A 40-year-old male patient presented with a 28-day history of gradual progressive weakness in both upper and lower limbs, characterized by difficulty in performing daily activities and dysphagia. Examination revealed proximal muscle weakness, reduced deep tendon reflexes, and no sensory or bowel/bladder involvement, alongside significant laboratory findings indicating inflammatory myopathy. Provisional diagnosis suggests inflammatory myopathy, with differential diagnoses including CIDP, motor neuron disease, and myasthenia gravis; muscle biopsy reports are awaited for confirmation.

1. LONG CASE
EXAMINATION OF NERVOUS SYSTEM
DR.ROMANCHIT UPADHYAYA
2ND Year Junior Resident
Department of General Medicine
S.C.B. Medical College & Hospital

2. Name-Bholanath dhal
Age : 40
Sex :male
Religion: hindu
Address:mayurbhanj
Occupation: masonry
Handedness: RIGHT
Informant: Self ,his wife

3. PRESENTING COMPLAINT
• Weakness of both lower limb since 28 days
• Weakness of both upper limb since 28 days

4. HISTORY OF PRESENT ILLNESS
• Patient was apprently all right 28 days back to start with
he developed difficulty in getting up from sitting
position,however he was able to get up from sitting by
placing hands over chair,after getting up he had no
difficulty in walking
• Also 28 days back he noticed difficulty in both upper
limb ,as he tried to put a bucket of water over his head
which he was not able to do ,but could able to hold the
mug
• 4 days later weakness progressed such that he started
taking a stick support to walk,at this point he denies any
difficulty to wear,grip slipper or slippage of the same
during walking,also he had difficulty in lifting objects and
taking food into mouth ,but he could able to pick objects
with hands,mix foods ,tear rotis

5. • For this he was admitted to baripada hospital,where the
weakness progressed to such extent that he started taking one
persons support to stand from sitting position,he described he
had difficulty in lifting his legs to walk.At that point of time he
has difficulty in picking objects,mixing foods.4 days later he
was not able to get up from bed and change sides,so his
attendants has to carry him for his daily pursuits and routine
works.The weakness was associated with dull pain on any
activity
• Then he developed difficulty in raising head while lying on bed
for which he was referred to SCB MCH
• For the last 8 days he had difficulty in swallowing,his
attendants described that he can able to chew,make bolus and
grind food by side to side movement of jaw,can push the food
back without cough or nasal regurtitation,following which he
felt as if food is stuck in midthroat and to make it swallow he
had to drink water everytime.Gradualy it progressed such that
he is not able to swallow any food or water .

6. He dines accumulation of food in gutters or leakage from angle of mouth.
Dysphagia was not associated with nausea,vomiting,cough
No h/o diurnal variation of weakness,not related to exhaustion
No h/o cramps/twitching/stiffness
H/o reduced appetite for 1and 1/2 months
His attendants denied any appreciable weightloss,thinning of muscles,loosening
of clothes recently
H/o yellowish discolouration of skin and sclera 1 and 1/2 months back which
subsided on medication
H/o blackish discolouration of face and upper arm since 3 months

7. No h/o band like sensation
No h/o sensory disturbance
No h/o bladder ,bowel disturbances
No h/o altered sensorium,fever,seizure
No h/o blood in vomitus,black tarry stool
No h/o ringing in ears or hearing loss
No h/o recent changes in voices
No h/o swelling of feet,facial puffiness,orthopnea,pnd,chronic cough,evening rise of
temperature

8. • No history of blurring of vision, diplopia, drooping of eyelids, facial
deviation,
• No history of any nasal regurgitation of foods.
• No history of any trauma.
• No history of heavy weight lifting.
• No history of joint pain, photosensitivity.
• No history of chronic back pain
• No history of exercise intolerance or red colored urine

9. HISTORY OF PAST ILLNESS
• No history of similar complaints in the past.
• No history of any malignancy.
No history of HTN T2DM or any chronic disease.
No history of any treatment with a drug for a long time.
No history of use of alternate or indigenous medication

10. PERSONAL HISTORY
•Belongs to lower socioeconomic group.
• schooling up to matriculation
•married since 15years blessed with 2 child.
•Takes both veg and non veg diet.
•He drinks alcohol on occasions since 4years,and abstinent for last
3 months
FAMILY HISTORY
no such history present in the family.

