MRI and differential diagnosis in patients suspected of having MS

Andrea Falini Italy MRI and differential diagnosis in patients suspected of having MS IMPROVING THE PATIENT S LIFE THROUGH MEDICAL EDUCATION www.excemed.org

Outline of presentation - Diagnostic criteria - MS features at diagnosis - Red flags - MS variants - Other demyelinating conditions - MS mimickers

Clinically Isolated Syndromes The three most common CIS syndromes seen at presentation in the MS diagnosis process include: Brainstem or cerebellum Spinal cord Optic nerve

Posterior fossa lesions topography The preferred areas of involvement are the floor of the IV ventricle, the cerebellar peduncles and the surface of the pons.

Spinal cord lesions characteristics Small size: < 2 vertebral segments < half of cord cross-sectional area Location: cervical > than the rest lateral + posterior columns central GM not spared Shape: cigar-like Signal characteristics: enhancement uncommon rarely T1 hypointense Effect on the cord: swelling (acute phase) local atrophy (chronic phase) Lycklama et al., Lancet Neurol 2003

OPTIC NERVE - Optic neuritis can be found in up to 75% of Patients with MS - 20 31% of patients with a clinically isolated syndrome present with acute optic neuritis T2 Hyperintensity T1 FAT- SAT enhancement

Clinically Isolated Syndromes The three most common CIS syndromes seen at presentation in the MS diagnosis process include: Brainstem or cerebellum Spinal cord Optic nerve

Double Inversione Recovery - DIR T2 FLAIR DIR 3D T1

Outline of presentation - Diagnostic criteria - MS features at diagnosis - Red flags - MS variants - Other demyelinating conditions - MS mimickers

A diagnosis of multiple sclerosis is based on showing disease dissemination in space and time and excluding other neurological disorders that can clinically and radiologically mimic multiple sclerosis The aim of the MAGNIMS workshop was not to review all the possible disorders that can enter a differential diagnosis with MS but rather to define a set of MRI red flags (features atypical for multiple sclerosis but instead suggestive of an alternative diagnosis) derived from evidence-based findings and educated guess

Outline of presentation - Diagnostic criteria - MS features at diagnosis - Red flags - MS variants - Other demyelinating conditions - MS mimickers

Differential Diagnosis Variants of multiple sclerosis Balo s concentric sclerosis Acute multiple sclerosis (Marburg type) Schilder s disease Tumefactive lesions

Tumefactive (pseudotumoral) lesions Given et al. AJR 2004

October Tumefactive (pseudotumoral) lesions

October

6 months after

15 April

21 May

Progressive Multifocal Leukoencephalopathy (polyoma virus JC)

Outline of presentation - MS feature at diagnosis - Diagnostic criteria - Red flags - MS variants - Other demyelinating conditions - MS mimickers

Differential Diagnosis Other demyelinating conditions Neuromyelitis optica spectrum disorder Acute disseminated encephalomyelitis Acute transverse myelitis

Neuromyelitis Optica (Devic) Idiopathic inflammatory demyelinating disease with severe synchronous or sequential clinical optic nerve and spinal cord involvement that typically spares the brain and tends to leave severe residual injury early with each attack, unlike MS. Neuromyelitis Optica Spectrum Disorder

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21.10. 0

NMO-optic neuritis ON is usually severe Often bilateral T2 Lesions frequently affect the posterior optic nerve and chiasm Enhancement in 94% at onset T1 SPIR c.e.

NMO Anti-AQP4-Ab+ vs. Anti-MOG-Ab+

Anti-MOG-Ab+

NMO Anti-AQP4-Ab+ vs. Anti-MOG-Ab+

LETM vs APTM Longitudinally Extensive Transverse Myelitis: >3 segments centrally located majority of transverse axis MS risk <2% Acute Partial Transverse Myelitis: < 2 segments eccentric or asymmetric location MS Risk: 10% at 61 months in MRI - 88% in MRI+ 21% at 20 yy Fisniku LK, Brex PA, Altmann DR, et al. Brain 2008

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Kim HJ et Al Neurology 2015

Brain involvement: MS vs NMO - Lateral ventricle and inferior temporal lobe lesion - Dawson fingers, or an S-shaped U-fiber lesions - Cortical lesions - Surround central venule in 80% on high-strength MRI

ADEM - Acute Disseminated Encephalo-Myelitis Monophasic immuno-mediated demyelinating disease that predominantly affects children in response to a preceding infection or vaccination Syncronous involvement of brain and spinal cord Symmetric lesion with poorly defined margins Involvement of both white and gray matter Predominant involvement of subcortical wm Scarse involvement of periventricular wm and cc Thalamic and basal ganglia involvement Brainstem and cerebellar involvement Synchronous enhancement

ADEM

ADEM NMO

Outline of presentation - MS feature at diagnosis - Diagnostic criteria - Red flags - MS variants - Other demyelinating conditions - MS mimickers

Differential Diagnosis Hypoxic-ischaemic vasculopathies and vasculitides Hypoxic-ischaemic vasculopathies Hypoxic-ischaemic cerebral small vessels disorders: The most common DD is the possibility that these lesions can also be caused by hypoxic-ischaemic cerebral small-vessels disorders, which are usually asymptomatic but can also present with migraine, TIA, stroke or subcortical arteriosclerotic encephalopaty. Prevalence of MS 1/1000 whereas MRI wm lesions due to smal vessels disesases occurs in 5-10% of patients aged 20-40 years Small Vessel Diseases lesions spare corpus callosum, subcortical U-fibres and spinal cord CADASIL Hyperhomocysteinaemia Mitochondrial disorders: Leber s hereditary optic neuropathy, chronic progressive ophthalmoplegia, and mitochondrial encephalopathy with lactic acidosis and stroke-like episode Susac s Syndrome

Hypertensive vasculopathy

CADASIL

Susac Syndrome Microangiopathy of brain, retina, and cochlea Baseline 1 month

Differential Diagnosis Hypoxic-ischaemic vasculopathies and vasculitides Vasculitides Primary angiitis of the CNS Systemic immune-mediated diseases (SLE, Behcet s)

PACNS Primary angiitis of the CNS is a rare disorder with an unknown cause that can be defined as a vasculitis affecting the CNS without an associated systemic disorder. Is characterised by non-specific granulomatous inflammation of small cerebral parenchymal and leptomeningeal arteries and veins. White matter lesions Deep Periventricular Subcortical

Cortical infarcts (50 %) PACNS

Hemorrhages (10%) PACNS

PACNS Enhancement (30%), leptomeningeal (10-15%)

Mural Enhancement PACNS

PACNS Subcortical MCP CC

F. 52 y.o. Acute onset of dizziness, dysarthria and mild left hemiplegia

Diagnostic criteria for MS Sites Periventricular Infratentorial Corpus callosum Juxtacortical (u-fibers) Shape Irregular Ovoid Dystribution Asymmetric Evolution?

Evolution: Behcet's Disease

Differential Diagnosis Infectious Disease Meningitis Encephalitis Intracerebral abscess Progressive multifocal leucoencephalopathy

Lyme Disease

Differential Diagnosis Other conditions Sarcoidosis Histiocytosis PCNSL???

F45 aa having visual disturbances plus macular oedema. Rule out MS

PVL - periventricular leukomalacia

MS: Differential Diagnosis CONCLUSION MRI play an important role in differentiating MS from other condition. Although there are overlapping profiles, the location and pattern of distribution of the lesions often favour a specific pathology. If the clinical information, MRI findings and laboratory data are analyzed together, a proper diagnosis can be established for most patients.