorders in Patients with Subacute Sclerosing Panencephalitis: A Systematic Review

Movement Disorders in Patients with Subacute Sclerosing Panencephalitis: A Systematic Review

Relevance of the Measles Virus Expression in Cancer - an Update

Myoclonic-Atonic Epilepsy Masquerading as Subacute Sclerosing Panencephalitis: A Clinical Conundrum

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