PG case presentation of paraplegia with Q&As.CNS - Spinal cord.

1. CNS CASE PRESENTATION

2. • NAME- X
• AGE- 30 YRS
• SEX- Female
• ADDRESS- Sholingar, Tiruttani.
• HANDEDNESS- Right handed.
• OCCUPATION- Unemployed.
• EDUCATION- 5th class.

3. • PRESENTING COMPLAINTS- Weakness of all 4
limbs for the past 17 years which is
progressive.
• HISTORY OF PRESENTING ILLNESS- Patient was
apparently normal before 13 yrs of age when
she had a slip and fall around 11 AM, with no
h/o head injury, headache, ENT bleed. On the
same day evening patient had an episode of
seizure involving all 4 limbs with tongue bite,
LOC and postictal confusion taken to hospital,
injections were given and discharged.

4. • No further episode of seizure.
• 1 month after the seizure patient noticed
weakness of the right lower limb in the form
of difficulty in gripping chappals, and slipping
of chappals knowingly, difficulty in brisk
walking, difficulty in clearing the ground,
frequent tripping and fall which is of insidious
onset and gradually progressive (chronic) over
the past 17 yrs. there is no difficulty in getting
up from sitting position.

5. MONOPARESIS- Causes
Check for generalised / isolated group of
muscles
Check for atrophy
Absence- cerebral cortex, vascular(thrombotic
or embolic)
Presence- Brachial- birth trauma, syringomyelia
ALS : Crural(leg)-trauma, tumor, myelitis,MS,
progressive muscular atrophy.

6. • 15 days later patient noticed weakness of right
upper limb in the form of difficulty in mixing
food, difficulty in hooking and unhooking clothes
and difficulty in gripping objects, insidious onset
and progressive, there was no difficulty in lifting
the arm above shoulder, no weakness of left
upper limb and lower limb.
• 30 days later patient noticed weakness of left
lower limb and upper limb simultaneously in the
form of difficulty in holding objects, hooking,
unhooking and gripping chappals weakness is
insidious and progressive till now.
• At the same time patient noticed thinning of
hands and calf and feet insidious and progressive.

7. Onset of weakness
• Sudden: stroke, toxins, trauma
• Progressive over days: infection,
inflammation, demyelination, metabolic
• Progressive over weeks: tumors
• Progressive over months to years:
degenerative – MND, muscular dystrophy

8. Symmetry of weakness
• Symmetric: intramedullary lesions, transverse
myelitis, vascular, hereditary, metabolic,
degenerative
• Asymmetric: extramedullary lesions,
mononeuritis, plexopathies,

9. Proximal vs distal
• Proximal: myopathy, muscular dystrophy,
myasthenia, myositis
• Distal: peripheral neuropathy, distal muscular
dystrophies, inclusion body myositis

10. What is Elsberg Phenomenon?
• In cervical cord lesions, one arm becomes
weak followed by ipsilateral leg, then
contralateral leg and finally contralateral arm
in U shape
• Seen in extra dural spinal cord tumors

11. • There is no difficulty in getting up from chair,
lifting arms above shoulders. No difficulty in
getting up from lying position and rolling
sideways and lifting the head from bed. There is
no diurnal variation.
• No h/o muscle twitching.
• No h/o stiffness, limbs are floppy.
• Patient is currently able to walk with out support.
Gives h/o swaying backwards on standing without
support. no h/o clumsiness of movements, no
h/o swaying while walking
• H/o involuntary movements of hands(tremors) on
gripping objects tightly, not present at rest or
during motion for the past 6 months.

12. Symptoms of NMJ disease?
• Girdle weakness without sensory changes is
indicative of myopathy & distal weakness is
indicative of neuropathy.
Fluctuating weakness & fatiguability
Weakness made worse by activity.
Special vulnerability of certain muscles(eye
lids, muscles of eye, face)

13. Causes of Swaying?
 Cerebellar ataxia
 Posterior column disease(sensory ataxia)
 Vertiginous ataxia
 Thalamic ataxia
Proximal muscle weakness

14. Tremors- types ?
• Physiologic- 8-13 Hz
• Parkinson –rest, 3-5Hz
• Cerebellar- intention, 2-4 Hz
• Essential- familial, 4-8Hz
• Postural – action, 5-8Hz
• Orthostatic – irregular, 4-8Hz
• Dystonic-irregular

15. • At the same time patient noticed worsening of
difficulty in walking with out light at night or
in a dark room and through narrow passage
ways and swaying on washing face, has to lean
over support to wash the face. No h/o
sensation of walking over cotton wool.
• Patient is able to fell clothes, differentiate
between hot and cold water, no h/o
numbness, no h/o altered pain perception, no
dysesthesias. c/o generalized myalgia on
exertion.

