Quetiapine Treatment for Self-Mutilation in Chorea-Acanthocytosis

Ak, Sertaç; Gürel, Şeref Can; Baş, Demet Funda; Yağcioğlu, Ayşe Elif Anil

doi:10.1097/jcp.0000000000000274

Cited by 4 publications

(3 citation statements)

References 13 publications

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“…Chorein is decreased in ChAc and involved in potassium‐induced DA release, affecting striatal dopamine levels . Self‐mutilation in ChAc is reported to improve with quetiapine, an atypical antipsychotic with a high affinity to 5‐hydroxytryptamine 2a (5HT2a) receptors and low affinity for D2 receptors, but not with typical antipsychotics with antagonistic activity for DA D2 receptors . This further suggests that serotonergic and dopaminergic pathways are affected in ChAc and are possibly involved in the pathophysiology of self‐mutilation.…”

Section: Discussionmentioning

confidence: 99%

Combined Dystonia With Self‐Mutilation in 6‐Pyruvoyl‐Tetrahydropterin Synthase (PTPS) Deficiency: A Case Report

Latorre

Salgado

Salari

et al. 2018

Movement Disord Clin Pract

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Section: Discussionmentioning

confidence: 99%

Combined Dystonia With Self‐Mutilation in 6‐Pyruvoyl‐Tetrahydropterin Synthase (PTPS) Deficiency: A Case Report

Latorre

Salgado

Salari

et al. 2018

Movement Disord Clin Pract

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“…Treatment of psychiatric symptoms is often the most important issue in terms of improving quality of life, can be challenging. Use of medications directed at obsessive-compulsive behaviours, such as citalopram [ 86 ], may be helpful, but quetiapine has also been reported to be very effective [ 87 ].…”

Section: Chorea-acanthocytosismentioning

confidence: 99%

Untangling the Thorns: Advances in the Neuroacanthocytosis Syndromes

Walker

2015

JMD

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There have been significant advances in neuroacanthocytosis (NA) syndromes in the past 20 years, however, confusion still exists regarding the precise nature of these disorders and the correct nomenclature. This article seeks to clarify these issues and to summarise the recent literature in the field. The four key NA syndromes are described here–chorea-acanthocytosis, McLeod syndrome, Huntington’s disease-like 2, and pantothenate kinase- associated neurodegeneration. In the first two, acanthocytosis is a frequent, although not invariable, finding; in the second two, it occurs in approximately 10% of patients. Degeneration affecting the basal ganglia is the key neuropathologic finding, thus the clinical presentations can be remarkably similar. The characteristic phenotype comprises a variety of movement disorders, including chorea, dystonia, and parkinsonism, and also psychiatric and cognitive symptoms attributable to basal ganglia dysfunction. The age of onset, inheritance patterns, and ethnic background differ in each condition, providing diagnostic clues. Other investigations, including routine blood testing and neuroimaging can be informative. Genetic diagnosis, if available, provides a definitive diagnosis, and is important for genetic counseling, and hopefully molecular therapies in the future. In this article I provide a historical perspective on each NA syndrome. The first 3 disorders, chorea-acanthocytosis, McLeod syndrome, Huntington’s disease-like 2, are discussed in detail, with a comprehensive review of the literature to date for each, while pantothenate kinase-associated neurodegeneration is presented in summary, as this disorder has recently been reviewed in this journal. Therapy for all of these diseases is, at present, purely symptomatic.

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“…32 High doses of quetiapine (600 mg/day) are also reported to be effective. 33 Frontal disinhibition can result in apparently criminal behaviors and can be very challenging to manage.…”

Section: Chacmentioning

confidence: 99%

Management of Neuroacanthocytosis Syndromes

Walker

2015

Tremor and Other Hyperkinetic Movements

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Background: The two core neuroacanthocytosis (NA) syndromes, chorea-acanthocytosis (ChAc) and McLeod syndrome, are progressive neurodegenerative disorders that primarily affect the basal ganglia. The characteristic phenotype comprises a variety of movement disorders including chorea, dystonia, and parkinsonism, as well as psychiatric and cognitive symptoms attributable to basal ganglia dysfunction. These disorders are symptomatically managed on a case-bycase basis, with very few practitioners seeing more than a single case in their careers. Methods: A literature search was performed on PubMed utilizing the terms neuroacanthocytosis, chorea-acanthocytosis, and McLeod syndrome, and articles were reviewed for mentions of therapies, successful or otherwise. Results: There have been no blinded, controlled trials and only one retrospective case series describing ChAc. The various therapies that have been used in patients with NA syndromes are summarized. Discussion: Management remains at present purely symptomatic, which is similar in principle to other more common basal ganglia neurodegenerative disorders such as Huntington's disease (HD) and Parkinson's disease (PD). However, there are some specific issues particular to NA syndromes that merit attention. An integrated multidisciplinary approach is the ideal management strategy for these complex and multifaceted neurodegenerative disorders.

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Quetiapine Treatment for Self-Mutilation in Chorea-Acanthocytosis

Cited by 4 publications

References 13 publications

Combined Dystonia With Self‐Mutilation in 6‐Pyruvoyl‐Tetrahydropterin Synthase (PTPS) Deficiency: A Case Report

Combined Dystonia With Self‐Mutilation in 6‐Pyruvoyl‐Tetrahydropterin Synthase (PTPS) Deficiency: A Case Report

Untangling the Thorns: Advances in the Neuroacanthocytosis Syndromes

Management of Neuroacanthocytosis Syndromes

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