11. TREATMENT HISTORY
He is admitted to MMW6, Department of medicine, SCBMCH and
currently receiving:
(1) Inj ceftriaxone 1gm iv bd
(2) inj ondem 8 iv sos
(3) Inj methylcobalamin 1500 im od
(4) Inj pantoprazole 40 iv od

12. SUMMARY
• A 40 Years old male presented with insidious onset gradual
progressive weakness of both upper and lower limb in the
form of proximal muscle involvement in the form of difficulty
in getting up from sitting position , , unable to perform
activity that are required above head motion like bathing or
combing hairs over a period of 28 days with truncal and neck
muscle involevement and dysphagia as evidence by difficulty
in raising head while lying on bed,difficulty in getting up
from supine position, roll over a bed,distal muscle weakness
in the form of unable to pick objects tear rotis and
esophageal phase of dysphagia since 8 days.Not associated
with sensory loss or bowel and bladder involvement.

13. GENERAL EXAMINATION
• Thin body built.
• Length = 160 cm,
• No pallor, icterus, cyanosis, clubbing, pedal edema or enlarged lymph
nodes.
• JVP is not raised.
• No thyromegaly
• Skin thickened over arm and forearm,palm associated with
desquamation
• Frontal recession of hair line
• Neurocutaneous marker –Absent
• Blackish discolouration of face,chest,shoulder area,pulp of toes

14. Pulse: 78/min, regular, normal in volume & character, no brachio-
femoral delay, arterial wall just palpable, all peripheral pulses are
well felt.
Blood Pressure: 110/70 mm of Hg Right arm supine position.
Respiratory rate: 16 /min, regular, abdomino thoracic.
Temperature - 98.8° F.

15. EXAMINATION OF NERVOUS SYSTEM
HIGHER FUNCTION:
• Conscious, Oriented to time, place and person.
• Speech is normal.
• Normal memory and intelligence.
• No delusion, hallucination.

16. EXAMINATION OF THE CRANIAL NERVES
Olfactory nerve :CN1
• No Anosmia
• No Parosmia.

17.  Optic Nerve:CN2
• FUNDOSCOPY: NORMAL
RIGHT LEFT
Visual acuity Normal Normal
Color Vision Normal Normal
Field of Vision Normal Normal

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

19. Trigeminal Nerve: CN5
• Sensations over face normal.
• Corneal reflex present on both side
• No weakness of muscles of mastication.
• Jaw jerk absent.
 Facial nerve :CN7
• No weakness of facial muscles.
• Taste sensation over anterior 2/3rd
of tongue intact.

20.  Vestibulocochlear Nerve :CN8
• Rinnes test: positive.
• Weber’s test not lateralized.
 Glossopharyngeal & Vagus Nerve: CN9&10
• whole of the oral cavity,palate,pharynx not visible due to
reduced opening of mouth
• Gag reflex elicited by touching the soft palate

21.  Accessory Nerve :CN11
• Definite weakness of sternocleidomastoid, trapezius.
 Hypoglossal Nerve :CN12
• No atrophy and fasciculation of tongue.
• No deviation of tongue to any side.

22. MOTOR SYSTEM EXAMINATION
Inspection: no atrophy , no fasciculations.
1.BULK:
2.TONE
Upper limb : hypotonia
Lower limb : hypotonia
RIGHT LEFT
ARM 23cm 23cm
FOREARM 21cm 21cm
THIGH 32cm 32cm
LEG 25cm 24cm

23. 3. POWER:
JOINT MOVEMENT RIGHT LEFT
Shoulder Abduction 1/5 1/5
Adduction 1/5 1/5
Flexion 1/5 1/5
Extension 1/5 1/5
Elbow Flexion 1/5 1/5
Extension 1/5 1/5
Wrist Flexion 2/5 2/5
Extension 2/5 2/5
Handgrip weak weak

24. JOINT MOVEMENT RIGHT LEFT
Hip Abduction 1/5 1/5
Adduction 1/5 1/5
Flexion 1/5 1/5
Extension 1/5 1/5
Knee Flexion 1/5 1/5
Extension 1/5 1/5
Ankle Dorsiflexion 2/5 2/5
Plantarflexion 2/5 2/5
Trunk muscle – weak
Neck muscles - weak

