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AbstractObesity, hypertension, obesity-related hypertension such as hypertension with diabetes are growing health problems.
Hypertension, insulin resistance, lifestyle modification, obesity, pharmacological treatments.
Robertson L, Santini H, O'Donovan KL, Squitieri F, Barker RA, Rakowicz M, Landwehrmeyer GB, Quarrell O.
Citation Robertson L, Santini H, O'Donovan KL, Squitieri F, Barker RA, Rakowicz M, Landwehrmeyer GB, Quarrell O. Department of Clinical Neurophysiology, Institute of Psychiatry and Naurology, Warsaw, Poland. Background: The clinical presentation of Juvenile Huntington’s Disease (JHD) can be very different from adult-onset HD with little evidence to guide symptomatic management. Methods: Patients were identified through the HD Association, Hospital Doctors and the European Huntington’s Disease Network REGISTRY study. We would like to acknowledge the funding for this project which we received from the Sheffield Children’s Hospital Charity (SCHC).
Huntington’s disease (HD) is a dominantly inherited, neurodegenerative disorder, due to a (CAG)n repeat expansion in the HTT gene [1] .
Juvenile HD (JHD)is defined as onset under 20 years of age [2] and represents approximately 1-10% of cases [3] [4] [5] [6] [7] . Adult HD is characterised by a triad of clinical features including: chorea, cognitive decline and psychiatric disturbance or behavioural disturbance [11] . There is currently no effective disease-modifying treatment for either adult onset HD or JHD; therefore, clinical care is symptomatic and supportive. For UK families affected by JHD, we composed a questionnaire, to be completed by the family, listing their child’s symptoms and treatments. As a second phase, we collated data on medications prescribed for JHD patients enrolled in the European Huntington’s Disease Network REGISTRY study (excluding the UK). When the data was combined the median number of medications prescribed was four, with an arithmetic mean of five (range 1-16) (Fig 1). In REGISTRY, the most commonly prescribed agents were anti-psychotics, anti-depressants, anti-parkinsonianmedications and anti-epileptics (Fig 2).
The most commonly prescribed individual medication was Valproic acid (anti-epileptic), followed jointly by: Tiapride (anti-psychotic), Dopa and Dopa derivatives (anti-parkinsonian) and Tocopherol (vitamin preparation) (Fig 4). Anti-depressants and anti-parkinsonian agents were also frequently prescribed (n=17 and n=15 respectively) and some (n=7) were taking a combination of anti-parkinsonian medications and anti-psychotics. The most commonly prescribed individual medication was Valproic acid (8), reflecting consistency in anti-epileptic prescribing. An apparent difference in prescribing habits was noted between the two populations of patients studied: within the UK, opioids were prescribed whereas in the REGISTRY group they were not, this may be due to variations in national restrictions [25] .

The diagnostic features of HD presenting in the first decade are a family history of HD, often in the father (though onset in the child may precede onset in the parent), and two or more of the following: declining school performance, seizures, oral motor dysfunction, rigidity and gait disorder [27] . We set out to identify which medications were being prescribed for symptomatic relief in JHD; this has not been done before. Identifying current practice is only a first step towards establishing an evidence base for treatment guidelines.
We would like to acknowledge the time and effort of the patients and families who participated in this study. Obesity, hypertension and diabetes are important, independent risk factors for the onset and development of cardiovascular diseases. Many patients were on multiple therapies, highlighting the need to rationalise medications.
There is an inverse correlation between the age at onset of disease and the number of (CAG)n repeats [8] [9] [10] .
However, in JHD, particularly in those with onset in the first decade, chorea is less likely to be present and instead, patients are more likely to present with rigidity, bradykinesia, dystonia and gait disorder, ie. The very different presentation means that different treatments are likely to be used in the management of JHD versus adult onset HD.
We used monitored case report forms from the European Huntington’s Disease Research Network (EHDN) REGISTRY project.
In the UK group, the family member completing the questionnaire was asked to give the indication for each medication, and for anti-psychotics this included: agitation, behavioural problems and psychiatric disturbances, none of which are listed as diagnostic features of JHD. This odd regime often reflects the combination of dystonia and parkinsonism seen in JHD, although whether it is a useful combination is unclear especially given the recognised parkinsonian side-effects of anti-psychotics [22] . This is in-line with prescribing practice in the management of childhood epilepsy of all causes [23] ; Valproic acid is widely used as first-line therapy for generalised seizures with a strong evidence base to support its efficacy [24] . The highest number of medications for any one patient was sixteen, raising issues of polypharmacy.
For certain individuals with JHD, pain can be a feature and from our own experience this can be difficult to manage and the input of a specialist pain team or palliative care specialists can be invaluable [26] . We analysed the data based on age of diagnosis (Fig 4); we hypothesized that those who were older at diagnosis may have a profile more akin to adult JHD with a lower incidence of rigidity, dystonia and epilepsy. We identified that anti-psychotics were the most commonly prescribed agent and that anti-depressants and anti-parkinsonian medications were also frequently prescribed in the JHD group. The diversity of their problems means they are often prescribed medications which exceed the area of expertise of a single hospital specialist. JHD is a rare condition which means that international collaboration is essential to gather information on the effectiveness of symptomatic management. Hypertension in obesity is characterized by stimulation of the renin-angiotensin-aldosterone system (RAAS), elevated sympathetic activity, insulin resistance and selective leptin resistance.

Given the rarity of the condition, each treating clinician is unlikely to have seen more than a few cases and there are no treatment guidelines. Patients were identified through the Patient’s Support Group and Hospital Clinicians (Neurologists and Clinical Geneticists). This is important as it is recognised that children presenting with behavioural or psychiatric problems often experience a significant delay in receiving a diagnosis of JHD [14] , and our data would infer that such problems are common in this age group affected by HD. This side-effect is less frequent with the atypical anti-psychotics than in first generation anti-psychotics, but does still occur and it may be difficult to distinguish from disease progression.
At present, as there is no disease-modifiying therapy available, the optimum management of JHD encompasses the optimum management of the associated symptoms. This is a particularly important issue in a group of patients with swallowing difficulties.
We therefore divided patients into three groups, those diagnosed at 10 years of age or under (n=6), those diagnosed between 11 to 15 years of age (n=12) and those diagnosed between 16 to 20 years (n=26). Their medications require regular review by a team of experts, in the face of evolving symptoms. It is known that these characteristics, even in isolation, constitute risk factors for cardiovascular disease onset and progression. In addition, JHD patients are more likely to develop epilepsy than adult-onset cases [18] . Therefore, gathering together the limited information available is of significant benefit to both families and physicians and helps form the basis of future trials. Three patients were on three or more anti-pyschotic agents or anti-parkinsonism agents simultaneously: which highlights the fact that many cases of JHD appear to be refractory to pharmacotherapies. Polypharmacy is an issue in this group, highlighting a potential need for reviewing and stopping medicines, especially when they appear to be ineffective. Ultimately we would hope that it will be possible to use such a database to identify affected patients and conduct a randomised trial on the effectiveness of different symptomatic treatments in this form of HD. Therefore, pharmacological treatments should be selected based on favourable effects on insulin resistance, stimulated RAAS and sympathetic nervous systems. The spectrum of features at presentation can also include ataxia, dysarthria, dysphagia, deterioration in school performance or severe behavioural problems [11] [14] [19] [20] . There was no apparent trend between younger age at diagnosis and the frequency of prescription of anti-epileptics or anti-parkinsonian agents.

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