Related Experiment Video
Updated: May 12, 2026

A Simple Composite Phenotype Scoring System for Evaluating Mouse Models of Cerebellar Ataxia
Published on: May 21, 2010
Segmental brainstem myoclonus in ADCK3-Related ataxia: A novel phenomenon?
Vito Luigi Colona1, Giacomo Garone2, Francesco Nicita3
1Unit of Neurorehabilitation, Bambino Gesù Children's Hospital, IRCCS, Rome, 00165, Italy.
Abstract:
Segmental Brainstem Myoclonus (SBM) is a rare movement disorder characterized by rhythmic contractions of muscles innervated by brainstem segments. We report a 20-year-old patient with ADCK3-related spinocerebellar ataxia type 9 (SCAR9) presenting with sudden-onset myoclonic movements of the throat, tongue, and soft palate. Brain MRI showed stable findings, including dentate nucleus hyperintensities. This case suggests a possible association between SBM and SCAR9, warranting further investigation.
Related Concept Videos
Seizures: Classification
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
Myasthenia Gravis ll: Pathophysiology
Alterations in Muscle Tone ll
Alterations in Muscle Tone lll

