ArticleEpilepsy & behavior reports2021
Juvenile Huntington's disease masquerading as progressive myoclonus epilepsy.
Article in Epilepsy & behavior reports, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
7 citing papers in PubMed, 7 citations in OpenAlex.
- Review
- Early Manifestations, Diagnostic Pathways, and Epilepsy in Juvenile-Onset Huntington Disease: A Three-Patient Case Series and Systematic Review.Brain sciences · 2026Article
- Movement Disorders in Developmental and Epileptic Encephalopathies.Movement disorders clinical practice · 2026Review
- Clinical Review of Juvenile Huntington's Disease.Journal of Huntington's disease · 2024Review
- Article
- The involvement of Purkinje cells in progressive myoclonic epilepsy: Focus on neuronal ceroid lipofuscinosis.Neurobiology of disease · 2023Review
- Forward Genetics-Based Approaches to Understanding the Systems Biology and Molecular Mechanisms of Epilepsy.International journal of molecular sciences · 2023Review
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Juvenile Huntington's disease (JHD) has an onset before 20 years of age, and is characterized by behavioural issues, epilepsy, rigidity, bradykinesia and dystonia. It contributes to 0.5-5% of all Huntington disease (HD) cases. JHD demonstrates a more rapid progression and is characterised by dystonia, as opposed to the slow progression with predominant chorea seen in adult-onset HD. Seizures are described in 38% of JHD as compared to 2% in the adult onset HD. The different types of seizures reported in JHD are generalized seizures, myoclonus, absence seizures and less commonly tonic and focal seizures with impaired awareness. JHD patients have good seizure control initially and develop drug-resistant epilepsy in the later stages of the disease which is rarely reported. Here, we report the case of a 13 -year-old boy, who initially presented with generalized tonic-clonic seizures followed by myoclonic jerks, with subsequent cognitive decline, ataxia, involuntary movements and drug resistant epilepsy mimicking a progressive myoclonus sepilepsy. His EEG changed from normal background with generalized interictal epileptiform discharges to diffuse slowing with fast activity devoid of epileptiform activity to reflect electroclinical evolution of the disease process.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.