MOTOR NEURON DISEASE

DEFINITION :
- Diseases or conditions that produce dysfunction of the motor neurons either upper or
lower motor neuron or both
- Classified according to location of moement!
I! "##E$ %OTO$ NE"$ON DI&E'&E ( "%D )
'! #$I%'$* +'TE$'+ &C+E$O&I& ( #+& )
- a rare, non-familial slowly progressie corticobulbar - corticospinal tract disease of
un.nown cause!
- 'ge of onset : /0-10 y2o
- Incidence : %3F
- Clinical manifestations
: spasticity on +E which will progress at "E and bulbar musculature!
: spastic dysphagia is life threatening 4 urinary incontinence occurs later
: surial is usually /-5 more decades
6! T$O#IC'+ &#'&TIC #'$'#'$E&I&
- found in clusters in the tropics as well as in Central &outh 'merica, India, 'frica and
7apan!
- Occurs primarily in blac.s - adults
- %3F
C! E#IDE%IC &#'&TIC #'$'#'$E&I&
- clinically similar to tropical spastic paraparesis but differs in such that this disorder also
affects children - because patients are seronegatie for human T-cell lymphotropic irus
type 8 ( 9T+:-8 )
D! +'T9*$I&%
- an disorder secondary to e;cessie consumption of chic.ling pea ( +athyrus &atius )
or its relaties 4 endemic in Indian subcontinent
- etiology : <-N o;alylamino - + - alanine ( 6O'') a to;ic agent agonist of the e;citatory
neurotransmitter glutamate!
- = % 4 are more seerely affected
- age of onset : % > ?-@0y2o, F 1-/0 y2o, both se;es 3 /-A y2o
E! F'%I+I'+ 9E$EDIT'$* &#'&TIC #'$'#+EBI'
- can be transmitted as autosomal dominant, occasionally as an autosomal recessie -
ery rarely as an ;-lin.ed recessie syndrome
- usually occurs in childhood or early adult life
- clinical manifestation :
: Complaints of stiffness and unsteadiness of the legs - gradually results
in a spastic paraplegia4 muscular atrophy
- un.nown pathogenesis
II! CO%6INED "%ND - +%ND
'! C+'&&IC '%*OT$O#9IC +'TE$'+ &C+E$O&I& ( '+& )
- benchmar. of motor neuron disorder 4 most common motor neuron disorder
- can either be progressie bulbar palsy ( affects 6ulbar %otor Neurons ) or progressie
muscular atrophy ( affects &pinal %otor Neurons )
- %=F ( ae! age at time of prognosis 3 1/ yrs! )
- Clinical %anifestations
: initially +%N signs ( atrophy, fasciculations, decrease DT$, wea.ness )
4later "%N signs ( spasticity, increase DT$, babins.i )
8C8
: affectation : unilateral -= bilateral 4 distal ( hands) -= pro;imal -= bulbar
musculature -=
trun. - +E
: muscle cramping 4 difficulty in ambulation secondary to spasticity -
clonus
: most stri.ing feature : focal asymmetrical wea.ness
: constipation due to poor nutritional inta.e or inactiity
: spares : bowel and bladder function 4 sensory 4 autonomic intellect
- un.nown pathogenesis
6! F'%I+I'+ '+&
- clinically same as classic '+& but has a younger onset ( @1 y2o ) and shorter surial
which is bimodal in distribution 4 pea.s at / and 8/ yrs! from time of diagnosis
- un.nown pathogenesis
- damaged proprioception due to degeneratie changes in middle Dones of dorsal
column, spinocerebellar tract - dorsal nucleus of Clar.e
C! 7":ENI+E INC+"&ION 6OD* '+&
- a rare condition clinically identical to '+& but more younger onset ( 8/-81 y2o ) - much
shorter surial
D! EE&TE$N #'CIFIC '+& - #'$FIN&ONI&% DE%ENTI' CO%#+EG
- high incidence in Eestern #acific
- Clinically same as classic '+& but with the signs and symptoms of par.insonism and
dementia
E! #O&TENCE#9'+ITIC '+&
- same with postencephalitic #D with an aerage onset of '+& of 80 yrs!
