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motorneuron-diseases-1313085650-phpapp02-110811130324-phpapp02.pptx
- 3. â« Each single motor neuron and the muscle fibers it innervates constitute a MOTOR UNIT. â« No .of muscle fibers in a motor unitvaries. â« HAND,MOTION OF EYE â 3-6 muscle fibers. â« LEG MUSCLES â 600 muscle fibers. â« Groupof muscle fibers (forming a motor unit ) can be intermixed in a muscle. â« ALL THE MUSCLE FIBERS IN A MOTOR UNIT ARE OF SAME TYPE.
- 4. â«Based on typeof muscle fibers innervated ,durationof twitchcontraction Motorunitsare divided into â«S- slow -- small units â«FR â fast resistant to fatigue â«FF fast fatiguable --- large units â«RECRUITMENTof motorunits followssize principle: â«S muscle units â relatively slow contraction,controlled contraction ---FR more powerful response âFF muscle units mostdemanding tasks..
- 8. S MUSCLE UNITS RELATIVELY SLOW CONTRACTION CONTROLLED CONTRACTION FR MUSCLE UNITS MORE POWERFUL RESPONSE FF MUSCLE UNITS MOST DEMANDING TASKS STANDING WALKING RUNNING OR JUMPING
- 9. â« Differ byactivity â« Increased activity â muscle hypertrophy âtype IIa ,IIb fibers â« Inactivity âatrophy â type I fibers aresusceptible If nerve to slow muscle is cut,the nerve toa fast muscle is spliced to thecutend The fast nerve growsand innervates the previously slow muscle Muscle becomes fast,changes in portein isoforms,changes in myosin ATPase activity
- 11. â«Original delineation of ALS â charcot â CHARCOTâS disease. â«Pathological apsectsof disease â Joffroy,gambault â«Labioglossolaryngealparalysis â progressive bulbarpalsy â«"Lou Gehrig'sdisease".
- 13. â« Amyotrophic comes from thegreek language: A- means "no", myo refers to "muscle", and trophic means "nourishment"; amyotrophic therefore means "no muscle nourishment," which describes the characteristic atrophicationof thesufferer'sdisused muscle tissue. â« Lateral identifies theareas in a person's spinal cord where portions of the nervecells thatareaffected are located. â« As thisarea degenerates it leads to scarring or hardening (âsclerosis") in the region.
- 14. â«Heterogenousgroupof neurodegenerativedisorders. â«Unknown etiology â«Selective lossof motor neuronscontrolling voluntary movements.
- 15. â«Weakness â«Nosensory loss â«Nosphincterdisturbances â«Progressivecourse â«No identifiable underlying cause
- 16. DISORDER PRIMARY SECONDARY UMN PRI. LATERAL SCLEROSIS HIV,SYPHILIS,HTLV,LYME, TB PROG.PSEUDO BULBAR PALSY LEAD,Hg,Al,LATHYRISM,V ASCULAR,TRAUMA HER.SPASTIC PARAP. LMN PROG.MUSCULAR ATR. AC ANTER.POLIO S.M.A POST POLIO SYN. P.J.B.P PARANEOPLASTIC HUNTINGTONS FREDERICHS COMBINED A.M.L HYPERTHYROIDISM HYPERPARATHYROIDIS POST RADIATION
- 18. â«M.C formof progressive motor neuron disease. â«Primeex. of neurodegenerativedisease. â«Mostdevastating of the neurodegenerativedisorders.
- 19. â«Prevalence 4-6/100,000 â«Incidence-1-3/100,000 â«Equal presentation in all racial groups â«20-90 yearsof age â«Peak between 50-70 years â«M:Fâ1.5:1 â«Relentlesslyprogressive â«Death from resp. paralysis â«Survival 3-5 years â«Risk factors-pesticides,smoking.
- 20. ⣠5-10% ⣠20%--SOD1 gene mutation ⣠21qchromosome ⣠Copper , zinc dependentsuperoxidedismutasegene ⣠Autosomal dominant ⣠Indistinguishable from sporadic ⣠Dynactin,senataxin ⣠Alsin âAR ⣠DYNACTIN
- 21. â«HALLMARK-deathof LMN ,.UMN â«LMN-ant.horncellsof spinal cord,bulbar muscles â«UMNâlayer 5 of motorcortex,descending via pyramidal tracts. â«Other motor neuron disease involvesonly subsetof motor neurons.
- 25. â«Onset-----UMN or LMN-----Involves both---absence of them ?diagnosis. â«Accof lipid pigmentedâLIPOFUSCHIN. â«Normallyseen in aged cells. â«Focal enlargementsare frequent âSPHEROIDâaccof neurofilamentproteins. â«Proliferation of astrocytes,microglia. â«Combined grey and white matterdisease. â«Motorcellsand motorfibre tracts. â«ATROPHY,DEGENERATION.LOSS OF MOTOR NEURONS OF CN..
- 26. Death of the peripheral motor neurons in brainstem,spinal cord. Denervation Consequentatrophyof the muscle fibres Histo chemical,electrophysiological Earlystages Reinnervation Less than poliomyelitis.peripheral neuropathy
- 28. â«Progressivedegeneration âmuscleatrophy is readily recognised in muscle biopsiesand on clinical exam,--- AMYOTROPHY. â«Thinning of corticospinal tracts â«Lossof fibers in the Lateral columnsâfibrillary gliosis-LATERAL SCLEROSIS. â«SELECTIVITY OF NEURONAL CELL DEATH.
