2. Case 1:
Purpose of presentation.
Rarity of the case
Peculiar clinical picture.
3. Case Scenario
24 year male, laundry business.
Involuntary jerky movements of extremities and
trunk - 8 months.
Difficulty while walking.
Interruption while speaking.
Involuntary movements are not suppressible voluntarily & are
absent during sleep
No stiffness of limbs, weakness or sensory symptoms.
No c/o forgetfulness, abnormal behaviour.
No visual disturbances, dysphagia.
No bowel, bladder incontinence.
4. Examination
General examination : normal
Nervous system :
Conscious oriented to TPP.
Higher mental functions: memory, intelligence is
normal
Speech: fluency is affected by pauses during the
jerks
Cranial Nerves: Normal
5. Contd..
Motor system:
Nutrition – normal
Tone – Normal
Power – Normal.
Involuntary movements:
- present at rest
- involves limb and axial musculature,
- abrupt , brief & arrhythmic
- repetitive with frequency of 15-16/min,
- large amplitude with dystonic posturing of
upper limbs
- not stimulus sensitive
6. Sensory system : Within normal limits
Cerebellar signs – absent.
Gait – normal except for pauses during the episodes of
jerks.
9. Bilateral & near symmetrical T2 hyperintense signal in posterior putamen,
Slightly larger on the right. Both reveal restriction of diffusion & are hypo on T1.
No cortical or white matter changes seen.
12. Subacute Sclerosing Pan Encephalitis
(SSPE)
Fatal neurodegenerative disorder- sequel to early
childhood measles.
Prevalence in India is 6-8/million population.
Virus remains dormant intracellularly & manifests as
SSPE a decade later.
Avg interval between measles & SSPE is 8-11 yrs.
Increasing age of onset, more aggressive is the course.
13. Diagnostic Criteria
Definitive: 3 out of 5 should be positive
Clinically progressive, subacute cognitive deterioration
or typical signs like myoclonus.
EEG -Periodic, stereotyped, high voltage discharges.
Cerebrospinal fluid raised gammaglobulin or
oligoclonal pattern
Measles antibodies raised titre in serum and/or
cerebrospinal fluid.
Brain biopsy suggestive of panencephalitis
. Subacute sclerosing panencephalitis. Neurol Clinical (31) 79–95
14. What’s atypical in our patient?
Age of onset.
Absence of other symptoms.
MRI abnormality.
Neurology India Sept-Oct 2012/Vol 60/Issue 5
Am J Neuroradiol 17:761–772, April 1996
16. Case 2
Purpose of presentation
Management issues in a common disease
18 yr old female student.
3 months h/o drooping of eyelid, difficulty in chewing,
swallowing & change of voice associated with
generalised weakness.
Symptoms worsen as the day progress and improve on
resting.
No limb weakness or sensory symptoms.
Examination reveals ptosis on Lt side, reduced
elevation of soft palate b’l with preserved gag reflex.
Fatigability of limbs on sustained contraction
19. Anti Ach receptor antibodies –
Positive
0.65 nmol/L(< 0.25nmol/L)
Thyroid functions are normal
20. Myasthenia Gravis
Incidence of 3-5/1,00,000 in India.
More common in females but in India
M>F.
Females in 1-2 decade but in males
in 5-6 decade.
Neurology India Jul-Aug 2009/Vol 57/Issue 4
23. Thymectomy in myasthenia
Absolutely indicated in cases of thymoma
regardless of age.
Nonthymomatous generalised MG : Early
onset Anti Ach receptor positive cases.
Less effective in seronegative Anti Musk
Ab positive patients.
Preferred approach is transthoracic
sternal splitting procedure
Bradley's Neurology in Clinical Practice- 6th Ed
Neurology India Jul-Aug 2009/Vol 57/Issue 4
24. Case 3
Purpose of presentation
Clinical syndrome of transverse myelitis is
common.
This can occur due to LETM.(post MRI era)
List of causes for LETM is long and
requires exhaustive workup.
Even after exhaustive workup cause may
not be known.
25. Case Scenario
55 yr old male . Building contractor
Tingling & numbness of both lower limbs 8 months
Progressive weakness of both lower limbs (L>R).
Hesitancy & dribbling micturition,
No retention or incontinence.
Involuntary movements of both lower limbs (L>R).
Band like sensation around the waist,
No backache or weakness of upper limbs.
