MYELITIS. A Mochan Neurology

Transcription

1 MYELITIS A Mochan Neurology

2 ATM MS LETM NMOSD

3 ATM LETM MS NMOSD Acute Transverse Myelitis Longitudinally Extensive Transverse Myelitis Multiple Sclerosis Neuromyelitis Optica Spectrum Disorders

4 ATM ADEM MS LETM NMOSD Acute Transverse Myelitis Longitudinally Extensive Transverse Myelitis Multiple Sclerosis Neuromyelitis Optica Spectrum Disorders Acute Disseminated Encephalomyelitis

5 (ACUTE) TRANSVERSE MYELITIS Acquired immune mediated spinal cord inflammation Rapid onset weakness, sensory loss, bladder/bowel dysfunction Duration of progression: > 4 hours < 21 days N.B. Always exclude compressive and noninflammatory causes of acute myelopathy

6 CLASSIFICATION Partial TM: Mild or very asymmetric spinal cord dysfunction MRI: lesion extending over 1 to 2 vertebral body heights or normal Complete TM: Symmetric and complete transverse neurological deficit below the level of the lesion MRI lesion extending over 1 or 2 vertebral segments Longitudinally extensive transverse myelitis (LETM): Complete or incomplete spinal cord dysfunction MRI lesion extending over 3 or more vertebral segments Subtypes imply distinct differential diagnoses and prognoses.

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9 Aetiology Post /para infectious Infectious: Herpes viruses; Enteroviruses; West Nile virus; Retroviruses; Zika virus Lyme; mycoplasma; mycobacteria; Syphilis Infectious causes of myelitis are rare. Neurosarcoidosis Paraneoplastic spinal cord disease

10 Aetiology Systemic autoimmune disorders with TM: SLE Sjögren syndrome Scleroderma Behçet disease Antiphospholipid antibody syndrome Ankylosing spondylitis Rheumatoid arthritis

11 TM Epidemiology Rare 1 to 5 new cases per million per year Female preponderance, especially with MS and more so with NMOSD (up to 9:1) Bimodal age peak 2 nd and 4 th decades 15 60% idiopathic

12 Myelopathy Profile at CHB Impact of HIV 100 consecutive non traumatic myelopathies referred to the Neurology Unit, 2006 History and Examination Laboratory Investigations: Blood, CSF, histology Chest X Ray MRI Non traumatic myelopathy at the Chris Hani Baragwanath Hospital, South Africa the influence of HIV Modi G, Ranchhod J, Hari K, Mochan A, Modi M. QJM. 2011; 104:

13 Results

14 HIV Positive Cohort Number of Patients = 50 Known HIV Positive: 31 Newly Diagnosed: 19 CD4 (cells/ml) Mean 154 CD4 Categories CDC1 38 CDC2 10 CDC3 1 ARV Medication: 9 patients were on ARVs 8 patients < 200cells/ml, 1 patient cells/ml Duration: 1month 3years

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16 Conclusions Approximately 50% of patients with non traumatic myelopathy in an HIV endemic region are HIV positive. HIV positive patients: Younger Infectious aetiologies Predominantly TB Advanced HIV infection Myelitis: infectious and autoimmune rare HIV negative patients: Older Neoplasms Cervical Spondylosis Myelitis: autoimmune rare

17 Myelitis Differential Diagnosis Compressive cord lesions Myelitis mimics: Guillain Barre syndrome Radiation Non inflammatory conditions

18 Non inflammatory conditions resembling myelitis Vascular myelopathy Anterior spinal artery infarction Spinal AVM Fibrocartilaginous embolism Metabolic/nutritional/toxic Myelopathies Vit B12 deficiency Vitamin E deficiency Copper deficiency Lathyrism Konzo

19 ATM LETM MS NMOSD Acute Transverse Myelitis Longitudinally Extensive Transverse Myelitis Multiple Sclerosis Neuromyelitis Optica Spectrum Disorders

20 Neuromyelitis Optica (NMO) Devic s disease Classic Features monophasic illness acute or subacute optic neuritis usually bilateral severe acute transverse myelitis within 8 weeks normal brain MRI high mortality severe morbidity/disability in survivors

