# Effects of Levetiracetam on Episodic Ataxia Type 2 and Spinocerebellar Ataxia Type 6 with Episodic Ataxic Symptoms: A Case Series

**Authors:** Haruo Shimazaki

PMC · DOI: 10.3390/genes16030335 · Genes · 2025-03-13

## TL;DR

This case series explores the effectiveness of levetiracetam in reducing ataxic episodes in patients with EA2 and SCA6, showing promising results over a seven-year period.

## Contribution

The study demonstrates that levetiracetam may be a more effective treatment for episodic ataxia than acetazolamide in some patients.

## Key findings

- Levetiracetam significantly reduced the number of ataxic attacks in two EA2 patients.
- The effect of levetiracetam remained stable for seven years in the studied patients.
- Levetiracetam and acetazolamide together reduced chronic cerebellar ataxia in an SCA6 patient.

## Abstract

Background: Episodic ataxia type 2 (EA2) is a rare disorder characterized by paroxysmal gait instability, dysarthria, and dizziness. It is caused by CACNA1A mutations. Spinocerebellar ataxia type 6 (SCA6) rarely causes episodic ataxia-like symptoms. Acetazolamide has limited effectiveness for treating episodic ataxia. Methods: We investigated the effect of drug therapy in two patients with EA2 and one patient with SCA6 who presented with episodic ataxia. All three cases were CACNA1A-associated diseases. Results: In these three cases, acetazolamide administration was partially and transiently effective for episodic ataxia attacks. After levetiracetam addition, the number of ataxic attacks was significantly reduced, although the durations of attacks were not changed. The effect of levetiracetam was stable and continued for seven years. Levetiracetam and acetazolamide reduced chronic cerebellar ataxia in an SCA6 patient. Conclusions: In this small number of cases, levetiracetam was considered effective in two patients with EA2 and mildly effective in one patient with SCA6.

## Linked entities

- **Genes:** CACNA1A (calcium voltage-gated channel subunit alpha1 A) [NCBI Gene 773]
- **Chemicals:** Levetiracetam (PubChem CID 5284583), Acetazolamide (PubChem CID 1986)
- **Diseases:** Episodic ataxia type 2 (MONDO:0007163), Spinocerebellar ataxia type 6 (MONDO:0008457), Episodic ataxia (MONDO:0016227)

## Full-text entities

- **Genes:** CACNA1A (calcium voltage-gated channel subunit alpha1 A) [NCBI Gene 773] {aka APCA, BI, CACNL1A4, CAV2.1, DEE42, EA2}
- **Diseases:** dysarthria (MESH:D004401), SCA6 (MESH:D020754), ataxic attacks (MESH:D001039), dizziness (MESH:D004244), Episodic Ataxic Symptoms (MESH:C580065), gait instability (MESH:D043171), EA2 (MESH:C535506), cerebellar ataxia (MESH:D002524)
- **Chemicals:** Levetiracetam (MESH:D000077287), Acetazolamide (MESH:D000086)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11942281/full.md

## References

22 references — full list in the complete paper: https://tomesphere.com/paper/PMC11942281/full.md

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Source: https://tomesphere.com/paper/PMC11942281