25. 4) REFLEXES
DEEP TENDON REFLEXES
Right Left
Upper
Limb
Biceps diminshed diminished
Triceps diminished diminished
Supinator diminished diminished
Lower Limb Knee diminished diminished
Ankle diminished diminished
Ankle and patellar clonus- Absent
Jaw Jerk- Absent

26. Superficial reflex right left
corneal present present
Abdominal reflex
Upper
Middle
Lower
Present
Present
present
Present
Present
present
plantar flexor flexor
Anal reflex present present
Cremasteric reflex present

27. SENSORY EXAMINATION
All primary modalities of sensation like
pain,temperature,light touch ,crude touch,vibration,Joint
position sense are normal in both upper limb and lower limb

28. •CORTICAL SENSATION : normal In both upper limb and lower limb
•Cerebellar Signs:
could not be elicitated
•GAIT: could not be asscced.
•AUTONOMIC NERVOUS SYSTEM: No resting tachycardia,
bradycardia,no abnormal sweating
•SKULL AND SPINE: no abnormality noted
•MENINGEAL SIGNS: absent
•PERIPHERAL NERVES : not palpable or tender

29. EXAMINATION OF CARDIOVASCULAR SYSTEM
INSPECTION :-
• Precordium normal in shape
• No dilated veins and visible scars seen
• Apical impulse not visible
• No other pulsation seen.
PALPATION:-
• Apical impulse -present in left 5th
ICS , ½ inch medial to Mid
clavicular line, normal in character.
• Pulmonary area : P2 not palpable.
• No thrill.

30. PERCUSSION:-
• 2nd
left intercostal space - resonant
• Cardiac dullness starts from left 3rd ICS & does not
extend beyond the apex.

31. AUSCULTATION:-
1) MITRAL AREA:
• 1st
heart sound normal
• No added sound.
• No murmur
2) PULMONARY AREA :
• Pulmonary Component of 2nd
Heart Sound Normal
• No Split
• No added sound

32. 3) AORTIC AREA :
Aortic component of second heart soundnormal
• No added sound
• No murmur
4) TRICUSPID AREA:
• First heart sound normal
• No murmur
5) NEOAORTIC AREA:
• No murmur

33. EXAMINATION OF RESPIRATORY SYSTEM
UPPER RESPIRATORY TRACT-normal
EXAMINATION OF CHEST:
INSPECTION :-
• Trachea appears to be central in position
• Apical impulse not visible
• Chest bilaterally symmetrical
• Bilateral Chest movement equal
• No paradoxical chest movement.

34. PALPATION:-
• Trachea is confirmed to be central
• Apical impulse is present in left 5th
ICS ½ inch medial to
MCL
• Chest expansion is 5 cm
• Vocal fremitus is normal
• There is no Intercostal tenderness

35. PERCUSSION :-
• Direct percussion over clavicle is normal bilaterally
• Percussion over left and right hemithorax is normally
resonant
AUSCULTATION :-
• Normal bilateral vesicular breath sound heard,no added
sounds

36. EXAMINATION OF GI SYSTEM
Mouth and oral cavity not fully visible due to reduced mouth
opening.
INSPECTION:-
•Shape of abdomen is normal
•Umbilicus central & inverted
•No engorged vein, no visible peristalsis
PALPATION:-
•Liver not enlarged, Spleen not palpable
PERCUSSION:-
•Abdomen is tympanitic.
• no shifting dullness
AUSCULTATION:-
•Bowel sound 3-4/min
•DRE - normal

37. SUMMARY
 A 40 Years old male presented with insidious onset gradual
progressive weakness of both upper and lower limb in the
form of proximal muscle involvement dificulty in getting up
from sitting position, unable to perform activity that are
required above head motion like bathing or combing hairs
over a period of 28 days with truncal and neck muscle
involevement and dysphagia as evidence by difficulty in
getting up from supine position, roll over a bed,raising head
from lying down position ,and esophageal phase of
dysphagia since 8 days . No associated sensory loss or bowel
and bladder involvement.
On examination: there is weakness of proximal muscle of
both the lower and upper limb ,trunk muscle,distal muscle.
 involvement of peripheral nerve – hypoactive knee jerk and
absent ankle jerk.

