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Review Article
The early diagnosis and treatments in multiple sclerosis - So Young Huh
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Kosin Medical Journal 2017;32(2):151-163.
DOI: https://doi.org/10.7180/kmj.2017.32.2.151
Published online: January 19, 2017
Department of Neurology, College of Medicine, Kosin University, Busan, Korea
- Corresponding Author: So Young Huh, Department of Neurology, College of Medicine, Kosin University, 262, Gamcheon-ro, Seo-gu, Busan 49267, Korea Tel: +82-51-990-6461 Fax: +82-51-990-3077 E-mail: caccu@naver.com
• Received: April 6, 2017 • Accepted: April 19, 2017
Copyright © 2017 Kosin University School of Medicine Proceedings
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Abstract
- Multiple sclerosis (MS) is a chronic inflammatory demyelinating disorder of the central nervous system that leads to neurological disability. The diagnosis of MS relies on the MRI criteria, which can demonstrate dissemination in space and time. Exclusion of other demyelinating mimics is essential because there are no specific biomarker for MS and MRI criteria are still have imperfect. There is incremental improvements in MS treatment option that have contributed to the delay of disease progression. The early initiation of DMT may ameliorate the neurological disability. In this review, we discusses the new diagnostic MS criteria and summarize the evidences supporting the early treatment in the course of MS.
Fig. 1.Example of lesion for multiple sclerosis MRI Criteria of dissemination in space (A) periventricular lesions; (B) juxtacortical lesions; (C) infratentorial lesions; (D) spinal cord lesion; (E) optic nerve lesion. Gadolinium-enhancing lesion in T1 gadolinium weighted image; (F) are visible in the patient with MS.
Table 1.CIS clinical features and likelihood of signaling an MS diagnosis4
Table 2.2010 McDonald criteria and 2016 MAGNIMS Diagnosis criteria8
Table 3.Clinical and MRI major red flags suggestive of alternative diagnosis to multiple sclerosis4
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Fig. 1. Example of lesion for multiple sclerosis MRI Criteria of dissemination in space (A) periventricular lesions; (B) juxtacortical lesions; (C) infratentorial lesions; (D) spinal cord lesion; (E) optic nerve lesion. Gadolinium-enhancing lesion in T1 gadolinium weighted image; (F) are visible in the patient with MS.
Fig. 1.
The early diagnosis and treatments in multiple sclerosis
CIS clinical features and likelihood of signaling an MS diagnosis4
| CIS features typically seen in MS | Less common CIS features which may be seen in MS | Atypical CIS features not expected in MS |
|---|---|---|
| Optic nerve | ||
| Unilateral optic neuritis Pain on eye movement Partial and mainly central visual blurring Normal disc or mild disc swelling |
Bilateral simultaneous optic neuritis No pain No light perception Moderate to severe disc swellingwithno hemorrhages Uveitis (mild, posterior) |
Progressive optic neuropathy Severe, continuous orbital pain Persistent complete loss of vision Neuroretinitis (optic disc swelling with macular star) Uveitis (severe, anterior) |
| Brain stem/cerebellum | ||
| Bilateral internuclear ophthalmoplegia Ataxia and multidirectional nystagmus Sixth nerve palsy Facial numbness |
Unilateral internuclear ophthalmoplegia, facial palsy, facial myokymia Deafness One-and-a-half syndrome Trigeminal neuralgia Paroxysmal tonic spasms |
Complete external ophthalmoplegia; verticalgaze palsies Vascular territory syndrome, e.g., lateramedullary Third nerve palsy Progressive trigeminal sensory neuropathy Focal dystonia, torticollis |
| Spinal cord | ||
| Partial myelopathy Lhermitte’s symptom Deafferented hand Numbness Urinary urgency, incontinence, erectile dysfunction Progressive spastic paraplegia (asymmetrical) |
Complete transverse myelitis Radiculopathy, areflexia Segmental loss of pain andtemperature sensation Partial Brown-Sequard syndrome (sparing posterior columns) Faecal incontinence Progressive spastic paraplegia (symmetrical) |
Anterior spinal artery territory lesion (sparing posterior columns only) Cauda equina syndrome Sharp sensory level to all modalities andlocalized spinal pain Complete Brown-Sequard syndrome Acute urinary retention Progressive sensory ataxia (posterior columns) |
| Cerebral hemispheres | ||
| Mild subcortical cognitive impairment Hemiparesis |
Epilepsy Hemianopia |
Encephalopathy (obtundation, confusion, drowsiness) Cortical blindness |
2010 McDonald criteria and 2016 MAGNIMS Diagnosis criteria8
| 2010 McDonald criteria | 2016 MAGNIMS criteria | |
|---|---|---|
| Dissemination in space |
≥2 of 4 characteristic location ≥1 periventricular lesion ≥1 juxtacortical lesion ≥1 asymptomatic brainstem lesion ≥1 asymptomatic spinal cord lesion |
