Related Experiment Video
Updated: Aug 11, 2026

Pentylenetetrazole-Induced Kindling Mouse Model
Published on: June 12, 2018
Phenytoin-induced choreoathetosis in patients with severe myoclonic epilepsy in infancy
1Department of Pediatrics, Tokyo Women's Medical University, Shinjuku-ku, Tokyo, Japan. saitoyo@tmin.ac.jp
Insights
Phenytoin can cause choreoathetosis, a movement disorder, in patients with severe myoclonic epilepsy in infancy (SME). Reducing phenytoin dosage resolves this adverse effect, highlighting its importance in differential diagnosis.
Area of Science:
- Neurology
- Epileptology
- Pharmacology
Background:
- Severe myoclonic epilepsy in infancy (SME) is a severe form of epilepsy.
- Phenytoin is a commonly used antiepileptic drug.
Observation:
- Three patients with SME developed choreoathetosis after phenytoin dosage increases.
- Movement disorder onset ranged from 2 days to 6 months post-dosage adjustment.
- Choreoathetosis resolved upon reduction of phenytoin dosage.
Findings:
- Phenytoin-induced choreoathetosis is a potential adverse effect in SME patients.
- The episodic and paroxysmal nature of choreoathetosis can mimic degenerative disorders.
- Ictal SPECT in one patient showed basal ganglia hypoperfusion contralateral to choreoathetosis.
- Polypharmacy may increase the risk of developing this side effect.
Implications:
- Phenytoin-induced choreoathetosis should be considered in SME patients with involuntary movements.
- Early recognition and management are crucial to prevent misdiagnosis and ensure effective treatment.
- SME patients may have increased susceptibility to phenytoin-induced movement disorders, suggesting basal ganglia involvement in SME pathophysiology.
Abstract:
We describe three patients with severe myoclonic epilepsy in infancy (SME) who suffer from choreoathetosis due to the adverse effect of phenytoin. Choreoathetosis appeared when these patients were 8, 19, and 21 years old, 2 days to 6 months after increasing the phenytoin dosage. Choreoathetosis disappeared when the phenytoin dosage was decreased. The two elder patients experienced episodic and rather paroxysmal onset of long-lasting choreoathetosis, requiring the differential diagnosis from degenerative disease. In one of the patients, an ictal SPECT revealed decreased perfusion in the basal ganglia contralateral to the unilateral choreoathetosis. Polypharmacy, including carbamazepine and zonisamide, may have facilitated the onset of choreoathetosis. Phenytoin-induced choreoathetosis in the patients with SME is an important differential diagnosis among degenerative disorders involving involuntary movements. The episodic and paroxysmal nature of this movement disorder can delay its diagnosis and effective treatment. Patients with SME appear to be particularly vulnerable to this side effect of phenytoin, indicating the possible involvement of basal ganglia in the pathophysiology of this type of epilepsy.
Related Concept Videos
Epilepsy and Seizures: Overview
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
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:
Antiepileptic Drugs: GABAergic Pathway Potentiators
The key GABA pathway potentiators used in epilepsy management are as follows.
Benzodiazepines are a well-known class of drugs used for their...
Antiepileptic Drugs: Glutamate Antagonists
Antiepileptic Drugs: Potassium Channel Activators
Ezogabine has gained approval as an adjunctive treatment...
Epilepsy ll: Types