16. Exertional Pain- causes?
• Glycogen storage disorders
• Inflammatory myopathies
• Infectious myositis
• Drugs

17. • Patient is able to perceive smell, no h/o
blurring of vision, able to identify red and
green colors, no diplopia, no numbness over
the face able to differentiate hot and cold, no
difficulty in chewing food, no difficulty in
closing eyes, no dribbling of saliva, able to
blow the cheeks, no decreased or altered
sensation of taste, no h/o of hearing difficulty
and reeling sensation in ears, no giddiness, no
change of voice, no h/o nasal regurgitation,
able to shrug shoulders, able to turn head
sideways, able to mix food well with tongue.

18. • Patient is able to feel bladder fullness, able to
hold, initiate and control voiding, no h/o
urgency, hesitancy or incontinence. No h/o
constipation or diarrhea.
• No h/o giddiness on getting up and altered
sweating.
• No h/o delusions, illusions or hallucinations.
• No h/o memory disturbances.
• No h/o toxin exposure, industrial exposure,
poison consumption, hospital admission
medication intake before the symptom onset.

19. Symptoms sugg. Of spinal cord?
• Can present as central cord, hemi cord
posterior cord, anterior cord or tranverse
myelitis
• Definite motor, sensory level
• Bladder and bowel involvement.

20. • Past h/o – h/o 1 episode of seizure at 13 years
of age. Not a k/c/o Diabetes, tuberculosis,
PTB, bronchial asthma.
• Treatment h/o- On vitamin tablets.
• Perinatal and developmental h/o- FTNVD,
cried immediately after birth, achieved mile
stones normally, discontinued education due
to poor scholastic performance.
• Surgical h/o- no h/o previous surgeries.
• Personal h/o- takes mixed diet, bowel and
bladder habits normal no substance abuse.

21. Nutritional causes
• Thiamine (B1)
• Niacin (B3)
• Pyridoxine (B6)- deficiency and excess
• Pantothenic acid
• Folate and B12
• Vitamin E

22. Seizures in quadriparesis?
• Cerebral palsy
• Paraneoplastic
• Phakomatoses

23. What is cerebal palsy?
• Non progressive neurological deficit since
birth
• Congenital abnormality of motor function
• H/O insult in prenatal, perinatal, postnatal
period
• Marked prematurity
• Many but not all have epilepsy in addition
• Germinal matrix(sub ependymal) hemorrhage,
peri ventricular leukomalacia, HIE, kernicterus

24. • Family h/o- No h/o similar complaint in the
family, 5th child in the family, had 2 elder
brothers and 2 elder and I younger sister all
are healthy.

25. • STRUCTURES INVOLVED- PERIPHERAL NERVE –
MOTOR FIBRE AND LONG FIBRE INVOLMENT.

26. • History summary- A 30 year old female with
no co-morbidities with h/o of weakness of all
4 limbs, distal weakness, chronic onset,
progressive for the past 17 years, asymmetric
at onset, currently symmetric of LMN type,
with no UMN features, with long fiber
symptoms, with no symptoms of small fiber
involvement, no cranial nerve involvement
and no ANS involvement and cerebellar
involvement.

27. • Systems involved- motor and sensory.
• Distribution- distal weakness, asymmetric at
onset, symmetric now.
• Nature of sensory involvement- long fiber.
• No evidence of UMN involvement.
• Temporal presentation- chronic and
progressive.
• Evidence of hereditary neuropathy- lacking.
• Associated medical conditions- nil.

