long case on motor neuron disease

1. LONG CASE
Dr. Dipti Prakash Mohapatra
Post Graduate Student
P.G. Department of Medicine
S.C.B. Medical College & Hospital

2. Patients Name:- Bhumisuta Bhoi
Age –60 yrs.
Sex - Female
Address- Padampur
Occupation- Housewife
Date of admission – 02-11-2021.
Date of examination- 10-11-2021

3. CHIEF COMPLAINTS
1. Weakness and thinning of left upper limb for 6
months
2. Difficulty in speaking for 6 months with dysphagia.
3. Weakness and thinning of right upper limb and right
lower limb for 5 months.
4. Weakness and thinning of left lower limb for 5
months
5. Twitching movement over all the limbs for 2 months
6. Difficulty in rolling on bed for 2 months
7. Difficulty in respiration for last 1 month

4. HISTORY OF PRESENT ILLNESS
•The patient was apparently alright 6 months back. To
start with-
•She developed insidious onset and gradually progressive
weakness of left upper limb in a manner that she was
unable to hold a glass of water or mix her food, difficulty
in combing hair.
•For last 5 months she developed similar weakness in
right upper limb.
•She developed difficulty in speech with nasal intonation
of voice for 6 months which was insidious in onset and
gradually progressive.
•She also developed difficulty in swallowing with nasal
regurgitation of food.

5. HISTORY OF PRESENT ILLNESS
• For last 5 months she developed weakness of right and left
lower limb which is again insidious and progressive. She was
unable to get up from sitting position started waddling while
walking and found difficulty in climbing stairs.
• She has difficulty gripping slipper though she has no difficulty
in sensing it.
• She experienced difficulty in turning on bed for last 2 months.
• For last 1 month she had mild difficulty in breathing with
worsening of pre-existing symptoms.

6. HISTORY OF PRESENT ILLNESS
•Patient’s family members noticed fine involuntary
twitching movements over arm and forearm for last 2
months.
•She has no history of any root pain, tingling or
numbness.
•No history of fecal or urinary incontinence or retention.
•No history of any fever, night sweat, cough or hemoptysis
•No history of loose stool.
•No history of any emotional disturbances.

7. HISTORY OF PAST ILLNESS
•The patient was vaccinated for covid-19 6 months
back.
•She has no history of diabetes, hypertension or
thyroid disorders.

8. PERSONAL HISTORY
• Belongs to average socioeconomic group
• Mixed indian diet
• Married and blessed with a daughter and 2 sons
• Bowel and bladder are regular, sleep is adequate.
• Non-alcoholic, non-smoker.
FAMILY HISTORY
• No history of similar illness in the family.
• No family history of diabetes mellitus,
hypertension and tuberculosis

9. TREATMENT HISTORY
•She was treated at VIMSAR, BURLA for this
condition with multivitamin and baclofen.
•Now she is admitted to Department of Neurology,
SCB MCH, Cuttack under treatment.

10. GENERAL EXAMINATION
•Patient is conscious and well oriented to time,
place and person
•Thin body built with generalized muscle wasting .
•Weight = 56 kg
•Height = 164 cm
•No pallor, Icterus, Cyanosis, Clubbing, edema,
Lymphadenopathy
•JVP is not raised
•No thyromegaly
•Skin, Hair & Nail – normal
•No neuro cutaneous marker present
BMI-20.8 KG/M2

11. Pulse: 88/min, regular, normal in volume & character.
No radio-femoral or radio-radial delay,
All peripheral pulses are well felt, arterial wall
is just palpable.
Blood Pressure: 130/80 mm of Hg Right arm supine
position.
Respiratory rate: 18 /min, thoraco-abdominal.
Temperature - 98.6° F.

12. EXAMINATION OF CNS
HIGHER FUNCTION:
•Conscious, Oriented to time, place and person.
•Speech: Dysarthria with hypernasality of voice
•Normal memory and intelligence.
•No delusion, hallucination.
•Right handed person.

