- Mr. K.M.K. 63yrs old retired teacher presented with slurring of speech, increased salivation, and weakness of both upper limbs and right lower limb for 4 months with dysphagia and nasal regurgitation. Examination revealed wasting of forearm muscles with fasciculation, spastic tongue, and dysarthria. There were no sensory abnormalities, convulsions, or sphincter disturbances. The findings were consistent with motor neuron disease.

1. SATURDAY FORUM.SATURDAY FORUM.
1414/ 05/ 2005/ 05/ 2005
Dr.Shantha Herath.Dr.Shantha Herath.

2.  Mr. K.M.K. 63yrs old retired teacher from WalapaneMr. K.M.K. 63yrs old retired teacher from Walapane
 c/o slurring in speechc/o slurring in speech
weakness of the R/S of the body / 4/12weakness of the R/S of the body / 4/12
 H.P.CH.P.C -pt was well before 4/12. Then he developed slurring of-pt was well before 4/12. Then he developed slurring of
 speech which was gradually progressive. After about 1/12,speech which was gradually progressive. After about 1/12,
 he noticed that increase salivation too and also developedhe noticed that increase salivation too and also developed
 difficulty in swallowing and sometimes nasal regurgitationdifficulty in swallowing and sometimes nasal regurgitation
 of fluid.of fluid.
 Sametime he noticed some weakness of the R/S ofSametime he noticed some weakness of the R/S of
 the body which was also progressive and about 3/12 laterthe body which was also progressive and about 3/12 later
 he noticed that his left arm also become weak.he noticed that his left arm also become weak.
 No convulsions, no numbness of the bodyNo convulsions, no numbness of the body
 No mouth ulcers,No mouth ulcers,

3.  No impairment of vision or double visionNo impairment of vision or double vision
 No retention or incontinence of urine or fecesNo retention or incontinence of urine or feces
no chronic cough,or haemoptysisno chronic cough,or haemoptysis
P.M.HP.M.H.. Diagnosed case of intermittent AF and calcific aortic valveDiagnosed case of intermittent AF and calcific aortic valve
 No D.M, HTNo D.M, HT
 No H/O polio myolitesNo H/O polio myolites
 No H/O musculo-skeletal disorderNo H/O musculo-skeletal disorder
 Drug/HDrug/H on warfarin for AFon warfarin for AF
no other drug history availableno other drug history available
 Social H.Social H. - lives with chilren- lives with chilren
- monthly income 6000/=- monthly income 6000/=
- adequate diet- adequate diet
- non-smoker- non-smoker
-non alcoholic-non alcoholic

4. Family- History - 4 childrenFamily- History - 4 children
- 3 boys- 3 boys
- wife died 5 years back- wife died 5 years back
- all are healthy- all are healthy
- no similar illness- no similar illness

5. SummarySummary
-- Mr. K.M.K. 63yrs old retired teacherMr. K.M.K. 63yrs old retired teacher
presented withpresented with
difficulty in speech, increasedifficulty in speech, increase
salivation and weakness ofsalivation and weakness of
both upper limbs and R/ lowerboth upper limbs and R/ lower
limb. For 4/12 with dysphagia and nasallimb. For 4/12 with dysphagia and nasal
regurgitation. Withregurgitation. With
no convalsions, no numbness of theno convalsions, no numbness of the
body , no visual impairment or diplopiabody , no visual impairment or diplopia
or sphincter disturbances.or sphincter disturbances.

6. EXAMINATIONEXAMINATION
G/E.G/E. ill lookingill looking
Conscious and rationalConscious and rational
No ptosis, pupils normalNo ptosis, pupils normal
Not pale, not ictericNot pale, not icteric
No CyanosisNo Cyanosis
No generalized lymphadenopathyNo generalized lymphadenopathy
No clubbing or splinter haemorrageNo clubbing or splinter haemorrage
No peripheral edemaNo peripheral edema

7. C.V.S BP 140/85 mmHgC.V.S BP 140/85 mmHg
Pulse 100/mm, irregularly irregularPulse 100/mm, irregularly irregular
No mNo m
R.SR.S Clinically normalClinically normal
AbdAbd Clinically normalClinically normal
C.N.S. Higher functions normalC.N.S. Higher functions normal

8. CRANIAL NERVECRANIAL NERVE
I – VIII – Clinically normalI – VIII – Clinically normal
- Spastic tongue with fasciculationSpastic tongue with fasciculation
- Bilateral palatal weakness +Bilateral palatal weakness +
- Slurring of Speech +Slurring of Speech +
UPPER LIMBSUPPER LIMBS
- Wasting of small muscle of both handsWasting of small muscle of both hands
and muscle of forearm with fasciculationand muscle of forearm with fasciculation
- Deep guttering of dorsum of both hands.Deep guttering of dorsum of both hands.

