NEURODEGENERATIVE DISORDERS =BY MIA.pptx

NDD

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

9. RISKFACTORS Age Family history of dementia Hypertension Dyslipidemia Cerebrovascular dse Altered glucose metabolism Brain trauma

10. DIAGNOSIS History Neurologic exam Montreal cognitive assessment MRI FDG-PET and SPEC Biomarkers Genetic testing

11. 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

12. 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

13. 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

14. 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

15. 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

16. NON MOTOR SYMPTOMS Cognitive dysfunction and dementia Psychosis and hallucinations Mood disorders Sleep disturbances Fatigue Autonomic dysfunction Dermatologic findings etc

17. DDX Alzheimers disease Dementia with lewy bodies Huntington disease Essential traum a Multiple system atrophy Spinocerebellar ataxias

18. 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 )

19. OTHER PARKINSONIAN SYNDROMES Typically more rapid progression than Parkinson's and less responsive to levodopa They include; Multiple System Atrophy (MSA) Progressive Supranuclear Palsy (PSP) Corticobasal degeneration (CBD) Wilsons disease Huntington's disease Ataxia

20. REFERENCES Davidson’s principles and practice of medicine (23 rd ed.).Elsevier Health Sciences. Ralston, S.H., Penman, I.D., Strachan, M.W.J.,& Hobson, R. (Eds.)(2018). Medscape