28. • DIFFERENTIAL DIAGNOSIS-
PERIPHERAL NEUROPATHY
- HEREDITARY SENSORY MOTOR
NEUROPATHY.
- CHRONIC INFLAMMATORY DEMYELINATING
POLYNEUROPATHY.
- NURTITIONL.

29. Acute Vs Insidious Weakness
Acute – GBS, porphyria, Toxins, diptheria
Sub acute – nutritional, alcoholic, toxins,
angiopathic
Chronic – diabetes, CIDP, paraneoplastic,
paraprotein
Long standing Heritable- CMT, Friedreich’s
ataxia
Recurrent- CIDP, porphyria, Refsum’s ,HNPP

30. Symmetric Vs Asymmetric
Weakness
• Symmetric – acquired- demyelinating :
hereditary
• Asymmetric – radiculopathies, plexopathies,
compressive mononeuropathy, multiple
mononeuropathy.

31. Proximal Vs Distal Weakness?
• Proximal and Distal- acquired demyelinating
• Distal- nutritional, metabolic, toxin
• Proximal- polymyositis,porphyria

32. GENERAL EXAMINATION
• Conscious, oriented to time, place and person,
afebrile.
• BMI- 20.9
• No pallor, icterus, cyanosis, clubbing,
lymphadenopathy and pedal edema.
• No peripheral nerve thickening.
• No neurocutaneous markers.
• Pescavus present in both feet.
• Lower limbs are in inverted champagne bottle
appearance.

33. Skin/mucosa markers in PN?
• Angiokeratoma- Fabry disease
• Photosensitive rash- Porphyria
• Orange tonsils- Tangier disease
• Icthyosis- Refsum disease
• Mask facies- scleroderma
• Leonine facies- leprosy

34. Pes cavus- significance?
• In early life,feet are pulled into talipes equinus
(plantar deviation) due to disproportionate
weakness of pretibial/peroneal muscles &
unopposed action of calf muscles.
• During life,extensors of toes dorsiflex PP &
flexors shorten the foot,heighten the arch,flex
the DP leading to claw foot/pes cavus.

35. • PR- 74/Min, regular, normal volume and
character.
• BP- 120/80 mm Hg in right UL in supine
position.
- 110/80 mm Hg in right UL 3 min after
standing.
• RR- 14/min, thoracoabdominal.
• Temp- 98.4 f.

36. ANS involvement in PN?
• Diabetes mellitus
• Amyloidosis
• Porphyria
• Congenital
• GBS
NOT seen in Radicular diseases.

37. CNS EXAMINATION
• HIGHER MENTAL FUNCTIONS
• MMS- 24/30.
• SPEECH- comprehension, fluency, word
output, naming, repetition- normal.
patient doesn’t know how to read and write.
• Memory- immediate, short term, long term
memory is normal.
• Reasoning, abstract thinking- normal.

38. CRANIAL NERVES
• I CN- able to appreciate smell.
• II CN- visual acuity- 6/6.
- color vision- normal.
- confrontation testing- no field defect.
- fundus- normal.
• III, IV, VI CN- no ptosis, PERL, light reflex(direct
and indirect)-present, accomodation- present,
extraocular movements - full, saccades, pursuits-
normal. Conjugated eye movements- normal.
• V CN- sensory- normal.
- muscles of mastication – normal.

39. • VII- taste ant 2/3- normal.
- able to frown, close eyes tightly, no deviation
of angle of mouth, able to blow air.
• VIII CN- Rinne’s test- AC>BC
Weber’s test- no lateralization.
• IX & X CN- uvula in midline, palatal movements-
present. Gag reflex- present bilaterally.
• XI CN- able to shrug shoulders, able to turn head
side ways against resistance.
• XII CN- no wasting, no fasciculations, no deviation
on protruding out.