III! +OEE$ %OTO$ NE"$ON DI&E'&E
'! &#IN'+ %"&C"+'$ 'T$O#9* ( &%' )
- 9ereditary %otor Neuropathy ( 9%N ) resulting in the degeneration of the ant! horn
cells
- Clinical manifestation : hypotonia 4 wea.ness 4 CN palsy
- differentiation :
&%' s! '+&
- +%ND - "%ND - +%ND
- +E -= "E - "E-=+E
- &ymmetrical at onset - unilateral at onset -= symmetrical
later
- #ro;imal -= distal - distal -= pro;imal
- slowly progressie ( e;cept Type 8 &%' ) - slowly progressie
- types
8! INF'NTI+E &%'
a! 'cute Infantile 2 Type 8 2 Eerdnig 9offman
- most seere type of &%' 4 autosomal recessie disorder 4 D; : at 5 mos!
- Clinical manifestations :
9ypotonia 4 atrophy
#rogressie muscle wea.ness
- fasciculations ( tongue fasciculation - almost pathognomonic for the disease ) 4
arefle;ia
- death secondary to respiratory failure ( intercostals muscle paralysis )
- un.nown pathogenesis
b! Chronic Infantile 2 Type II 2 Chronic Eerdnig 9offman
- autosomal recessie ( gene : Chromosome ?H ) 4 more slowly progressie
8C/
- Clinical signs present by 5mos! - 5yrs! 4 median age of death at 8/yrs, some
surie till the 5rd decade
- Clinical manifestations :
wea.ness - pro;imal atrophy - lower limbs = "E
decreased or (-) DT$ 4 normal sensation
&coliosis, thoracic, eIuines deformities of foot
- un.nown pathogenesis
/! 7":ENI+E 'ND 'D"+T &%'
- autosomal recessie inheritance 4 surial : J/0 yrs!
a! 7uenile - 'dult #ro;imal &%' 2 Type III 2 Fugelberg-Eelander 4 Type I: 4 Type :
- characteriDed by slowly progressie wea.ness - atrophy of the pro;imal limb, pelic
girdle musculature, face, tongue and pharyn;
- onset : usually /-8A y2o 4 duration range : /-@0 yrs 4 %=F
- Clinical %anifestations:
&ymmetrical atrophy - wea.ness of pelic girdle, pro;! lower limbs -=
inoling shoulder girdle - upper arms -= leg and forearms!
Fasciculations 4 %ild Dysphagia and Dysarthria
- un.nown pathogy
b! 6ulbar disease of Childhood
- slowly progressie wea.ness of facial muscles, tongue - pharyn; 4 onset : /-8/ yrs!
Type 8 6rown -:ialetto - :an +eare Type / FaDio +onde
6ilateral deafness (8st symptom) (>) (-)
CN affectation CN A-8/ all CN
c! Distal &%'
- a!.!a! K&pinal form of Charcot %arie Tooth diseaseK - KDistal 9ereditary %otor
NeuropathyK
- autosomal recessie Luenile 4 onset : mild : /-80y2o 4 seere: @ mos!-/0 y2o
- autosomal dominant onset : in Luenile : /-/0 y2o 4 in adults : /0-@0 y2o
clinical manifestations : wea.ness - atrophy distally in the legs esp! in ant! tibial -
peroneal muscle, may also inole thigh muscle
- un.nown pathogy
5! 'D"+T FO$% OF 6"+6'$ 2 6"+6O&#IN'+ %"&C"+'$ 'T$O#9IE&
a! &capuloperoneal 2 Fascioscapuloperoneal %'
- autosomal dominant 4 slow progression 4 onset : 50-?0 y2o
- progression of wea.ness and atrophy -= begins in leg muscles ( intrinsic muscles
are spared ) -= shoulder girdle muscle -= thigh -= pelic girdle , upper arm, nec.