9. R/ULR/UL L/ULL/UL
PowerPower 4/54/5 4/54/5
ToneTone SlightlySlightly SlightlySlightly
ReflexesReflexes BJ- BriskBJ- Brisk BriskBrisk
TJ- BriskTJ- Brisk BriskBrisk
SJ- BriskSJ- Brisk BriskBrisk

10. LOWER LIMBSLOWER LIMBS
R/LCR/LC L/LCL/LC
PowerPower 4/54/5 5/55/5
ToneTone IncreaseIncrease NormalNormal
ReflexesReflexes KJ- BriskKJ- Brisk NormalNormal
AJ- BriskAJ- Brisk NormalNormal
Planter- ?Planter- ? NormalNormal

11.  No sensory involvementNo sensory involvement
 Cerebellar functions normalCerebellar functions normal
SUMMARYSUMMARY
 - Mr. K.M.K. 63yrs old retired teacher presented with- Mr. K.M.K. 63yrs old retired teacher presented with
slurring of speech, and weakness of both upperslurring of speech, and weakness of both upper
limbs and R/ lower limb. For 4/12 with dysphagia andlimbs and R/ lower limb. For 4/12 with dysphagia and
nasal regurgitation and wasting of both forearms andnasal regurgitation and wasting of both forearms and
hand muscles with fasciculation and also spastic tonguehand muscles with fasciculation and also spastic tongue
with dysarthria. With no convulsions, no numbness of thewith dysarthria. With no convulsions, no numbness of the
body , no visual impairment or diplopia or sphincterbody , no visual impairment or diplopia or sphincter
disturbances.disturbances.

12. Motor Neuron DiseaseMotor Neuron Disease
-Selective loss of functions of lower/upper motorSelective loss of functions of lower/upper motor
neuronsneurons
- The voluntary muscles of limbs and bulbar region- The voluntary muscles of limbs and bulbar region
- Family of diseases- Family of diseases
- In practice – DDS – on – clinically, electro- In practice – DDS – on – clinically, electro
physiologicallyphysiologically
-In clinical diagnosisIn clinical diagnosis
- -Anatomical differentiation is augmented by-Anatomical differentiation is augmented by
- age of onset- age of onset
-rate of deterioration-rate of deterioration
- familial occurrence- familial occurrence
Sensation and cognition – normalSensation and cognition – normal
-Lower motor neuron diseaseLower motor neuron disease
- Wasting- Wasting
- Fasciculation- Fasciculation
- - Flaccid weakness- Flaccid weakness

13. ** * Tendon reflexes are retained until late stage* Tendon reflexes are retained until late stage
 * Nerve conduction studies to exclude peripheral* Nerve conduction studies to exclude peripheral
neuropahtyneuropahty
 * Muscle biopsy –* Muscle biopsy –
 to exclude myopathy.to exclude myopathy.
eg.-progressive proximal limbeg.-progressive proximal limb
weaknessweakness
-bulbar-bulbar
weaknessweakness
 upper motor neuron diseasesupper motor neuron diseases
- spasticity- spasticity
 - clonus- clonus

14. * extensor planter response* extensor planter response
- weakness- weakness
 *abd – reflex often preserved.*abd – reflex often preserved.
 * Sphincters and sexual functions usually preserved* Sphincters and sexual functions usually preserved
 M.N.D.-incurableM.N.D.-incurable
 - but- help disabilities- but- help disabilities
 Eg: -malnutrition due to dysphasiaEg: -malnutrition due to dysphasia
- NG tube- NG tube
- gastrostomy- gastrostomy
 - assisted ventilation- assisted ventilation
 -spasticity-spasticity
drugs – Beclofendrugs – Beclofen
DantroleneDantrolene
diazapamdiazapam

15.  - Weel chairs- Weel chairs
 - Amitriptallin for emotional lability of bulbar palsy- Amitriptallin for emotional lability of bulbar palsy
Classification of M.N.DClassification of M.N.D
* - A.L.S* - A.L.S
- sporadic- sporadic
- familial adult onset (A/D)- familial adult onset (A/D)
- familial juvenile onset (A/R)- familial juvenile onset (A/R)
 * Lower motorneuron disease* Lower motorneuron disease
 - proximal hereditary M.N.O- proximal hereditary M.N.O

16.  -acute infantile (warding Hoffman)type-acute infantile (warding Hoffman)type
 - chronic childhood form- chronic childhood form
 (Kugelburg-welander) A/R(Kugelburg-welander) A/R
 - adult onset-AR- adult onset-AR
- adult onset-AD- adult onset-AD
- hereditary bulbar palsy- hereditary bulbar palsy
 - with deafness- with deafness
 - without deafness- without deafness
 - X linked bulbospinal neuropathy- X linked bulbospinal neuropathy
 (Kennady syndrome)(Kennady syndrome)
 - multifocal motor neuropathy- multifocal motor neuropathy
 - post polio syndrome- post polio syndrome

17.  - post irradiation syndrome- post irradiation syndrome
 * Upper motor neuron syndrome* Upper motor neuron syndrome
 - primary lateral scleroses- primary lateral scleroses
 - hereditary spastic paraplegia (AD)- hereditary spastic paraplegia (AD)
 - lathyrism- lathyrism
 Upper/Lower M.N.DUpper/Lower M.N.D
ALS - male>femaleALS - male>female
- with age- with age
- unusual before 5- unusual before 5thth
decaddecad
- unknown cause- unknown cause
- ass c dementia and parkinsonism- ass c dementia and parkinsonism

18.  Pathology-Pathology-
 - L.M. neuron loss in the cord and brain stem- L.M. neuron loss in the cord and brain stem
 - bumina bodies in surviving neuron- bumina bodies in surviving neuron
 - Betz cells in motor cortex- Betz cells in motor cortex
 - pyramidal tract degeneration- pyramidal tract degeneration
 Clinical features-Clinical features-
 - balbar-spinal symptoms- balbar-spinal symptoms
 Prognosis- median survival range 20/12Prognosis- median survival range 20/12
 Rx -Rx -
 - speech therapist- speech therapist
 - occupational therapist- occupational therapist
 - social worker- social worker
 - physician- physician

19. THANK YOU.THANK YOU.