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'Approach to cns demyelinating disorders
Oligodendrocytes produce myelin sheaths around axons that insulate and speed up nerve conduction. Demyelinating disorders involve damage to existing myelin sheaths, while dysmyelinating disorders impair myelin formation. Common demyelinating conditions include multiple sclerosis, acute disseminated encephalomyelitis, and neuromyelitis optica spectrum disorder. Diagnostic tools include neurological exam, MRI, and lumbar puncture. Differential diagnosis requires considering alternative causes such as infection, autoimmune disease, or metabolic derangement.
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'Approach to cns demyelinating disorders
- 2. INTRODUCTION • Lipid containingmyelinsheath made by oligodendrocytes ( in spinal cord by schwann cell). • Covers the axon, working as a insulator and speed up the nerve conduction.
- 3. DEMYELINATING VS DYSMYELINATINGDISEASE DEMYELINATING (Myelin sheath was formed normally, then damaged ) Inflammatory Vascular Toxic/ Metabolic eg Binswanger disease eg. Alcohol, Hypertensive encephalopathy, Osmotic demyelinating syndrome, B12 deficiency (a) Idiopathic – Clinically isolated syndrome , Multiple sclerosis & its Varient’s, NMOSD, ADEM, (b) Infective – Progressive Multifocal Leucoencephalopathy, HIV, Lyme disease (c) granulomatous – Sarcoidosis, Wegener’s Granulomatosis (d) CTDs-SLE, Sjogrens syndrome, Behcets ds
- 4. • DYSMYELINATING DISORDERS: Involves inborn error in formation of myeline sheath • Examples: ▪ Metachromatic leukodystrophy ▪ Krabbe’s disease ▪ Adrenal leukodystrophy ▪ Zelwagar disease ▪ Canavan’disease ▪ Alexender’s disease
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- 6. DIAGNOSTIC TOOLS HISTORY Other Neurophysiologic studies: (visual,auditory and somatosensory ) Hematologic and biochemical analysis of peripheral blood (complete blood count, erythrocyte sedimentation rate (ESR) Coagulogram, C reactive protein, PROTIEN S Immunologic analysis (ANCA, ANA,C3, C4) Thyroid gland hormones; Vitamin B12 and folic acid; Serologic analysis for HIV, hepatitis B and C VDRL, TPHA MRI BRAIN/SPINE CSF EXAMINATION
- 7. Sign & symtompssuggestive of demyelination HISTORY EXAMINATION STEP 1 STEP 2 STEP 3
- 8. WHEN? VISUAL • Blurred vision •Unilateral loss of vision • Afferent pupillarydefect (APD) • Diplopia • Nystagmus • Internuclearophthalmoplegia (INO) MOTOR •Muscle weakness •Spasticity •Hyperreflexia •Gait disturbance •Imbalance problems SENSORY •Numbness •Paresthesias •Dysesthesias •Lhermitte’s sign •Trigeminal neuralgia •Allodynia •Proprioception deficits
- 9. Cerebellar: tremor ,ataxia , incoordination Genitourinary • Urgency/frequency/retention • Incontinence • Frequent UTI • Constipation • Impotence • Anorgasmia • Dyspareunia Neuropsychiatric •Cognitive impairment •Depression •Irritability •Anxiety Other symptoms •Prominent intractable fatigue with no other cause •Dizziness and vertigo
- 11. HISTORY • Age ,Gender • Family history • H/o vaccination • H/o recent infection • H/o alcoholand any other drug intake
- 12. EXAMINATION CNS Higher mental functions:encephalopathy – ADEM Cognitive impairment/ apraxia/ aphasia- vascular origin demyelinating ds Cranial nerves- cranial nerve 3/4/6- MS Sensory- with definitive level- TM Motor- hypertonia, hyperreflexia , babinski sign
- 13. Initial diagnostic workup(i.e MRI BRAIN & Spine, Infectious, Rheumatological workup If no red flag suggesting alternate (infectious,neoplastic,metabolic,genetic) diagnosis If red flag present Look for another causes MONOPHASIC RECURRENT ALTERED MENTAL STATUS YES YES NO NO MRI BRAIN & SPINE: LARGE CONFLUENT WHITW MATTER LESION MRI SPINE LESION OR OPTIC NERVE ALONE Mc DONALD CRITERIA ON MRI MRI BRAIN: HYPOTHALAMUS/ AREA POSTREMA LETM & AQP4Ab ADEM ON/TM/CIS MULTIPHASIC ADEM MS NMOSD STEP 4
- 14. CIS • The firstneurologic attack of an acute inflammatory demyelinating event in central nervous system, lasting at least 24hrs in absence of fever/infection (Polaman et al, 2011) • This definition also provides no DIS nor DIT by MRI brain and cervical cord.
