Choreoathetosis

Choreoathetosis is a condition where the brain does not produce enough cerebrospinal fluid (CSF) to keep the body healthy. The condition occurs when there are problems with the production or absorption of CSF by one or both sides of the brain. This may occur due to various diseases such as:

A blockage in a blood vessel leading from the heart, which prevents it from pumping enough blood through your head and neck area.

A blockage in a vein supplying the brain, causing it to become starved for oxygen.

An infection of the lining of the brain (encephalitis).

The most common cause of choreoathetosis is cerebral palsy (CP), but other conditions can lead to its development. The condition affects approximately 1 in every 100 children under five years old.

It is estimated that up to 30% of children with CP will develop choreoathetosis.

Symptoms of choreoathetosis include:

Difficulty breathing (paroxysm) or difficulty swallowing (peristalsis).

Headache.

Other symptoms may include: dizziness, weakness, numbness and tingling sensations in the hands and feet, loss of balance, poor coordination and memory problems.

Treatments for choreoathetosis may include:

Carbidopa and levodopa (Sinemet)

Dopamine agonists such as Rotigotine (Neuroderm) or Comtan (entacapone)

Dopamine antagonists such as Tolcapone (Tasmar)

Propranolol (Inderal)

Corticosteroids such as prednisone or dexamethasone

Sources & references used in this article:

Familial paroxysmal dystonic choreoathetosis and its differentiation from related syndromes by JW Lance – Annals of Neurology: Official Journal of the …, 1977 – Wiley Online Library

Familial myoclonus epilepsy and choreoathetosis: hereditary dentatorubral‐pallidoluysian atrophy by H Naito, S Oyanagi – Neurology, 1982 – AAN Enterprises

Choreoathetosis, hypothyroidism, and pulmonary alterations due to human NKX2-1 haploinsufficiency by H Krude, B Schütz, H Biebermann… – The Journal of …, 2002 – Am Soc Clin Investig

Familial paroxysmal choreoathetosis: preliminary report on a hitherto undescribed clinical syndrome by LA Mount, S Reback – Archives of Neurology & Psychiatry, 1940 – jamanetwork.com

Paroxysmal kinesigenic choreoathetosis: a report of 26 patients by MK Houser, VL Soland, KP Bhatia, NP Quinn… – Journal of …, 1999 – Springer