≥2 of 5 characteristic location ≥3 periventricular lesion ≥1 cortical/juxtacortical lesion ≥1 brainstem lesion ≥1 spinal cord lesion ≥1 optic nerve lesion *If a patient has a brainstem or spinal cord syndrome, or optic neuritis, the symptomatic lesion (or lesions) are not excluded from the criteria and contribute to the lesion count. †This combined terminology indicates the involve- ment of the white matter next to the cortex, the involvement of the cortex, or both, thereby expanding the term juxtacortical lesion. |
| Dissemination in time |
- Simultaneous presence of asymptomatic Gd-en- hancing and non-enhancing lesion at any time - A new T2 and/or Gd-enhancing lesion on follow-up MRI irrespective of timing of baseline scan |
- Simultaneous presence of asymptomatic Gd-en- hancing and non-enhancing lesion at any time - A new T2 and/or Gd-enhancing lesion on follow-up MRI irrespective of timing of baseline scan - Clinical symptoms should be associated with radiologically isolated syndrome |
Clinical and MRI major red flags suggestive of alternative diagnosis to multiple sclerosis4
| Red flag | Type | Examples of alternative diagnosis |
|---|---|---|
| Bone lesions | Clinical | Histiocytosis; Erdheim Chester disease |
| Lung involvement | Clinical | Sarcoidosis; Lymphomatoid granulomatosis |
| Multiple cranial neuropathies or pol-yradiculopathy | Clinical | Chronic meningitis, including sarcoidosis and tuberculosis; Lyme disease |
| Peripheral neuropathy | Clinical | B12 deficiency; adrenoleukodystrophy; metachromatic leukodystrophy, Lyme disease |
| Tendon xanthomas | Clinical | Cerebrotendinous xanthomatosis |
| Cerebral venous sinus thrombosis | MRI | Behçet’s disease; vasculitis; chronic meningitis, antiphospholipid or anticardiolipin antibody syndromes |
| Cardiac disease | Clinical | Multiple cerebral infarcts; brain abscesses with endocarditis or right to left cardiac shunting |
| Myopathy | Clinical | Mitochondrial encephalomyopathy (e.g.,MELAS); Sjögren’s syndrome |
| Cortical infarcts | MRI | Embolic disease; thrombotic thrombocytopenic purpura; vasculitis |
| Hemorrhages/ microhemorrhages | MRI | Amyloid angiopathy; Moya Moya disease; CADASIL; vasculitis |
| Meningeal enhancemen | MRI | Chronic meningitis; sarcoidosis; lymphomatosis; CNS vasculitis |
| Extrapyramidal features | Clinical | Whipple’s disease; multisystem atrophy; Wilson’s disease |
| Livedo reticularis | Clinical | Antiphospholipid antibody syndrome; systemic lupus erythematosus; Sneddon’s syndrome |
| Retinopathy | Clinical | Mitochondrial encephalomyopathy; Susac, and other vasculitides (retinal infarction); neuronal ceroid lipofuscinosis |
| Calcifications on CT scans | MRI | Cysticercosis; toxoplasmosis, mitochondrial disorders |
| Diabetes insipidus | Clinical | Sarcoidosis; histiocytosis |
| Increase serum lactate level | Clinical | Mitochondrial disease |
| Selective involvement of the anterior temporal and inferior frontal lobe | MRI | CADASIL |
| Hematological manifestations | Clinical | Thrombotic thrombocytopenic purpura; vitamin B12 deficiency; Wilson’s disease (hemolytic anemia); copper deficiency |
| Lacunar infarcts | MRI | Hypertensive ischemic disease; CADASIL; Susac syndrome |
| Persistent Gd-enhancement and continued enlargement of lesions | MRI | Lymphoma; glioma; vasculitis; sarcoidosis |
| Mucosal ulcers | Clinical | Behçet’s disease |
| Myorhythmia | Clinical | Whipple’s disease |
| Hypothalamic disturbance | Clinical | Sarcoidosis; neuromyelitis optica; histiocytosis |
| Recurrent spontaneous abortion or thrombotic events | Clinical | Antiphospholipid antibody syndrome; thrombotic thrombocytopenic purpura; metastatic cancer with hypercoagulable state |
| Simultaneous enhancement of all lesions | MRI | Vasculitis; lymphoma; sarcoidosis |
| Rash | Clinical | Systemic lupus erythematosus; T-cell lymphoma; Lyme disease, Fabry disease |
| T2-hyperintensity in the dentate nuclei | MRI | Cerebrotendinous xanthomatosis |
| Arthritis, polyarthalgias, myalgias | Clinical | Systemic lupus erythematosus; Lyme disease; fibromyalgia |
| Amyotrophy | Clinical | Amyotrophic lateral sclerosis; syringomyelia; polyradiculpathy |
| Headache or meningismus | Clinical | Venous sinus thrombosis; chronic meningitis; lymphoma or glioma, vasculitis, systemic lupus erythematosus |
| T1-hyperintensity of the pulvinar | MRI | Fabry disease; hepatic encephalopathy; manganese toxicity |
| Persistently monofocal manifestations | Clinical | Structural lesion (e.g., Chiari malformation); cerebal neoplasm |
| Large and infiltrating brainstem lesions | MRI | Behçet’s disease; pontine glioma |
| Predominance of lesions at the cortical/subcortical junction | MRI | Embolic infarction; vasculitis; progressive multifocal leukoencephalopathy |
Table 1. CIS clinical features and likelihood of signaling an MS diagnosis4
Table 2. 2010 McDonald criteria and 2016 MAGNIMS Diagnosis criteria8
Table 3. Clinical and MRI major red flags suggestive of alternative diagnosis to multiple sclerosis4
CADASIL; cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, MELAS; mitochondrial myopathy, encephalopathy, lactic acidosis, stroke-like episodes syndrome
