neurologic disorders
Huntington Disease
Symptoms and Signs of Huntington Disease
Symptoms and signs of Huntington disease develop insidiously, starting at about age 35 to 40, depending on phenotype severity. Dementia or psychiatric disturbances (eg, depression, apathy, irritability, anhedonia, antisocial behavior, full-blown bipolar or Schizophreniform Disorder ) develop before or simultaneously with the movement disorder. These symptoms predispose patients to suicidal ideation and suicide, which are much more common among patients with Huntington disease than among the general population. Abnormal movements appear; they include Chorea, Athetosis, and Hemiballismus , myoclonic jerks, and pseudo-tics (one manifestation of tourettism). Tourettism refers to Tourette-like symptoms that result from another neurologic disorder or use of a drug; tourettism also includes the repetitive gestural movements and/or phonatory sounds that patients with chorea make. Unlike true tics, the pseudo-tics of Huntington disease cannot be suppressed. Typical features include a bizarre, puppet-like gait, facial grimacing, inability to intentionally move the eyes quickly without blinking or head thrusting (oculomotor apraxia), and inability to sustain a motor act (motor impersistence), such as tongue protrusion or grasping. Huntington disease progresses, making walking impossible and swallowing difficult; it results in severe dementia. Most patients eventually require institutionalization. Death usually occurs 13 to 15 years after symptoms begin. Patients with Huntington disease may become depressed or anxious and/or develop Obsessive-Compulsive Disorder (OCD) . 