In March 2018 the EMA announced stronger measures aimed at avoiding the exposure of babies to valproate medicines in the womb. Under the new restrictions, valproate medicines are contraindicated during pregnancy due to the high risk of congenital malformations and developmental problems in the child. Valproate medicines must not be used in female patients of childbearing potential unless there is a pregnancy prevention programme in place and certain conditions are met. These include:
An assessment of the patient’s potential for becoming pregnant
Pregnancy tests before starting and during treatment as needed
Counselling about the risks of valproate treatment and the need for effective contraception throughout treatment
A review of ongoing treatment by a specialist at least annually
A risk acknowledgement form that patients and prescribers will go through at each such annual review to confirm that appropriate advice has been given and understood.
The EMA said the new measures were put in place because of evidence suggesting that information on the risks of valproate use in pregnancy was still not getting through to women despite earlier steps aimed at ensuring this. In the context of Huntington’s disease, anticonvulsants (including valproate medicines) are used for the treatment of behavioural and mood problems (e.g., irritability, mood swings, aggression, frequent temper outbursts).See Management: approach See Management: treatment algorithm
Huntington's disease is an autosomal dominant neurodegenerative disorder.
Often presents in mid-life but may appear at any age.
Clinical manifestations include chorea, cognitive decline, loss of coordination, and personality change.
Depression and suicide may be comorbid events.
In the absence of available effective treatment, many at-risk individuals forgo predictive genetic testing.
Huntington's disease is a slowly progressive, neurodegenerative disorder characterised by chorea, incoordination, cognitive decline, personality changes, and psychiatric symptoms, culminating in immobility, mutism, and inanition.  It is an autosomal dominant, trinucleotide repeat disorder that affects men and women equally. It characteristically appears in mid-adult life but can occur at any age. Treatment for depression, and to some degree behavioural problems and chorea, is possible. There are no disease-modifying therapies.
Professor of Clinical Neurology
Honorary Consultant Neurologist
UCL Institute of Neurology
SJT receives grant funding for her research from CHDI Foundation, the BBSRC, Dementia and Neurodegenerative Disease Network UK, European Huntington’s Disease Network, Huntington’s Disease Association of the UK, the Medical Research Council UK, Takeda Pharmaceuticals, the UCL/UCLH Biomedical Research Centre, and the Wellcome Trust. In the past year, SJT has been on advisory boards or had consultancies with F. Hoffmann-La Roche Ltd, Ixico Technologies, Shire Human Genetic Therapies, Takeda Pharmaceuticals International, and TEVA Pharmaceuticals. All honoraria for these consultancies and advisory boards were paid to UCL. Through the offices of UCL Consultants Ltd, a wholly owned subsidiary of University College London, SJT has undertaken consultancy services for F. Hoffmann-La Roche Ltd and GSK. ST is also an author of references cited in this topic.
Wellcome Trust Clinical Research Fellow
UCL Institute of Neurology
PM declares that he has no competing interests.
Senior Lecturer in Neuropsychiatric Genetics
University of Manchester
Manchester Academic Health Sciences Centre and Central Manchester University Hospitals NHS Foundation Trust
St Mary's Hospital
DC has received fees for advisory board membership from Hoffmann-La Roche Ltd.
Dr Sarah Tabrizi, Dr Peter McColgan, and Dr David Craufurd would like to gratefully acknowledge Dr Marianne Novak and Dr Francis Walker, previous contributors to this monograph. FW declares that he has no competing interests. MN is author of a reference cited in this monograph.
Massachusetts Eye and Ear Infirmary
Harvard Medical School
AP declares that he has no competing interests.
Neurological Clinical Research Unit
Institute of Molecular Medicine
TM declares that he has no competing interests.
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