40. CN involvement in PN ?
Olfactory- Refsum disease
Optic- B12 deficiency, Refsum disease
3,4 ,6th –diabetes,
Facial- GBS, leprosy
9,10th- GBS

41. • MOTOR SYSTEM
• APPEARANCE- wasting of forearm and thenar and
hypothenar muscles of the hand with guttering of
dorsum of hand., inverted champagne bottle
appearance of lower limbs, foot drop present in both
feet, pescavus guttering of dorsum of both feet
present. No fasciculation, no muscle tenderness.
• BULK- RIGHT LEFT
mid arm 23cms 23cms
mid fore arm 17cms 17cms
mid thigh 41cms 41cms
mid calf 22cms 22cms

42. Denervation Vs Disuse atrophy?
• Denervation- reduces muscle volume by 75 to
80 %
• Disuse – does not reduce muscle volume by
more than 25 to 30 %

43. • TONE
RIGHT LEFT
Shoulder normal normal
Elbow hypotonia hypotonia
Wrist hypotonia hypotonia
Hip normal normal
Knee hypotonia hypotonia
Ankle hypotonia hypotonia

44. • POWER-
RIGHT LEFT
Shoulder(flexion, extension 5/5 5/5
adduction, abduction, IR,ER)
Elbow (flexion, extension) 4+/5 4+/5
Wrist(flexion, extension) 4/5 4/5
Hand muscles- thumb- adduction, abduction,
opposition, flexion, extension- weak
- FDP- weak
- Flexor sublimus – weak
- lumbricals- weak
- palmar & dorsal interossi- weak

45. RIGHT LEFT
HIP (flexion, extension 5/5 5/5
adduction, abduction, IR,ER)
Knee (flexion, extension) 4/5 4/5
Ankle(plantar flexion, 1/5 1/5
dorsiflexion, inversion, eversion)
EHL 1/5 1/5

46. • REFLEXES
• DEEP TENDON REFLEXES-
RIGHT LEFT
Biceps - -
Triceps - -
Supinator - -
Knee jerk - -
Ankle jerk - -

47. Areflexia/proprioception
discordance
• Posterior column disease

48. • SUPERFICIAL REFLEXES-
RIGHT LEFT
Corneal present present
Conjunctival present present
Gag reflex present present
Abdominal(upper,lower) absent absent
Anal reflex present
Plantar mute mute

49. UMN involvement with PN ?
• B12 deficiency
• Copper deficiency
• Adrenomyeloneuropathy(AMN)
• HIV infection
• Severe hepatic disease

50. • Primitive reflexes- absent.
• Finger flexion jerk absent absent
• Hoffman’s sign absent absent
• Wartenberg’s sign absent absent

51. • SENSORY SYSTEM
- pain, temperature normal
- touch, pressure normal
- vibration- duration of appreciation reduced in all the areas
when compared to examiner.
- joint position sense- UL- normal
LL- normal.
Romberg’s sign- positive.
- cortical sensory-
tactile localization, two point discrimination, stereognosis,
graphesthesias- normal.

52. • Autonomic nervous system
Postural drop in BP- absent.
no features of horners syndrome.
Carotid sinus reflex- absent.

53. • CEREBELLAR SYSTEM- Speech- normal. No
nystagmus.
RIGHT LEFT
Finger-nose, finger-finger normal normal
Writing name normal normal
Dysdiadokinesia normal normal
Knee-heel normal normal
Rebound phenomenon- absent absent
Tandem walking – not able to perform.

54. • Gait- high stepping gait.
• SPINE AND CRANIUM-
Movements of spine (flexion, extension,
rolling over bed)- normal.
Vibration over spine- reduced compared to
examiner.
No tenderness over spine.
• No muscle tenderness.
• No signs of meningeal irritation.

55. What is stamping gait ?
• Caused by impairment of joint position/
muscular kinesthetic sense
• Seen in lesions of peripheral nerves, posterior
column of cord or medial lemnisci
• Recognised by stamp and stick
• Brusqueness of movement of legs and
stamping of feet as the foot is forcibly brought
down onto the floor.

56. • CVS- S1+ S2+
• RS- NVBS, clear.
• P/A- soft, no organomegaly, BS +.

57. • DIAGNOSIS-
PERIPHERAL NEUROPATHY
- HEREDITARY SENSORY MOTOR NEUROPATHY.
- CHRONIC INFLAMMATORY DEMYELINATING
POLYNEUROPATHY.

58. NCS- uses in PN ?
• To diffentiate between axonal and
demyelinating neuropathies.
• Demyelinating: GBS, DM, CIDP, diphtheria,
MMN
• Axonal: toxic, metabolic, HIV, DM