Mechanisms Preventing Huntington’s Disease Progression Identified

What is Huntington's Disease?

Huntington's disease is a medical condition wherein certain brain cells are damaged, causing further deterioration and gradual loss of function of the central nervous system. This can affect movement, cognition (perception, awareness, thinking, judgement) and behaviour. Some early symptoms of Huntington's disease may include personality changes, mood swings and unusual behaviour.

Huntington's disease is caused by a faulty gene that runs in families. Fifty percent of the gene can be passed on to children, so that this disease is called autosomal dominant. People who have Huntington's disease have a variety of signs and symptoms such as psychiatric problems and difficulties with behaviour, feeding, communication and movement. Some patients may manifest personality changes, mood swings and unusual behaviour. At any age, Huntington’s disease may manifest itself, but it is said to be more common between the ages of 35 and 55. The disease may then progress and get worse for 10 to 20 years until death claims the life of the patient.

Huntington's disease may bring about behavioural changes, which may include a lack of emotions and not recognising the needs of others in the family, alternating periods of aggression, excitement, depression, apathy, antisocial behaviour and anger, difficulty concentrating on more than one task which causes irritability, short-term memory lapses and problems with orientation. The patient may include a lack of drive, initiative and concentration which may be mistalen for laziness. He or she may develop a lack of interest in hygiene and self-care. Depression and other psychiatric problems may also set in, such as obsessive behaviours, schizophrenia and suicide.

Huntington's disease may also bring about movement problems such as uncontrollable movements of the face, jerking, flicking or fidgety movements of the limbs and body. Patients may also have feeding problems and may lose weight despite having a good appetite. There may be loss of coordination which can lead to spillage of food, swallowing problem, choking and difficulty in speaking. The patient may also have communication and cognition (perception, awareness, thinking and judgement) problems. Sexual problems may also ensue and may stem from lack of sexual interest. Once Huntington's disease becomes serious, it can lead to total dependence and need for full nursing care. Death is usually from a secondary cause, such as heart failure, pneumonia or another infection.

Study on Huntington’s Disease Progression

A recent study by researchers from the University of British Columbia have shown that blocking a specific class of glutamate receptors, called extrasynaptic NMDA receptors, can improve motor learning and coordination, and prevent cell death in animal models of Huntington disease. The results could lead to future promising preventive treatments that may delay the onset of symptoms and neurodegeneration. The researchers used a drug that selectively blocks extra-synaptic NMDARs early, before the appearance of any symptoms, to delay the onset of Huntington-like symptoms in a mouse model of the disease. The drug they used was memantine which is currently being used to treat moderate-stage Alzheimer disease patients. The results of this study are promising because they could lead to new treatment avenues for Huntington patients, and delay the appearance of symptoms.

Extra-synaptic NMDARs have also been implicated in some neurodegenerative diseases, such as Alzheimer disease, and in damage caused by traumatic brain injury and some forms of stroke. Thus it is not only Huntington's disease that may benefit from this potential treatment but also other neurodegenerative didseases. These avenues can protect neurons before the appearance of symptoms of neurodegeneration.

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