Amyotrophic Lateral Sclerosis ALS

Amyotrophic Lateral Sclerosis ALS also known as - answer-Charcot's disease Lou Gehrig's disease Amyotrophic Lateral Sclerosis ALS is an - answer-incurable fatal neuromuscular disease characterized by progressive muscle weakness, resulting in paralysis. It is characterized by rapid deterioration and the selective death of motor neurons in the cerebral cortex, brainstem, and spinal cord Motor neurons, which control the movement of voluntary muscles, deteriorate and eventually die. When the motor neurons die, the brain can no longer - answer-initiate and control muscle movement. Because muscles no longer receive the messages they need in order to function, they gradually weaken and deteriorate. ALS - connection between muscle and - answer-CNS is blocked muscle begins to atrophy ALS patients retain full - answer-intellectual capacity and intelligence However, approximately 15% will develop dementia Early stages of the disease involve surviving motor neurons "sprouting" - answer-new branches (similar to cardiac collateral flow!!) to reinnervate affected muscle fibers in an attempt to preserve muscle strength. However, as the disease progresses the reinnervation fails. ALS causes (unknown) or - answer-excess of a neurotransmitter called glutamate builds up in the synapse causing eventual nerve cell death and subsequent muscle atrophy of the muscle attached to that nerve -or autoimmune problem ALS hereditary - answer-10% FALS (familial ALS) In familial ALS, the disease is autosomal ______: if a parent has ALS the offspring have______ a chance of inheriting the gene, not all people with ALS gene will develop the disease. - answer-dominant 50% Environmental factors contributing to ALS - answer-smoking exposure to agricultural chemicals and solvents Who gets ALS - answer-all races Men 40-70 yrs of age (avg 55-65 yrs) ethical issues involving passing disease - answer-onto children. May not even know you have it til after family is started % of ALS clients that die within ____ months of diagnosis - answer-50 18 ___ % survive 5 years - answer-20 ___ % live longer than 10 years - answer-10 _____ dependent ALS clients may live for many years - answer-ventilator Early symptoms of ALS - answer-Fasciculations (twitching) of the face Dysarthria (slurred speech) (80% of pts) Dysphagia (difficulty swallowing) Limb weakness ( 65%) • Symptoms of ____ may not be evident until about 80% of the motor neurons are lost • Defective articulation • Slow laborious speech • Imprecise consonant production • Marked hypernasality - answer-dysarthria ________ in about 65% of patients: foot drop, difficulty walking , loss of hand dexterity or shoulder weakness - answer-• Limb weakness symptoms of ALS - answer-tripping, stumbling, falling lose muscle control/strength n hands/arms difficulty breathing chronic fatigue muscle twitching/cramping common progression - - answer-difficulty walking,cane-walker-wchr lose ability to write, type, feed self difficulty speaking/swallowing -----may need feeding tube---- decrease in lung capacity (50%=vent) progression can be 6 yrs or many yrs Diagnosis of ALS - answer--History -EMG -MRI and spinal fluid analysis -muscle biopsy -PFTs Diagnosis typically takes________ process of eliminating other disorders - answer-weeks or months, done to diagnose abnormal electrical activity of involved skeletal muscles - answer-Electromyography (EMG) MRI of brain and cervical region done to - answer-exclude other conditions Other "mimicking" disorders include: - answer-hyperthyroidism, hypoglycemia, compression of the spinal cord, toxic agents, infections. Spinal fluid may be done to rule out - answer-other causes Muscle biopsy demonstrate small, - answer-angulated atrophic fibers PFTs(Pulmonary function tests): reveal - answer-motor strength deficits such as decreased vital capacity Riluzole (Rilutek): antiglutamate: 1st drug developed to treat ALS - answer-slows progression of disease by inhibiting release of GLUTAMIC acid in the CNS and protects the neurons Rilutek should be taken on an - answer-empty stomach same time each day Rilutek is expensive - answer-$600-700 for a 30 day supply, but most insurance covers it Rilutek common S/E - answer-fatigue somnolence N/D dizzy May cause increased liver enzymes Rilutek Dosage: - answer-50 mg po every 12 hours treatment for cognitive decline and depression - answer-amitriptyline or SSRI depressants treatment for emotional liability - answer-dextromethorphan hydrobromide quinidine sulphate treatment for Sialorrhoea - answer-transdermal scopolamine injections of botulinum toxin Cognitive symptoms in ALS are usually due to - answer-frontotemporal impairment and include personality change, irritability, obsessions, poor insight and impairment in language Depression & anxiety are more common in patients who are at___________________ (about half of caregivers deal with anxiety). However recent study showed severe depression in 20% of patients with ALS as compared to 40-50% of patients with other neurodegenerative motor conditions such as MS, Parkinson's and Huntington's however 50% of patients with ALS struggled with some depression - answer-high age at onset of the disease