and face
- (>) of dysphagia and dystonia
b! Chronic 6ulbospinal %uscular 'trophy of +ate Onset
- a!.!a! KFennedyMs DiseaseK
- slow progressie pro;imal spinal and bulbar musculature atrophy 4 onset : 5rd -
1th decade
- ;-lin.ed recessie with se; limited e;pression ( gene defect localiDed to pro;imal
long arm of ;-chromosome)
clinical manifestations: pro;! muscular wea.ness ( shoulder - pelic girdle) - distal
muscle wea.ness but not as seere as in the pro;imal muscles4 bulbar changes
( dysarthria, dysphagia, atrophy of tongue )
- un.nown pathology
c! %onomelic ( &egmental ) &%'
- not an inherited disorder 4 primarily occurs in the far east 4 segmental in
distribution
- male Lueniles or young adults4 insidiously progressie oer 8 N 5 years - almost
always remains focal
@! %*OC+ON'+ B'%%O#'T9* - %OTO$ NE"$ON DI&E'&E
8C5
- occur in association with paraproteinemia4 can be +%N only or combined +%N
-"%N identical with '+&
- diffuse muscle wea.ness - atrophy are seen in patients with +%N findings
6! #O+IO%*E+ITI&
- a highly communicable - infectious acute infection caused by a group of
enteroiruses
- attac.s primarily the BIT, secondarily the nerous system
- irus tends to affect the anterior horn cells of Brey %atter of spinal cord - brain
stem resulting to flaccid paralysis
- a!.!a! K9eine %edin DiseaseK4 K'cute 'nterior #oliomyelitisK4 KInfantile #aralysisK
- %=F 4 mostly affects children - young adults
- C?-CCO of patients are symptomatic4 common during the summer
Etiology
- etiological agent : #O+IO:I$"&4 $N' irus, member of enteroirus grp! of
picornairus family4
- 5 types ( specific action on each type with no cross immunity)
Type 8 6runhilde : most common - paralytogenic ( seere)
Type / +ansing : /nd most paralytic - freIuent
Type 5 +eon : least seer and common
- #redisposing Factors
'ge - maLority of cases occur in J8? y2o
Climate - summer and early fall4 more freIuent in temperate Dones
Oral &urgery - increased ris. with tonsils remoed and tooth e;tracted
+actation - &urgery
Fatigue - Oere;ertion
$ecent "pper $espiratory Tract Infection ("$TI)
#athophysiology
- route: fecal-oral 4 mouth ( ingestion) - irus multiplies in lymphoid tissue of
nasopharyn; - BIT 4 blood ( iremia) 4 incubation period : A-8@ days
Clinical Types
'! 'symptomatic Carrier Type 2 Inapparent infection
- no symptom but irus is present in the pharyn; - intestine usually seen in
the blood
6! 'bortie 2 %inor Illness
- nonspecific febrile illness
C! Non-paralytic #olio
- signs present :
tight nec., bac. - hamstring muscle
(>) Fernig, 6rudDens.i - spine signs
head drop sign
changes in superficial - deep refle;es
usually an eleated spinal fluid cell count
D! #aralytic #olio
@ Types
8! &pinal - most freIuent4 inolement of e;tremities - trun.4 wea.ness in C,
T, +, areas
/! 6ulbar - most seere4 affects brainstem, CN&, resp!, cardiac - asomotor centers
are inoled
5! 6ulbospinal - mi;ed type
@! #olioencephalitis - rare4 inoles brainstem4 stupor, coma, hyperirritability
- conulsions may occur
Clinical %anifestations
'! 'cute 2 early phase ( 8-1 w.s )
8! Nonparalytic2 preparalytic
- feer, headache, BIT symptoms, muscle tenderness 2 pain4 meningeal
irritation
/! #aralytic
8C@
- assymetrical patchy wea.ness ( hallmar.)4 +E="E4 pro;imal=distal
most common in +E : Iuads, hip e;tensors4 in "E : deltoids
- (>) +%N+ signs
