A Good CNS-Spinalcord presentation by PG SRMC


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

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A Good CNS-Spinalcord presentation by PG SRMC

  2. 2. • NAME- X • AGE- 30 YRS • SEX- Female • ADDRESS- Sholingar, Tiruttani. • HANDEDNESS- Right handed. • OCCUPATION- Unemployed. • EDUCATION- 5th class.
  3. 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. 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. 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. 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. 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. 8. Symmetry of weakness • Symmetric: intramedullary lesions, transverse myelitis, vascular, hereditary, metabolic, degenerative • Asymmetric: extramedullary lesions, mononeuritis, plexopathies,
  9. 9. Proximal vs distal • Proximal: myopathy, muscular dystrophy, myasthenia, myositis • Distal: peripheral neuropathy, distal muscular dystrophies, inclusion body myositis
  10. 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. 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. 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. 13. Causes of Swaying?  Cerebellar ataxia  Posterior column disease(sensory ataxia)  Vertiginous ataxia  Thalamic ataxia Proximal muscle weakness
  14. 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. 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. 16. Exertional Pain- causes? • Glycogen storage disorders • Inflammatory myopathies • Infectious myositis • Drugs
  17. 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. 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. 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. 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. 21. Nutritional causes • Thiamine (B1) • Niacin (B3) • Pyridoxine (B6)- deficiency and excess • Pantothenic acid • Folate and B12 • Vitamin E
  22. 22. Seizures in quadriparesis? • Cerebral palsy • Paraneoplastic • Phakomatoses
  23. 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. 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.
  26. 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. 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.
  29. 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. 30. Symmetric Vs Asymmetric Weakness • Symmetric – acquired- demyelinating : hereditary • Asymmetric – radiculopathies, plexopathies, compressive mononeuropathy, multiple mononeuropathy.
  31. 31. Proximal Vs Distal Weakness? • Proximal and Distal- acquired demyelinating • Distal- nutritional, metabolic, toxin • Proximal- polymyositis,porphyria
  32. 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. 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. 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. 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. 36. ANS involvement in PN? • Diabetes mellitus • Amyloidosis • Porphyria • Congenital • GBS NOT seen in Radicular diseases.
  37. 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. 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. 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. 40. CN involvement in PN ? Olfactory- Refsum disease Optic- B12 deficiency, Refsum disease 3,4 ,6th –diabetes, Facial- GBS, leprosy 9,10th- GBS
  41. 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. 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. 43. • TONE RIGHT LEFT Shoulder normal normal Elbow hypotonia hypotonia Wrist hypotonia hypotonia Hip normal normal Knee hypotonia hypotonia Ankle hypotonia hypotonia
  44. 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. 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. 46. • REFLEXES • DEEP TENDON REFLEXES- RIGHT LEFT Biceps - - Triceps - - Supinator - - Knee jerk - - Ankle jerk - -
  47. 47. Areflexia/proprioception discordance • Posterior column disease
  48. 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. 49. UMN involvement with PN ? • B12 deficiency • Copper deficiency • Adrenomyeloneuropathy(AMN) • HIV infection • Severe hepatic disease
  50. 50. • Primitive reflexes- absent. • Finger flexion jerk absent absent • Hoffman’s sign absent absent • Wartenberg’s sign absent absent
  51. 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. 52. • Autonomic nervous system Postural drop in BP- absent. no features of horners syndrome. Carotid sinus reflex- absent.
  53. 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. 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. 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. 56. • CVS- S1+ S2+ • RS- NVBS, clear. • P/A- soft, no organomegaly, BS +.
  58. 58. NCS- uses in PN ? • To diffentiate between axonal and demyelinating neuropathies. • Demyelinating: GBS, DM, CIDP, diphtheria, MMN • Axonal: toxic, metabolic, HIV, DM