13. EXAMINATION OF THE CRANIAL NERVES
Olfactory nerve :
Sense of smell is intact
No parosmia
No anosmia.
Optic Nerve :
Fundoscopy: Normal
RIGHT LEFT
Visual acuity Normal Normal
Color Vision Normal Normal
Field of Vision Normal Normal

14. Oculomotor, Trochlear, Abducens:
•No Ptosis , Extraocular movements are normal in all
directions.
•Pupils are of normal size and shape in both eyes.
•Light reflex: present.
• Accomodation reflex: Present
RIGHT LEFT
DIRECT PRESENT PRESENT
CONSENSUAL PRESENT PRESENT

15. Trigeminal:
•Sensations over face, scalp normal.
•Corneal reflex present on both side
•No weakness of muscles of mastication.
•Jaw jerk- Absent
Facial nerve
• No deviation of angle of mouth, no drooling of
saliva.
• Taste sensation from anterior 2/3rd of tongue
intact.

16. Vestibulocochlear nerve :
• Rinne’s test: Positive in both ears
• Weber’s test: Not lateralized
Glossopharyngeal & vagus nerve:
• Gag reflex diminished.
• Uvula deviated to right side.
• Palatal movement diminished on right side.

17. Accessory nerve :
•No weakness of trapezius or
sternocleidomastoid bilaterally.
Hypoglossal nerve :
•Tongue atrophy- present
•Fasciculation and fibrillation- present
•No deviation of tongue to any side.

18. MOTOR SYSTEM EXAMINATION
1.BULK
•B/L atrophy of thenar and hypothenar muscles.
• There is visible fasciculation over both deltoid,
biceps, triceps, wrist flexors, extensors of thigh.
2.TONE
Normal in upper limbs and lower limbs.
RIGHT LEFT
ARM 20cms 21cms
FOREARM 19cms 20cms
THIGH 38cms 36cms
LEG 28cms 29cms

19. 3. Power:
JOINT MOVEMENT RIGHT LEFT
Shoulder Abduction 3/5 3/5
Adduction 3/5 3/5
Flexion 3/5 3/5
Extension 3/5 3/5
Elbow Flexion 3/5 3/5
Extension 3/5 3/5
Wrist Flexion 3/5 3/5
Extension 3/5 3/5
Hand grip weak weak

20. JOINT MOVEMENT RIGHT LEFT
Hip Abduction 3/5 3/5
Adduction 3/5 3/5
Flexion 3/5 3/5
Extension 3/5 3/5
Knee Flexion 3/5 3/5
Extension 3/5 3/5
Ankle Plantar flexion 2/5 2/5
Dorsi flexion
Inversion
Eversion
2/5
2/5
2/5
2/5
2/5
2/5

21. REFLEXES
DEEP TENDON REFLEX
Right Left
Upper
Limb
Biceps Brisk Brisk
Triceps Brisk Brisk
Supinator Brisk Brisk
Lower
Limb
Knee Brisk Brisk
Ankle Absent Absent

22. SUPERFICIAL REFLEX :
• Abdominal reflex – Absent
• Bilateral Plantar – Non-responsive
Hoffmann Sign- present
Wartenberg sign- present

23. 5.CO-ORDINATION : Couldn’t be tested
6. GAIT – Waddling Gait.
7. INVOLUNTARY MOVEMENT : fasciculations present
8.MENINGEAL SIGNS: Absent

24. SENSORY EXAMINATION
All primary modalities of sensation like pain, touch,
vibration, position sense and pressure are intact.
All cortical sensations are intact.

25. AUTONOMIC NERVOUS SYSTEM
•No resting tachycardia.
• No Urinary retention and constipation, urgency,
hesitancy or precipitancy.
•No Postural hypotension
•PERIPHERAL NERVE : Not thickened

26. SKULL AND SPINE
•Skull is normal in size and shape
•Spine: No swelling, tenderness, deformity

27. EXAMINATION OF CARDIOVASCULAR SYSTEM
INSPECTION :-
Precordium normal in shape
No dilated veins and visible scars seen
Apical impulse seen ½ inch medial to Lt mid clavicular
line .
No other pulsation seen.

28. PALPATION:-
Apex beat -felt in left 5th ICS at the ½ inch
Medial to Mid clavicular line, normal in
character.
No palpable sounds felt in apical area.
Pulmonary area : No Palpable P2,
No parasternal heave

29. PERCUSSION:-
2nd left intercostal space - resonant
Cardiac dullness start from 3rd ICS & does not
extend beyond apex.
Right cardiac border corresponds to right sternal
border
Left cardiac border corresponds to apex beat.