- 16. Arch Neuropsychiatr 2015;52: (Supplement 1): S1-S11 • DOI: 10.5152/npa.2015.12608
- 17. Unilateral visual loss, Pain, Afferentpupil defect, Retrobulbar or mild disc swelling, Visual loss does not progress beyond two weeks Arch Neuropsychiatr 2015; 52: (Supplement 1): S1-S11 • DOI: 10.5152/npa.2015.12608
- 18. Unilateral visual loss, Pain, Afferentpupil defect, Retrobulbar or mild disc swelling, Visual loss does not progress beyond two weeks Bilateral visual loss No pain, Retinal exudates, Retinal hemorrhages, Severe disc swelling, No visual recovery after one month Arch Neuropsychiatr 2015; 52: (Supplement 1): S1-S11 • DOI: 10.5152/npa.2015.12608
- 19. Arch Neuropsychiatr 2015;52: (Supplement 1): S1-S11 • DOI: 10.5152/npa.2015.12608
- 20. Arch Neuropsychiatr 2015;52: (Supplement 1): S1-S11 • DOI: 10.5152/npa.2015.12608
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- 24. EPIDEMIOLOGY F:M RATIO AGE OFONSET PREVALENCE MS Moderate female predominance (2:1) Median of 29 years, uncommon in children and >50 years 5-10 Per 100000 NMOSD High female predominance (9:1– 3:1). Mean of 40–45 years, with wide distribution from young children to elderly 3.5 Per 100000 Ther Adv Neurol Disord. 2017 Jul; 10(7): 265–289. Published online 2017 May 24. doi: 10.1177/1756285617709723
- 25. CLINICAL FEATURE OPTIC NEURITIS Visualfield defect otherthan cecocentralscotoma (per se, complete blindness Severe visual loss (bilaterally ⩽0.1) in the chronic stage MS Uncommon Uncommon (4.2% in 11 years of disease onset) NMOSD Relatively common (up to 25%) Common (50% in 10 years of disease onset) Ther Adv Neurol Disord. 2017 Jul; 10(7): 265–289. Published online 2017 May 24. doi: 10.1177/1756285617709723
- 26. CLINICAL FEATURE MYELITIS Severe myelitiscausing complete paraplegia Painful tonic spasm associated with myelitis BRAIN Intractable hiccup and nausea (associated with area postrema lesions) MS Rare Rare Rare NMOSD Relatively common (30–70% at first attack) Relatively common (up to 25%) Relatively common (12–17%) Ther Adv Neurol Disord. 2017 Jul; 10(7): 265–289. Published online 2017 May 24. doi: 10.1177/1756285617709723
- 27. RADIOIMAGING • MRI hasrole in diagnosis as well as prognosis • T1 image • T2 image • FLAIR image for diagnosing demyelinatinglesions • T1 with Gadoliniumenhancement image
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- 33. Area postrema 3rd ventricleInternalcapsule Lateral ventrciles NMOSD
- 34. Pattern of CNSatrophy CSF Pleocytosis Oligoclonal bands OPTICAL COHERENCE TOMOGRAPHY Retinal nerve fiber layer thickness reduction MS Severe atrophy of Brain Mild to moderate Present in most patients (up to 97% with repeated test); rarely disappear in follow-up sampling Mostly in temporal quadrant NMOSD Of spinal cord Can be severe (up to 1,000/mm 3 ) Can be present in some patients (33–43%); mostly disappear in follow-up sampling Mostly in superior/inferior quadrant Ther Adv Neurol Disord. 2017 Jul; 10(7): 265–289. Published online 2017 May 24. doi: 10.1177/1756285617709723
- 35. RED FLAGS TOMS •Normal MRI •No abnormal findings on neurological exam •Bilateral vision loss •Peripheral neuropathy •Rigidity; sustained dystonia •Seizures •Headache •Early dementia •Abrupt onset of symptoms •Cortical deficits (aphasia, apraxia, alexia, neglect) •Intractable hiccups •Onset before age 10 or after age 50
- 39. ADEM • Monophasic(90%) butrelapse can occur in within 1st 3months • Antigen-antibodycomplex mediated( with in 2wk after antigen challenge) • Children(<15yrs) • No gender predilection • Incidence: 0.4/100000person years
- 40. • C/f :encephalopathy, fever, headache , seizure, multiplefocal deficit , meningeal sign • Anti- MOG AB are presents in 50% • CSF : pleocytosis • MRI brain : lesions are multiple , b/l, larger with poorly defined Margins ( ms – oval shaped, discrete) with same intensity Site- brainstem, thalamus, deep gray matter • In more severe cases – petechial hemorrhages can be seen (Hurts disease) • Spinal cord – confluentlesionswith swelling
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- 43. CASE 25 year oldmale hiv + having generalized weakness
- 44. HIV ENCEPHALOPATHY • Syndromeof cognitive, behavioural and motor abnormalitydue to direct effect of hiv • In 15-20% • Lower CD4 count, longer duration, older age are at higher risk • MRI brain: diffuse b/l, symmetrical periventricularhyperintensitieswithout mass effect/ enhancement Atrophyis a hallmark
- 45. CASE • 55year oldmale , HIV + ……noncompliant to HAART
- 46. PMLE • Progressive demyelinatingdisorders caused by jc virus • CD4 < 100 • MRI brain: confluentasymmetric t2 hyperintensitiesin peripheral white matter and subcortical U- fibres No mass effect, no enhancement
- 47. CASE • 24 yearold female with autoimmune disorder and intractableseizures
- 49. CNS LUPUS • SLEis a multisystem autoimmune vasculopathywith CNS involvement in upto 75% • Image: focal area of cortical and subcorticalt2, extensively confluenthyperintensities Vasculitis/infarcts • May have superimposed PRES
- 50. CASE • 42 yrsold female k/c/o HTN with persistent headache
- 53. PRES • Dysfunction incerebrovascular regulation • Etiology : HTN, preeclampsia/eclampsia, chemotherapy • MRI brain: patchycortical and subcorticalhyperintensities ✓95% in parieto-occipital lesions, b/l but asymmetric ✓+/- Basal ganglia, pons, cerebellum ✓+/- Hemorrhage • Usually dwi is normal
- 54. CASE • 72 yearsold female , with altered mental status.
- 55. ODS • Acute demyelinationcaused by rapid shift in osmolality ( rapid correction of hyponatremia) • 65% pons, 35%extra-pontine( basal ganglia, thalami, white matter) • T2 hyperintensityclassically spares corticospinaltracts and periphery
- 59. TAKE HOME MESSAGE Thusa sound knowledge of clinical and paraclinical characteristicallows clinician to differentiate various demyelinating disorders.
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