Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content ++ Huntington’s chorea ++ 333.4 Huntington's chorea ++ G10 Huntington’s disease ++ 5E: Impaired Motor Function and Sensory Integrity Associated with Progressive Disorders of the Central Nervous System1 +++ Description ++ Degeneration of nerve cells in the brainHereditary disorder Children of people with Huntington’s have 50% of inheriting the gene +++ Essentials of Diagnosis ++ Genetic dysfunction on 4th chromosomeAutosomal dominant inheritanceCharacterized by choreoathetosis and dementia +++ General Considerations ++ Family history and genetic testing to confirm diagnosis +++ Demographics ++ Adult onset generally in 4th or 5th decade of lifeEarly onset, not common but may occur in adolescenceMost common in Caucasians of European ancestry +++ Signs and Symptoms ++ Slight but evident alteration in character initially, followed by issues of self-control, eventually leading to failure of all cognitive functionDifficulty with attention and concentration, poor mental flexibilityMovement abnormalities begin in hands and face, initial slowing of movement and loss of coordination, progressing to chorea, then athetosisDegree and continuous nature of movement causes most patients to experience extreme weight loss and malnutritionBehavioral changesIrritabilityRigidityTremorsParanoiaDementiaUnsteady gaitAbnormal reflexesMemory loss +++ Functional Implications ++ Loss of ability to interact in society and eventually with family membersLoss of ability to care for self mentally, emotionally, physicallyLoss of functional mobility and associated cognitive processes Difficulty swallowingLimited ambulationPoor communication, speech impairments +++ Possible Contributing Causes ++ Genetic disorder; no other known contributing causes +++ Differential Diagnosis ++ Parkinson’s disease Alzheimer’s disease Cerebral infection Hyperglycemia Stroke Thyrotoxicosis Tardive dyskinesia Lupus Sydenham chorea +++ Laboratory Tests ++ DNA/genetic testing +++ Imaging ++ Head CT may show loss of brain tissue ++ To neurologist for imaging, disease managementMedicine: fluoxetine, carbamazepine, anti-psychotic agentsTo support groups for psychological support Huntington’s Disease Society of AmericaTo hospice for end-of-life careTo geneticist for genetic counseling ++ ArousalAttentionBehaviorCognitionGaitWheelchair mobilityBalanceStatic sitDynamic sitStatic standDynamic standMoving base of support (BOS)Muscle strengthMuscle recruitmentCoordinationPostural control (due to choreoathetosis)Posture (later stages)Range of motion (ROM)ReflexesDeep tendon reflexes (DTR)BabinskiClonusMuscle toneMotor planningBed mobilityTransfersEnduranceAerobic capacitySelf-careHome managementFine motorRespiratory compromise ++ Cognition: Mini-mental State Examination (MMSE), executive function tests, memory, comprehension, speed of processing, attention and concentrationStrength and muscle enduranceCoordination: gross and fine motorMuscle tone: look for fluctuations between normal and high tone; tone changes with patient’s level of anxiety, ... Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Download the Access App: iOS | Android Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.