21 Neuromyelitis Optica (NMO) Devic s disease PROBLEMS Strictly unilateral optic nerve or partial spinal lesions progression to multiphasic illness, sometimes with a typical MS type pattern more widespread demyelination in the brain on MRI and at postmortem examination than was clinically manifest in the optic nerves and spinal cord reminiscent of ADEM or MS expanding intervals between index events of as long as 2.5 years

22 Neuromyelitis Optica (NMO) Devic s disease Diagnostic criteria: a severe transverse myelitis, para or quadriparesis, with or without sphincter involvement, evolves over 1 14 days with a sensory level and no cord compression an acute unilateral or bilateral optic neuropathy no clinical involvement beyond spinal cord or optic nerves monophasic or multiphasic illness O Riordan et al 1996

23 MS NMO Common in caucasians 30 years disease rarely confined to optic nerves and spinal cord cognitive, brainstem and cerebellar involvement is common lymphocyte pleocytosis Common in Japanese, Asians, and in the tropics 40 years disease restricted to optic nerves and spinal cord cognitive, brainstem and cerebellar involvement absent Polymorphs and lymphocytes

24 MS NMO Oligoclonal IgG present in 90% signal change predominantly periventricular, swelling and cavitation are rare new lesions with resolution of old lesions on serial scans demyelination with minimal oedema, no cavitation and necrosis only white matter involved no change in vascular morphology relatively good prognosis Oligoclonal IgG absent/transiently present signal change predominantly in optic nerves and cord, with swelling and cavitation on going resolution with no newlesions on serial scans demyelination with oedema, cavitation and necrosis grey and white matter affected thickened hyalinised blood vessels poor prognosis (35 50% mort.)

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27 Diagnosis of neuromyelitis optica and longitudinally extensive transverse myelitis Jarius S et al. (2008) Mechanisms of Disease: aquaporin-4 antibodies in neuromyelitis optica Nat Clin Pract Neurol /ncpneuro0764

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30 NMOSD Aquaporin 4 IgG mediated immune reactivity and complement deposition Astrocyte damage on histology vs. Oligodendrocyte damage in MS Optico spinal MS is NMO Implications on treatment New nomenclature Characteristic extra optico spinal syndromes

31 International consensus diagnostic criteria for neuromyelitis optica spectrum disorders by Dean M. Wingerchuk, Brenda Banwell, Jeffrey L. Bennett, Philippe Cabre, William Carroll, Tanuja Chitnis, Jérôme de Seze, Kazuo Fujihara, Benjamin Greenberg, Anu Jacob, Sven Jarius, Marco Lana-Peixoto, Michael Levy, Jack H. Simon, Silvia Tenembaum, Anthony L. Traboulsee, Patrick Waters, Kay E. Wellik, and Brian G. Weinshenker Neurology Volume 85(2): July 14, American Academy of Neurology

32 Figure 1 Spinal cord and optic nerve MRI patterns in neuromyelitis optica spectrum disorder Spinal cord imaging in the context of acute myelitis in neuromyelitis optica spectrum disorders (NMOSD) usually reveals a longitudinally extensive transverse myelitis (LETM) lesion extending over 3 or more vertebral segments. Dean M. Wingerchuk et al. Neurology 2015;85: American Academy of Neurology

33 Figure 2 Dorsal medulla, area postrema, and other brainstem lesions in neuromyelitis optica spectrum disorder Sagittal T2-weighted fluid-attenuated inversion recovery (FLAIR) MRI shows a lesion in the dorsal medulla (A; arrow). Dean M. Wingerchuk et al. Neurology 2015;85: American Academy of Neurology

34 Figure 3 Diencephalic and cerebral lesions in neuromyelitis optica spectrum disorder A variety of brain lesion patterns are associated with neuromyelitis optica spectrum disorder. Dean M. Wingerchuk et al. Neurology 2015;85: American Academy of Neurology

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37 TREATMENT Any TM: high dose intravenous Methylprednisolone Treatment of underlying/associated systemic autoimmune disorder Treatment strategies for attack prevention in NMO and MS differ. Some MS immunotherapies appear to aggravate NMO, indicating an imperative for early, accurate diagnosis. Humoral/B cell mediated immunity targeted: PE, Rituximab, Anti IL6 receptor monoclonal antibodies

38 ATM MS LETM NMOSD THANK YOU The End