NEURODEGENERATIVE DISORDERS =BY MIA.pptx
- 1. NEURODEGENERATIVE DISORDERS MOHAMED ISMAIL ABDIKADIR Dr. ASAAD (Supervisor)
- 2. DEFINITION • The neurodegenerative disease is an umbrella term for a range of pathological conditions that leads to specific neuronal death causing relentlessly progressive symptoms with incidence rising with age • Alzheimers disease and parkinsons disease are the most common
- 3. NEURODEGENERATIVE DISORDERS • Alzheimers disease • Parkinson disease • Parkinsonian syndromes; multiple system atrophy, progressive supranuclear palsy, cortical basal degeneration, wilsons disease, huntingtons disease • Ataxias • Tremor disorders; Dystonia, Hemifacial spasm, Motor neuron disease, Spinal muscular atrophy
- 4. DEMENTIA • Broad category of brain disease that cause a long term and often gradual increase in the ability to think and remember great enough to affect functionality. • Over 55 million people have dementia worldwide. Over 60% from low and middle income countries. 10 million new cases every year. Incidence doubles every 10 years after age 60
- 5. Dementia • A dementia diagnosis requires a change from a persons usual mental functioning and a greater decline than one would expect due to aging. • The most common type of dementia is Alzheimer's disease.
- 6. ALZHEIMER’S DISEASE • It is a neurological brain disorder named after a German physician Alois Alzheimer who fisrt described it in 1906.
- 7. Alzheimer's disease •Alzheimer's disease is irreversible progressive brain disease that slowly destroys memory and thinking. •Alzheimers advances in stages progressing from mild forgetfulness and cognitive impairment to widespread loss of mental abilities.
- 8. PATHOGENESIS • While pathogenesis of AD remains unclear, all forms of AD appear to share overproduction or decreased clearance of a family of proteins known as amyloid beta peptides by neuronal cells • Presence of neurotransmitter abnormalities has been described e.g. cholinergic transmission impairment
- 10. RISKFACTORS • Age • Family history of dementia • Hypertension • Dyslipidemia • Cerebrovascular dse • Altered glucose metabolism • Brain trauma
- 12. DIAGNOSIS • History • Neurologic exam • Montreal cognitive assessment • MRI • FDG-PET and SPEC • Biomarkers • Genetic testing
- 13. DDX • Vascular dementia- caused by either ischemic or hemorrhagic strokes or small vessel cerebrovascular disease • Dementia with lewy bodies- most common type of degenerative dementia after AD • Frontotemporal dementia- is a neuropathologically and clinically heterogeneous disorder characterized by focal degeneration of the frontal or temporal lobes
- 14. MANAGEMENT • Cholinesterase inhibitors- donepezil, rivastigmine, galantamine • Donepezil- 5mg orally od as starting dose, 10mg daily as maintenance dose • Rivastigmine- 1.5mg orally od , 24mg od • Galantamine- 4mg orally bd, 12mg bd • Memantine- moderate to severe disease • 5mg od( max 20mg) • Non pharm mgt- nutrition, cognitive rehabilitation, exercise programs and occupational therapy
- 17. PATHOPHYSIOLOGY • Arises due to progressive degeneration of dopamine producing neurons in the basal ganglia including the substantia nigra in the mid brain • Dopamine depletion occounts for the emergence of the classic motor phenotype as well as a wide range of nonmotor and neuropsychiatric manifestations that affect function and quality of life
- 18. CARDINAL FEATURES • These include; tremor, bradykinesia, rigidity and postural instability • Tremor- typically described as pill- rolling, is a rest tremor meaning it is most noticeable when the tremulous body part is supported by gravity and not engaged in purposeful activities • Bradykinesia- is generalized slowness of mvt and is present at onset of PD • Rigidity- is an increased resistance to passive movement about a joint • Postural instability- an impairement of centrally mediated postural reflexes that cause a feeling of imbalance and a tendency to fall with a significant risk of injury
- 19. MOTOR FEATURES OF PD • Craniofacial- hypomimia, decreased eye blinking, speech disturbance, dysphagia, sialorrhea • Visual- blurred vision, impaired contrast sensitivity, hypometric saccades, lid apraxia, impaired upward gaze and convergence, impaired vestibuloocular reflex • Musculoskeletal- micrographia, dystonia, myoclonus, stooped posture, pisa syndrome, kyphosis, scoliosis, camptocormia • Gait- shuffling, short stepped gait, freezing, festination
- 20. NON MOTOR SYMPTOMS •Cognitive dysfunction and dementia •Psychosis and hallucinations •Mood disorders •Sleep disturbances •Fatigue •Autonomic dysfunction •Dermatologic findings etc
- 21. DDX •Alzheimers disease •Dementia with lewy bodies •Huntington disease •Essential trauma •Multiple system atrophy •Spinocerebellar ataxias
- 22. MANAGEMENT • Non pharm mgt- education, social prescribing, exercise, speech therapy, nutrition • Dopaminergic therapy is the mainstay of pharm Rx for PD • Levodopa- 25mg/100mg • Amantadine- 100mg bd/tds tabs • MAO B inhibitors (selegiline, rasagiline, safinamide)
- 24. OTHER PARKINSONIAN SYNDROMES • Typically more rapid progression than Parkinson's and less responsive to levodopa • They include; 1. Multiple System Atrophy (MSA) 2. Progressive Supranuclear Palsy (PSP) 3. Corticobasal degeneration (CBD) 4. Wilsons disease 5. Huntington's disease 6. Ataxia
- 29. REFERENCES • Davidson’s principles and practice of medicine (23rd ed.).Elsevier Health Sciences. Ralston, S.H., Penman, I.D., Strachan, M.W.J.,& Hobson, R. (Eds.)(2018). • Medscape