- 29. â«UBIQUITIN-marker fordegeneration is seen. â«Nucleusof Onuf âinnervates bowel ,bladder is not involved. â«Max involvement in cervical spinal cord â«Lossof large pyramidal cells BETZ cells in motor ,premotorcerebral cortex. â«Gliosisof lateral cords â«NONE ARE PATHOGNOMONIC.
- 30. â«Cause notwell defined.. â«Excitatory neurotransmitters. â«Glutamateparticipate in death of motor neurons in ALS. â«EAAT2. â«SOD1âcellulardefenseagainstexcitotoxicity â«When mutatedâcatalytic. â«Non neuronal cells âinfluences thediseasecourse.
- 31. â«Variable on motor neurons involved. â«Asymmetricweakness ,usuallydistally in one of the limbs. â«Insidiousonset â«Developmentof cramps with volitional movements in early hoursof morning. â«Weakness â«Wasting â« Atrophy
- 32. ⣠Spontaneus twitching of motorunitsor fasciculation ⣠EXTENSORS >FLEXORS in upper limbs ⣠Difficulty in chewing ,swallowing,movementsof face and tongue. ⣠Early involvementof resp.musclesâdeath ⣠Hyperactivityof musclestretch reflexes ⣠Spasticresponse topassive movements ⣠Musclestiffnessoutof proportion ⣠Exaggeration of motorexpression of emotionâ weeping,laughingâpseudobulbareffect
- 33. â«Asymmetricâsymmetric â«Whether UMN or LMN atonsetâboth later â«Sensory,bowel,bladderânot involved. â«Cognitive function,ocularmotility âpreserved â«Dementia is nota componentof sporadic ALS â«FamilialâALS+ FRONTO TEMPORAL DEMENTIA.
- 34. ⣠Fatigue ⣠Weight loss ⣠Widespread musclepains ⣠Experience fear,anxiety,depression ⣠Limbsymptoms>bulbar ⣠Proximal weaknessâlimbs,trunks,neck ⣠Hemiplegia ⣠Spasticparaparesis ⣠Footdrop ⣠Pes cavus
- 35. â«Upper limb> lowerlimb â«Distal> proximal â«Extensor>flexor â«Clawing of hands â«Asymmetricatonset
- 36. â«Spontaneusclonus â«DTRâinc â«Babinskiâpostive â«Abd. Reflexes âpreserved â«Bed soresare uncommonâcollagen â«Dysphagia worse for liquids > solids â«Choking â«Flaccid dysarthria â«Inability topurse lips
- 37. â«THE ENTIRE SENSORYAPPARATUS â«REG. MECH. FOR CONTROL AND COORDINATION OF MOVEMENTS â«COGNITION â«SEXUAL FUNCTION â«OCULAR MUSCLES
- 38. ⣠WORLD FEDERATION OF NEUROLOGY ⣠Simultaneous inv. Of UMN,LMN ⣠PROGRESSIVE WEAKNESS ⣠EXCLUSION OF ALTERNATE DIAGNOSIS ⣠DEFINITE ALS-3 or 4 --- bulbar,cervical,thoracic,lumbosacral ⣠2sitesâprobable ⣠1 site-possible ⣠exception âmutation in geneencoding SOD1-21q
- 39. â«MUSCLE BIOPSY â«ELECTROMYOGRAPHY â«NERVE CONDUCTION STUDIES â«MRI â«OTHER ROUTINE INVESTIGATIONS
- 40. â«ATYPICALâonly UMN or LMN â«Inv. of neuronsother than motor neurons â«Motor neuronal conduction block â«1.cervical â«2.MFMN CB â«3.lead poisoning â«4.thyrotoxicosis â«5.recoveryfrom poliomyelitis â«6. parkinsonism
- 41. â«No treatmentarrests the progression. â«RILUZOLEâ100mg/day â« Increases survival,18 months â« M.O.A- not known â« Decreasesglutamate release â« well tolerated â«IGF â1 â«Ceftriaxoneâanti excitotoxic â«Inhibitory RNA
- 42. â«Footdropsplints â«Fingerextension splints â«Respiratory support â«Respiratorydevices â«Cough assistdevices â«Gastrostomy â«Speech synthesizers
- 43. â«1.KENNEDY DISEASEâX LINKED SPINOBULBAR MUSCLE ATROPHY â« Males â« Androgen receptor insensitivity â« Gynaecomastia â« Absenceof pyramidal tract signs â« Sensory symptomspresent â« CAG
- 44. â«2.TAY SACHSâhexosaminidase â« Cerebellaratrophy â« Absentspasticity â« Dysarthria â«3.SMA-5q â« Floppy infant â« Infantileâwerding hoffmandisease â«4.MMNCBâ â« Improved on immunoglobulins,chemotherapy.
- 45. â«1.primary lateral sclerosis â« Sporadic â« No fasciculations â« Nodenervation â« Selective l oss of pyramidal cells â«2.familial spasticparaplegia â« AD â« Respiratory function spared
- 47. â«1.what is a motorunit?differentiate UMN and LMN signs? Add a noteon ALS ?
- 48. Thank you