Introduction Huntington’s disease is a chronic progressive, hereditary disease of central nervous system in which degeneration cause progressive chorea (involuntary and irregular movement ) and dementia.
Huntington’s disease is an inherited Disease that causes the progressive breakdown of nervous cell in the Brain.
Huntington’s disease is also called Huntington’s chorea, chronic progressive chorea and adult chorea. It was first describe in 1872 by American George Huntington.
DEFINITION Huntington’s disease is an chronic progressive hereditary Disease of central nervous system.
Or Huntington’s Disease marked by degeneration of brain cells and causing chorea and progressive dementia.
ETIOLOGY AND RISK FACTOR Infection Family history of the disease Genetic disorder Age: people between 25 to 55 Gender: Affecting men and women equally
ClINICAL MANIFESTATIONS Mild tremor Clumsiness Lack of concentration Irritability Short term memory Mood changes
Later symptoms fall into Three categories A) movement disorder: movement disorder associated with the huntington’s disease Involuntary Jerking or writhing movement Involuntary, sustained contracture of muscle (dystonia )
Slow fine movement Abnormal eye movements Difficulty swallowing ( Dysphagia)
B) CONGNITIVE DISORDER Difficulty planning organizing Difficulty in learning Lack of flexibility Lack of start a task or conversation
PSYCHIATRIK DISORDER The most common Psychiatric disorder associated with Huntington’s disease is depression Feeling of sadness or unhappiness Insomnia Social Withdrawal Change in appetite Loss of memory Personality change Confusion Fatigue
EARLY FEATURES Stumbling Depression Irritability Clumsiness
LATE FEATURE A) CONGNITIVE CHANGES Disorientation Difficulty in concentration
B) EMOTION CHANGE Frustration Anger Apathy Abnormal eye movements
C) PSYCHIATRIK ABNORMALITIES Insomnia Anorexia Confusion Social withdrawal
3 Stage of Huntington’s disease Early Stage Middle stage Late stage
1 Early Stage Depression Mood change Involuntary movement Irritability Lack of coordination
2 Middle stage Shakes More difficulty performing Normal Activities Slurred speech
3 Late stage Depending others Struggles to sleep, chew Eat and walk
DIAGNOSTIC EVALUATION History collection Physical examination CT- scan EEG(Electroencephalogram) Neurological Examination PET(Positron Emission Tomography) MIR( magnetic reasons imaging)
3. Stage of Huntington's disease Early Stage Middle stage Late Stage
1 EARLY STAGE Depression Mood change Involuntary movement Irritability Lack of coordination
2. Middle stage Shakes More difficulty performing Normal Activities Slurred speech
3) Late stage Depending on others Struggles to sleep, chew Eat and walk
MANAGEMENT 1) Medical management Administer tetrabenazine to treat involuntary movement jerky movement Mood stabilizer drug e.g. Volproic acid Antidepressant drugs E.g.Fluoxetine
Antiparkinaonian medication such as levodopa
Nursing management Asses the level of anxiety Provide a calm quiet Environment for the patient Provide psychological support Administer drugs as prescription
REHABILITATION 1) PSYCHOTHERAPY A psychotherapist can provide talk therapy To help person manage behavior problems, Develop coping strategies
A speech therapist can help improve ability to speak clearly 2) Speech therapist
3 physics therapy A physical therapy can teach appropriate and safe exercise
4) Occupational therapy An occupational therapy can assist the person with Huntington’s disease, family members and caregivers
COMPLICATIONS High blood pressure Heart failure Kidney Failure