Huntington's chorea

Huntington’s chorea

Chorea, the Greek word for “dance,” is used to describe the involuntary movements of the body especially of arms, legs and face. In Huntington’s chorea, these movements tend to be spastic and ballistic, and they dramatically affect normal functioning.

Huntington’s chorea is a dominantly inherited disease which is passed down through families by an autosomal dominant form of inheritance. It was first described by George Huntington in 1872. Incidence is 5-10/100,000.

The disease often presents as “nervousness“, and depression. Eventually progresses to include dementia and slowed eye movements. In juvenile HD, rigidity and parkinsonian tremor may be the primary manifestation. Generally, symptoms are first observed when the victim is in the ’20s or ’30s, and the degeneration slowly progresses, ending in the victim’s death some 10 to 20 years later.

The disease progresses with increasing chorea and loss of mental abilities. Eventually, abnormal movements and dementia become so severe that the person can no longer care for his or her self. 

Huntington’s chorea is due to slow degeneration in the basal ganglia, which eventually leads to cell death in the brain and the decrease and increase of various neurotransmitters. The symptoms of the disease are caused by a significant reduction (volume and activity) of two principal neurotransmitters (naturally occurring chemicals in the brain) – namely Acetylcholine and GABA, in turn affecting the activity of the neurotransmitter Dopamine, which becomes hyperactive. The disorder is partly characterized by an increase in the availability of dopamine, which can cause symptoms of chorea. HD is a basal ganglia disease; the portions most severely affected are caudate and putamen.

Huntingtons can be diagnosed on MRI by caudate atrophy with appropriate history, and also by genetic testing.

At present, there is no cure. The aim is to treat symptoms and support and protect the patient. Drugs can control the chorea somewhat, but benefits are often outweighed by the side effects. Caretakers must see to the person’s basic needs.

These include

  • hygiene
  • skin care
  • bowel and bladder care
  • feeding.

One must stay alert for suicide attempts. The person’s family often needs emotional support. Caring for the person at home is often beyond the family’s capacity.

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This entry was posted in Diseases & Conditions, Genetics and tagged by Manbir & Gurpreet. Bookmark the permalink.

About Manbir & Gurpreet

Gurpreet Kaur’s journey in this world .... Gurpreet Kaur was a Musician. She was a singer and a composer of music. Her interest was composing and singing Gurbani Shabads in Indian Classical style. She sang Shabads in All the Raags mentioned in Sri Guru Granth Sahib Ji. She also taught Gurmat Sangeet at Gurmat Gian Missionary College, Jawadi, Ludhiana. Elder child to Pushpinder Kaur and Dr. Brig. Harminder Singh, was born in Amritsar on 13th Jan 1962. She attended various convent schools as a child because her father would get frequent Army postings as a dental surgeon. She graduated with Music Honors from Govt. College for Women, Chandigarh. Music was her hobby and she composed and sang Raag based Gurbani Shabads. Doing Kirtan was part of growing up nurtured by her parents. She learned music from her father Dr. Brigadier Harminder Singh who was a dental surgeon in Indian Army and a very good singer himself. Gurpreet’s Bhua (father’s sister), Ajit Kaur retied as a Head of Department of Music from Govt. College for Women Ludhiana, and was a renounced Punjabi singer of her time. Gurpreet Kaur also learned nuances of Indian Classical Music from Pandita Sharma. She was a mother of three children, and a grandmother. Her daughter Keerat Kaur is a Computer Engineer. Her two sons Gurkeerat Singh and Jaskeerat Singh are doctors in USA. Her daughter Keerat Kaur too was part of her group ~ Gurmat Gian Group. Gurpreet Kaur left this world at the age of 54yrs on 12th Sept 2016 in Baltimore USA. She had recorded around 25 cds of Gurbani Keertan. 'Raag Ratan' Album (6 CDs) is a Compilation of Shabads in All the 31 Sudh Raags of Sri Guru Granth Sahib Ji. 'Gauri Sagar' Album (3 CDs) is a Compilation of All forms of Raag Gauri in Sri Guru Granth Sahib Ji. 'Nanak Ki Malhaar' ~ ((3 CDs) is an album of Raag Malhar Shabads in various forms of Malhar. 'Gur Parsaad Basant Bana' ~ (3 CDs) is an album of Shabads in Raag Basant sung in various forms of Raag Basant. Har Ki Vadeyai Sarni Aayea Sewa Priya Kee Preet Piyaree Mohan Ghar Aavho Karo Jodariya Mo Kao Taar Le Raama Taar Le Tere Kavan Kavan Gun Keh Keh Gawan Mera Baid Guru Govinda Saajanrraa Mera Saajanrraa

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