6! Intermediate 2 Conalescent #hase ( 1 w.s! - 1 mos! )
- atrophy - contractures
C! +ate 2 $esidual 2 permanent phase ( = 1 mos! )
- deformities : shortening of limbs - eIuinoarus 4 scoliosis - genu
recuratum
Complications
- dysphagia
- respiratory failures4 cardioascular complications
- atrophy4 facial contracture
- genu recuratum4 limb shortening - malalignment of Loint structures
#rognosis
- $ecoery depends on
8! no! of motor neurons
a! unimpaired
b! that recoer - resume normal function
c! that deelop sprouting
/! age
5! duration of paralysis : after 1 months 3 permanent paralysis
@! type of polio : 'bortie - nonparalytic 3 complete recoery
#aralytic 3 maLority with residual paralysis
%edical - &urgical %anagement
Treatment is mainly supportie designed to preent respiratory complications
- aoid Loint contractures during acute - conalescent stages
In the residual stage, operations are performed to :
Correct deformities - reduce the need for braces
Improe muscle balance and stabiliDe the Loint
#rocedures include
'! Operations for the correction of deformities of long standing (hip- .nee fle;n!
Contracture)
- surgical release of fascia, muscle or tendons when deformities cannot be corrected
by casts and e;ercises
- preent recurrence after correction by
bracing, surgical stabiliDation for bony structures - tendon transfers for muscle
balance
6! Operation on %uscles and Tendons
Tenotomy - seering of a tendon to oercome a contracture of the muscle
attached to it
%yotomy - diision of muscles for the same purpose
Fasciotomy
C! Operation on bones - Loints
Osteotomy - cutting a wedge from a bone - closing the incision so as to alter
the bone line
'rthrodesis - most impt! of operations4 done by remoing the articular
cartilage, cutting thin slices from bones - fi;ing them in apposition!
D! Operations to eIualiDe the length of the legs
'fter poliomyelitis, growth of affected leg may decline! Degree of shortening
depends on the seerity of paralysis - age at which the paralysis began!
8! Epiphyseal 'rrest - procedure done to slow the growth of a normal bone,
correct the discrepancy between the / +E in children with =/ cm!
/! +eg +engthening - for children with ++D of =1-P cm!
5! +eg shortening - for adults4 when epiphyseal can no longer be done - leg
lengthening procedures are dangerous4 a segment of bone is resected!
E! Operations on the Neres
8C?
Nere anastomosis - less common, consists of Loining seeral healthy neres to
distal end of cut nere!
#T Ealuation - 'ssessment
'! Electrodiagnosis
- &ome - possibly all muscles will be reinnerated by either terminal a;on sprouting or
regrowth of a;on bac. to the muscles
- the results of inneration by sprouting from terminal a;on are more muscle fibers
per motor unit giing an increase number of diaphragmatic spi.es
- an increase delay in transmission can be recorded during the 8st 1-8/ mos! of
reinneration as newly formed a;ons sprout - end plates mature! This will result in
instability or ariability of motor unit action potential in siDe - shape with repetitie
discharge
6! Tonal 'ssessment
8! #assie motion Testing
- limbs fell heay and unresponsie upon moement
/! Drop Test 2 #lacing test
- the arm or leg is passiely raised by the therapist - is suddenly
dropped! Normal limb temporarily falls then maintains position! ' hypotonic limb would
fall abruptly!
