Huntington's disease (HD) is an incurable, neurodegenerative disease, which manifests via a triad of progressive symptoms: motor impairment, psychiatric disorders, and cognitive decline. Conventionally, the HD diagnosis is based on the presence of involuntary choreiform movements and a positive genetic test for the CAG-expanded allele gene. Although the diagnosis focuses on the motor part of the triad, there is increasing evidence that both cognitive and neuropsychiatric symptoms can, and often do, present decades before the onset of motor symptoms. In this paper, we review the evidence regarding the symptoms in the HD premotor phase and summarize the most relevant and robust studies in the last few years. Regarding neuropsychiatric symptoms, higher levels of depression, anxiety, apathy, irritability, psychosis, disinhibition, hostility, and sleeping problems were found. In terms of cognition, there was impairment in attention, working memory, episodic memory, language, recognition of facial emotions, empathy, and theory of mind. These early symptoms of HD can be very debilitating and are often disregarded by doctors. It is necessary to acknowledge them so that they can be treated or alleviated. Moreover, an earlier diagnosis can lead to the implementation of neurodegenerative prevention therapies, which may slow the progression of the disease and prolong overall functioning. This will improve the patient's independence and their quality of life.
BackgroundPeople living with a long-term neurological condition (LTNC) make up nearly one-fifth of hospital admissions in the UK. Though LTNCs are diverse, the common trigger for deterioration is often systemic infection. More data on the problem is required to inform service planning.MethodsThe case notes of 157 consecutive patients with an LTNC presenting to University Hospital South- ampton for urgent care, were reviewed, as part of an ongoing and prospective service evaluation. Data was collected on mode of presentation, acute diagnosis, specialty input, length of stay, complications, and change in social needs.ResultsDementia (43%) and stroke (27%) were the most common LTNCs. Systemic infection was the trigger for admission in 52% of patients. The mode of presentation was most commonly deterioration in func- tional abilities and cognition. Mean length of stay was 5.7 days. Care needs increased in 11% of patients.ConclusionThis data provides a snapshot of the urgent care needs of people with LTNCs, which should help inform future service planning. In particular, the impact of systemic infection is significant and strate- gies to mitigate this are discussed.ritaramos.jc4@gmail.com
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