No h/o diminution of vision, breathing difficulty
26. Examination
Vitals : normal
Nervous System:
- Conscious oriented to TPP.
- Higher mental functions : normal
- Findings are confined to motor & sensory
system.
27. contd….
Motor system examination:
Beevors sign: positive
Neck muscles: power 5/5
RUL LUL RLL LLL
Nutrition N N N N
Tone N N ↑↑ ↑↑
Power 5/5 5/5 4-/5 3/5
Coordination Normal Normal Impaired Impaired
Involuntary
movements
Absent Absent Clonus Clonus
28. Reflexes : Upper limb DTR were brisk,
Hoffman's & Wartenberg negative
Lower limb reflexes exaggerated with
ankle & patellar clonus.
Abdominal and cremastric reflexes absent.
Plantars are extensor bilaterally.
No cerebellar signs.
Romberg's: positive.
Sensory system examination:
Pain, temperature, vibration & joint position
sense absent till level of ASIS.
36. MRI Spine
Evidence of a long segment intramedullary T2
hyperintense signal seen in dorsal cord
extending from D7 upto D11/12 segments. Both
half of cord appear involved and involved cord
appears edematous.
The possibility of inflammatory demyelinating
disease like LETM is more likely.
37. Longitudinally Extensive Transverse
Myelitis(LETM)
Spinal cord inflammation extending 3 or
more vertebral segments in length.
Can involve entire length of spinal cord,
are much rarer.
Associated with greater morbidity than
typical transverse myelitis.
European Neurological Journal 2011; 3:(1)
38. Neuromyelitis Optica(Devic’s disease)
Well recognised cause of LETM.
Demyelination of spinal cord and optic nerve.
Can have monophasic or relapsing course
Diagnosis is by clinical, radiological & serological
criteria.
Poorer prognosis than MS
European Neurological Journal 2011; 3:(1)
39. Diagnostic Criteria for NMO
1: Optic Neuritis
AND
2: Myelitis
AND
3: Atleast two of the following
(a) Contiguous spinal cord MRI lesion extending 3 or
more segments
(b) Brain MRI not meeting criteria for MS
(c) NMO IgG seropositive status
European Neurological Journal 2011; 3:(1)
40. In our patient
Optic neuritis - Visual evoked potential is
normal
Myelitis - Satisfies the criteria.
Anti NMO antibody - negative
41. Multiple Sclerosis
Commonly associated with partial
transverse myelitis spanning 2 or less
spinal segments.
LETM common in Indians with the optico-
spinal variant.
Have coexistent brain lesions.
Bradley's Neurology in Clinical Practice- 6th Ed
42. In our patient
CSF Analysis:
- Opening pressure: 10 cm of H2O.
- Pandy’s : Negative.
- Glucose : 63 mg%(40-70)
- Protein : 101 mg%(15-40)
- Total cells: 2 (100% lymphocytes)
- Oligoclonal bands: No band seen.
- ADA : 5
44. Systemic Lupus
Erythematosis(SLE)
Most severe and rare complication of SLE
seen in 1-3% patients.
Majority occurs shortly after SLE is
diagnosed
Recurrence is frequent
Preceded usually by non specific
prodromal symptoms
Myelitis in the course of SLE- POLSKIE ARCHIWUM 2009,119
45. Diagnostic Criteria for
myelitis in SLE by ACR
Rapid onset of one or more of the following
Bilateral weakness of legs with or without arms, may be asymmetrical
Sensory impairment with cord level similar to that of motor weakness
www.rheumatology.org/publications/1999/499ap10
Exclusion Criteria
Mass lesion causing compression of or within spinal cord
Cauda equina lesion
46. In our patient
CBC : Normal
Urinanalysis : Normal
ESR : 10 mm/hr.
CRP : 0.6 (N-0.6)
ANA -0.649 (positive >1.4)
Anti dsDNA – Negative
aPTT – 35 sec
47. HIV
Myelopathy seen in 5-10%
Most common being vacuolar myelopathy.
Seen mostly in advanced stages
Rare after introduction of cART.
In our patient- HIV 1 & 2 both were
negative.
VDRL -Nonreactive
Intern Med 50: 1615-1617, 2011
48. Sarcoidosis
Mainly subacute or chronic in
presentation.
Most common in males in their early 40s
Lesions most often located in cervical or
upper thoracic region.
Associated commonly with respiratory
symptoms.
European Neurological Journal 2011; 3:(1)