38. STRUCTURES INVOLVED FUNCTIONS LOST
skeletal muscle:
(Proximal muscle of upper limb and
lower limb ,trunk muscle ,back muscle
Esophageal muscle)
Weakness of both upper limb
And lower limb
Difficulty in getting up from sitting
position and rolling over.
Esophageal phase of deglutination
Peripheral nerve absent ankle jerk and hypoactive knee
jerk

39. PROVISIONAL DIAGNOSIS
This is a case of insidious onset gradual progressive
symmetrical quadirparesis of flaccid type involving
both upper limb and lower limb
(Proximal >distal) with involvement of truncal and
neck muscles
Etiology: inflammatory myopathy
(dermatomyositis)

40. DIFFERENTIAL DIAGNOSIS
•CIDP
•Motor neuron disease
•Mystehenia gravis

41. Differntial diagnosis Points favors Points against
CIDP Insidious onset
Pure motor lmn
weakness
Hypoactive jerks
Proximal >distal
Symmetrical pattern of
weakness
Presence of jerks+
MND pure motor lmn
weakness
Hypoactive jerks
Absence of fasciculation
Weakness>atrophy
Symmetrical pattern
MYSTHENIA GRAVIS proximal muscle
involvement
dysphagia
No diurnal variation
No extra ocular
involvement
No periodicity

42. INVESTIGATIONS
 Hemoglobin: 9.8 gm%
 TLC: 7300/ cmm
 Neutrophil: 75.4%
 Lymphocyte: 16.7%
 Eosinophil: 2.7%
 Monocyte 5.1%
 TPC 2.58lakh / cmm
 ESR: 106 mm in 1st
hr
 Urine (Routine/Microscopy)
Albumin: +
Sugar: Nil
RBC: Nil
Pus Cell: 0-2/HPF
Epithelial Cell: 1-2/HPF

43. INVESTIGATIONS
Serum Urea: 67mg/dL
Serum Creatinine: 0.8mg/dL
Serum Sodium: 138meq/l
Serum Potassium: 4.7 meq/l
HBsAg: Negative
HCV : Negative
HIV: Negative
Calcium(ionised)-1.2
Creatine phospho kinase-18500u/l
Liver function test:
Sr Bilirubin total-
0.3mg/dl
Sr Bilirubin direct-
0.1mg/dl
AST-986IU/L
ALT-289 IU/L
ALP-226 IU/L
Serum Protein-
5.7mg/dl
Serum Albumin-
3.3mg/dl
CRP(Q)-10.6

44. ANA HEP 2: 3+ Homogenous pattern in 1:100 dilution
Sr. cortisol : 8 am 15.19u/dl
TSH :2.16, fT3 :4.22, fT4 :14.48

45.  USG abdomen/pelvis-b/l acute medical renal disease,cholelithiasis with
cholecystitis
 XRAY CHEST: normal
MRI cervical spine- diffuse disc bulge at C5/C6 ,C6/C7 level indenting ventral thecal
sac and mild narrowing of spinal canal with bulging this abutting left C6/C7
level exiting nerve roots without any significant compression
MRI brain- few small T2 flair hyperintense lesion noted in b/l frontal ,lt temporo-
parietal,subcortical white matter(?demyelination ?small vessel ischemic
changes)

46. EMG STUDY:
Short duration ,small
amplitude normal
morphology with polyphasic
MUAP with complete
recruitment seen in all
tested muscle s/o-
myopathic pattern

53. MUSCLE BIOPSY THIGH:
Specimen taken from right vastus lateralis for study, reports awaited

54. FINAL DIAGNOSIS
INSIDIOUS ONSET GRADUAL
PROGRESSIVE FLACID TYPE of
QUADRIPARESIS EQUALY INVOLVING ALL
LIMBS ,(PROXIMAL> DISTAL) ,WITH
INVOLVEMENT OF NECK AND TRUNK
MUSCLE DUE TO INFLAMMATORY
MYOPATHY (?DERMATOMYOSITIS)

55. THANK YOU