C! $O%
- especially hip - .nee fle;ion 2 e;tension - an.le dorsifle;ion
D! %%T
- with special attention to hip fle;ors - e;tensors
E! DT$
F! &pecial Tests
a! 6rudDens.iMs Test - indicates meningeal irritation! #t! lies supine, head is eleated
from table! Test is (>) is pt! c2o of nec. - low bac. discomfort - tries to reliee
discomfort by inoluntary fle;ion of hips and .nees!
b! FernigMs Test - for meningeal or nere root irritation! #t! lies supine with hip - .nee
fle;ed! #ositie if thereMs pain in low bac., nec. - or head!
c! +eg +ength Test - #t! lies supine with pelis set sIuare or balanced on legs! +egs
should be 8?-/0cm apart - parallel to each other! The e;aminer places one end of tape
measure against distal aspect of '&I&, holding it firmly against the bone! The inde;
finger of other hand is placed immediately distal to the medial or lateral malleolus -
pushed against it! $eading is ta.en where the thumb and finger touch together! ' slight
difference upto 8!0-8!? cm is considered normal but can still be significant!
B! #osture and Bait 'nalysis
C! #O&T #O+IO &*ND$O%E
- onset of new health - 'D+ problems seeral years after acute poliomyelitis
- theory of etiology : motor neurons preiously damaged by acute poliomyelitis
- criteria
(>) history of paralytic poliomyelitis
partial 2 fairly complete neurological - functional recoery
period of neurological- functional stability for 8? yrs!
onset of = / health problems
unaccustomed fatigue - most common
muscle and Loint pain 4 wea.ness in preiously affected and unaffected muscle
cold intolerance
new atrophy
no other diagnosis to e;plain problem
- ae! age of onset of typical post polio patient : ?-80 yrs
- New 'D+ problems:
Eal.ing - most common
&tair climbing - dressing
8C1
#$INCI#+E& OF $E9'6I+IT'TION %'N'BE%ENT
I! BENE$'+ #T %'N'BE%ENT
'! #$O&#ECTI:E C'$E
- inclines all usual measures proided to all people regards loss of their health
status!
6! EG#ECT'NT C'$E
- anticipation of complications that might be e;pected during the course of pts!
%otor neuron disease that includes
pain and wea.ness
muscle tightness
deformities of bones and Loints
impaired entilation and functional actiities
8! %anagement of #ain
- in acute poliomyelitis
- physical treatment : heat treatments with stretching
- pharmacologic treatment : salicylates or N&'ID&, narcotic analgesics,
tricyclic antidepressants for neurotic pain
/! %anagement - preention of muscle tightness - soft tissue contracures
- application of superficial heat
- #$O% - '$O%
- actie stretching - appropriate positioning
- bracing
II! &#ECIFIC #T %'N'BE%ENT
'! #O+IO%*E+ITI& - #O&T #O+IO &*ND$O%E
8! #$E-#'$'+*TIC - #'$'+*TIC &T'BE&
ObLectie: preention of deformity
Treatment:
a! Constant bed rest - pt! placed in absolute bed rest until all meningeal
symptoms hae disappeared - adeIuate fluid inta.e is proided! &edaties are
contraindicated because of their depressant effects on the CN&! Intensie re education
should not begin @-1 w.s!, by which all symptoms hae disappeared!
b! &plinting or supporting Loints which are placed in neutral position to aoid
contractures!
c! #roper bed positioning
- pt! is placed in a firm bed with boards beneath the mattress
- pt! lies flat on his bac. with hip - .nee fle;ed by a ery small pillow
under the .nee
- padded footboard maintains foot in a neutral position - preents footdrop
deformity
"E position : hips - abducted - in neutral position
Fnees - slight fle;ion with feet at right angles to the legs
d! Fenny 9ot #ac.s 2 9%#
- should coer the entire muscle grps! 4 acts as counter irritant
- repeated application rela;es muscle, decreases pain, spasm - soreness
thus brea.ing the muscle spasm - pain cycle!
