SSPE, myasthenia n LETM
Transcript of SSPE, myasthenia n LETM
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CLINICAL CASE DISCUSSION
UNIT 6
GUIDES: Dr.A.Pauranik
Dr.P.Shivde
Dr.A.Verma
Candidate: Dr.Amith R
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Case 1:Purpose of presentation.
Rarity of the case Peculiar clinical picture.
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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.
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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
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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
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Sensory system : Within normal limits Cerebellar signs – absent. Gait – normal except for pauses during the
episodes of jerks.
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MRI
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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.
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EEG
Stereotyped periodic complexes with normal backgroundStrongly suggestive of SSPE.
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CSF Analysis
Routine: Glucose-64 mg/dl
Protein- 68 mg/dl
Cells- 2/cu.mm Measles IgG antibodies by EIA
Serum IgG 3764 U/ml
CSF IgG 16205 U/ml
CSF/Serum Quotient 2.62 (N- <1.3
E- 1.3-1.5
P- >1.5)
CLINICALCHEMISTRY, Vol.37, No.7, 1991
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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.
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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
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What’s atypical in our patient?
Age of onset. Absence of other symptoms. MRI abnormality.
Neurology India Sept-Oct 2012/Vol 60/Issue 5Am J Neuroradiol 17:761–772, April 1996
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Treatment
No curative therapy. Disease modifying agents
- Isoprinosine
- Levamisole
- Amantidine
- Interferan alpha
- IVIg
Postgrad Med J 2002. 7863–70.70.
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Case 2Purpose 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
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Diagnostic tests
Edrophonium (Prostigmin)test. Anti- AchR antibodies. Electrodiagnostic testing. Ocular cooling.
Bradley's Neurology in Clinical Practice- 6th Ed
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Prostigmin test
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Anti Ach receptor antibodies – Positive
0.65 nmol/L(< 0.25nmol/L) Thyroid functions are normal
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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
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Treatment
Pyridostigmine Prednisolone. Azathioprine. Thymectomy…
Bradley's Neurology in Clinical Practice- 6th Ed
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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 EdNeurology India Jul-Aug 2009/Vol 57/Issue 4
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Case 3Purpose 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.
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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
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Examination
Vitals : normal Nervous System:
- Conscious oriented to TPP.
- Higher mental functions : normal
- Findings are confined to motor & sensory
system.
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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
Involuntarymovements
Absent Absent Clonus Clonus
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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.
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Diagnosis
Anatomical:
Myelopathy
Lower Dorsal (D11- 12)
Pyramidal, Posterior column, Spinothalamic
± Autonomic involvement
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Diagnosis
Pathological:
- Inflammation = Transverse myelitis.
- Nutritional = Vit B12 deficiency
- Ischemic = Spinal cord infarction.
- Dural AVM
- Tumour & other causes of compression
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Investigations
X RAY L-S SPINE
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MRI SPINE
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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.
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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)
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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)
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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)
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In our patient
Optic neuritis - Visual evoked potential is normal
Myelitis - Satisfies the criteria. Anti NMO antibody - negative
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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
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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
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MRI Brain : No abnormality detected
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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
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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
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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
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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
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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)
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In our patient
Serum ACE – 26mcg/L (N < 40mcg/L)
CRP – normal. S. Calcium – 9.7
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Other investigations
Serum B12 – 931.7 (211-911). USG abdomen & pelvis:
enlarged prostate. LFT : Normal. TSH: 1.56
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CAUSES OF LETM
Inflammatory
Neuromyelitis Optica
Multiple Sclerosis
SLE
Behcet’s syndrome
Sarcoidosis
Sjogren’s syndrome
Infectious
HIV
HTLV I & II
Neurosyphilis
CMV
Herpes simplex
Varicella zoster
European Neurological Journal 2011; 3:(1)
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Vascular
Dural AVM
Spinal cord infarction
Intramedullary spinal neoplasms
Ependymoma
Astrocytoma
Radiation myelitis
European Neurological Journal 2011; 3:(1)
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