30. AUSCULTATION:-
MITRAL AREA:
S1 normal.
No murmur heard
PULMONARY AREA : P2 (N) heard. No murmur.

31. AORTIC AREA :
S1 S2 heard.
No extra sound, No murmur.
TRICUSPID AREA: S1(N) heard. No murmur.

32. EXAMINATION OF RESPIRATORY
SYSTEM
EXAMINATION OF CHEST:
 INSPECTION :-
•Trachea appears to be central in position
Apical impulse is seen to be in lt 5th ICS 1/2inch
medial to mid clavicular line.
•Chest bilaterally symmetrical. NO fullness,
hollowing, intercostal retraction
•Chest movement equal b/l

33. PALPATION:-
•Trachea is confirmed to central.
•Apical impulse is confirmed to be in lt 5th ICS ½ inch
medial to Lt MCL
•Chest expansion is 5 cm.
•Vocal fremitus is normal b/l
• There is no Intercostal tenderness.

34. PERCUSSION :-
•Direct percussion over clavicle is normal Bilatarally
•Percussion over left and right hemithorax is
normally resonant.
AUSCULTATION :-
•Normal vesicular breath sound heard
•Normal vocal resonance
•No adventitious sound.

35. EXAMINATION OF GI SYSTEM
Mouth and oral cavity normal.
INSPECTION:-
•Shape of abdomen is scaphoid
•Umbilicus central & inverted
•No engorged vein, no visible peristalsis
PALPATION:-
•Liver not enlarged, Spleen not palpable

36. PERCUSSION:-
•Abdomen is tympanitic
AUSCULTATION:-
•Bowel sound is 3-4/min
PR Examination: Not done

37. SUMMARY
A 60 year old female presented with insidious onset
progressive quadriparesis, dysphagia, dysarthria over last 6
month, and dyspnea for last one month without any
sensory loss and bowel bladder involvement.
O/E there is
1. Loss of muscle mass of both upper and lower limbs.
2. Deep tendon reflexes exaggerated in both upper and
lower limbs.
3. B/L Planter non-responsive.
4. Fasciculations and 9,10,12 cranial nerve involvement.

38. Structures involved:
1. Cranial nerve nuclei :- 9,10,12.
2.B/l Cortico spinal tract.
3.Anterior horn cell.

39. Provisional diagnosis
MOTOR NEURON DISEASE
-AMYOTROPHIC LATERAL SCLEROSIS

40. Differential diagnosis
• CV JUNCTION ANOMALY.
• SYRINGOBULBIA.
• CERVICAL COMPRESSIVE MYELOPATHY.

41. INVESTIGATIONS
 Hemoglobin: 11.1gm%
 TLC: 9200/ cmm
 Neutrophil: 76%
 Lymphocyte: 20%
 Eosinophil: 02%
 Monocyte 02%
 TPC 2 lakh/cmm
 ESR: 20 mm in 1st hr,
 Urine (Routine/Microscopy)
Albumin: Nil
Sugar: Nil
RBC: Nil
Pus Cell: 0-1/HPF
Epithelial Cell: 1-2/HPF

42. INVESTIGATIONS
 RBS: 88mg/dL
Serum Urea: 35 mg/dL
Serum Creatinine: 0.9mg/dL
Serum Sodium: 137 meq/l
Serum Potassium: 4.8 meq/l
HBsAg: Negative
HCV : Negative
HIV: Negative
Serum calcium: 2.2mmol/l (Total)
1.23mmol/l (ionised)
Liver function test:
Sr bilirubin TOTAL- 0.3
DIRECT-0.1
AST-47
ALT-46
ALP-208

43. ELECTRODIAGNOSIS
• EMG- High amplitude, Polyphasic MUAP with
incomplete recruitment seen in right FDL, left Deltoid,
right biceps brachi, right vastus medialis, and tongue
muscles.
• Spontaneous activity in the form of fasciculation seen
in right FDL, left Deltoid, right biceps, Thoracic
Paraspinal & right vastus medialis.
• Suggestive of GENERALISED ANTERIOR HORN CELL
DISEASE invloving bulbar, cervical,thoracic and
lumbosacral system.
• NCS- axonal neuropathy b/l median nerve

44. MRI Cervical Spine with Screening Whole
Spine.
• CERVICAL SPONDYLOSIS WITHOUT ANY SIGNIFICANT
NEURAL COMPROMISE.
• NO SPINAL CANAL STENOSIS.
MRI BRAIN WITH MRA
• Moderate cerebellar shrinkage

45. FINAL DIAGNOSIS
MOTOR NEURON DISEASE (CLINICALLY DEFINITE
AMYOTROPHIC LATERAL SCLEROSIS)

46. THANK YOU