e! #$O%
- begin '&'# to obtain at least a useful $O%
- ery impt! in early stages since muscles are deried of their nere supply
- gien gently so as not to stretch the neres or press on tender muscle
which may increase pain - spasm
f! %assage
- ery gentle effleurage - .neading with light friction around Loints
- impt!: to maintain circulation in paralyDed muscles
/! &T'BE OF CON:'+E&CENCE O$ $ECO:E$*
ObLecties: #reention of contractures - deformities
$etard atrophy - teach muscle re education
8CA
$estoration of the loss of Loint motion
Treatment:
a! #roper bed positioning (#6#)
- dropped foot is .ept at right angle
b! %assage
- more stimulatory but neer be heay of forcible
- effleurage - .neading 4 stretching - manipulation of the antagonist
muscle
c! #$O%
- continued as before to preent contractures - presere Loint mobility
d! &tretching
- muscles liable for stretching: &houlder 'bductors, hip, calf, bac.
muscle - an.le dorsifle;ors
e! '$O%
- begin slowly - gradually: most impt! part of treatment4 increases
strength - f;n!
f! Ehirlpool 6ath
- improes circulation
g! #ool Therapy
- warmth of water improes circulation- prepares muscles for wor.
- graity is eliminated - buoyancy of water is used to assist moements
- water should reach halfway up the pts! chest for support - assistance
in leg moements4 progress to shallower water!
- should be followed by /0 minute rest4 wal.ing re education can start
in the pool
h! #NF
- repeated contractions : reersal techniIues, rhythmic stabiliDation
i! Fitting of Orthotic Deice
- to preent deformity: protect wea.ened muscle groups
- to proide support : increase function of Loints - e;tremities
L! Electrotherapy
- useful in older patients 4 improes nutrition for paralyDed muscles
Faradism - used according to the needs of the case but not suitable for
children
&urging &inusoidal Current - for warming deep structures of the body
I$$ - used before e;ercise specially if limbs are cold
":$ - sometimes gien for a general tonic effect
5! $E&ID"'+ C9$ONIC &T'BE 2 &T'BE OF #E$%'NENT DI&'6I+IT*
ObLecties: restoration of whateer useful function - correction of deformities
Treatment :
a! #roision of suitable apparatus for support
- caliper with special boots - for cases where muscles around hips are
paralyDed
- progression in wal.ing: horiDontal bars-=crutches-=stic.s-=unaided
b! &urgical Treatment
6! DEFO$%IT*
- #ostural asymmetry in positioning .ids in wheelchairs with surge type seat
leads to contractures and to subseIuent deformity
- %inimal wheelchair should include: firm seat with adeIuate bac. and arm
support
- aoid malalignment by appropriate stretching, positioning and bracing
- surgical interention
C! &CO+IO&I&
- prescribe the appropriate wheelchair for the indiidual child
Criteria:
E2C be measured for each child after assessing the childMs needs
8CP
Child must maintain symmetrical sitting posture with adeIuate upper and +E
support
The sling should be aoided since it permits asymmetrical pelic rotation
' solid foam-padded seat cushion can be used to leel pelis
D! EE'FNE&&
- %anagement might include strengthening e;! if prescribed Ludiciously
following carefully
- +ow intensity, non fatiguing e;! may be beneficial for maintainance
E! $E&#I$'TO$* '&&I&T'NCE
- 9ypo;ia occurs secondary to motor wea.ness and deformity
- %anagement: appropriately prescribed entilatory aids enhance gas e;change in
recumbent position
- Can be used anytime using the E2C battery
Oral positie pressure entilation, pneumobelt, cuirass entilators
F! F"NCTION'+ '6I+IT*
- #rimary goals of rehab team in treating a pt! with a motor neuron dse! are: assist pt!
in maintainance of f;n, independence - Iuality of life for as long as possible!
- $ehab team - family coordinated prospectie - e;pectant care of the pt!
- Functional training for 'D+ are practiced as deelopmentally appropriate
- 'ssistie deices, substitution training - selectiely surgical procedures
B! F'TIB"E
- reduced by :
increasing rest time, napping or reducing oerall leel of actiity during the
day
reducing physical - emotional stress
use of orthotic deices or aids to enhance pt!Ms ability to function
psychological counseling or a post polio support group
8CC