E. Lee Murray, MD
OVERVIEW
The hospital neurologist is often consulted to coordinate the evaluation and management of a host of demyelinating diseases. Among the most common scenarios are:
•Deficits that may be a new diagnosis of multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), or neuromyelitis optica (NMO)
•Visual loss that may be optic neuritis
•Myelopathy that may be transverse myelitis or NMO
•Abnormal magnetic resonance imaging (MRI) suggestive of demyelinating disease
MULTIPLE SCLEROSIS
MS is a common diagnosis for outpatient and inpatient neurology care. The hospital neurologist’s responsibilities for patients with MS usually revolves around one of the following:
•Neurologic presentation in a patient not previously known to have MS
A detailed discussion of comprehensive MS management is beyond the scope of this text and will not be discussed here. Here, we concentrate on the implications of MS for hospital neurology practice.
Overview of MS
MS usually presents in relative youth, from childhood to middle age with an average age at diagnosis of about 30 years.
PRESENTATION can be with almost any central neurologic deficit, but most common are visual loss, diplopia, gait and/or limb ataxia, paraparesis, or hemiparesis. Onset can be subacute, developing over hours to days.
DIAGNOSIS is suspected when a patient seen in the hospital or ED with neurologic deficit has either multifocal deficits, history of a subacute progression, or history of previous attacks of neurologic deficit. Relatively young age supports the diagnosis.
•MRI brain is performed and shows areas of increased signal on T2 and fluid attenuated inversion recovery (FLAIR). There are defined criteria that have undergone revision.1
•CSF is most helpful if it shows oligoclonal bands. There may be a mild pleocytosis and mild elevation in protein. Because of liberalization of MRI criteria, CSF is not universally evaluated.
•Evoked potentials have historically been used for identification of lesions, but this is less used now, especially in a hospital setting.
The diagnosis of MS is divided into categories based on clinical history and lesion identification on tests such as MRI and evoked potential:
•Definite MS: Two or more attacks + two or more test-identified lesions
•Probable MS: Either single attack + multifocal test-identified lesions, or a single identified lesion + two or more attacks
•Clinically isolated syndrome (MS): A single attack typical of MS without test evidence of another lesion
Location of the lesions is implicated in the criteria. The criteria from the working group cited earlier indicate that, for identification of spatially distinct lesions, there should be at least two lesions that are in separate CNS arenas—periventricular, juxtacortical, infratentorial, and spinal cord.
When the disorder is believed to be MS, there are subdivisions:
•Relapsing-remitting: Episodic attacks with no progression between them
•Secondary progressive: Progressive phase after years of relapsing-remitting
•Primary progressive: Progressive disease from the onset
MANAGEMENT in the hospital setting begins with treatment of an acute attack. Then, disease-modifying therapy can be introduced, but we often do not start this therapy in the hospital since office follow-up for care coordination and monitoring is required.
Acute attack: Whether a first attack or a relapse, corticosteroids are used. IV administration is usually used first, and then oral is typically used for subsequent attacks. After the high-dose administration, a taper is sometimes used. Regimens that have been used include :
•Methylprednisolone 1 g/day IV × 3–5 days
•Prednisone 1,000 mg/day PO × 3–5 days
•Dexamethasone 160 mg/day PO × 3–5 days
Disease-modifying therapy is used especially for relapsing-remitting MS and is often offered to patients who do not meet criteria but are felt to potentially be at high risk for MS. Detailed discussion is beyond the scope of this book. There are numerous medications approved for disease-modifying therapy, just a few of which are interferons, glatiramer, natalizumab, mitoxantrone, fingolimod, and dimethyl fumarate Others have been released and more are in development. All of these are approved for relapsing forms of MS, mitoxantrone is also used for select patients with secondary progressive MS.
First Diagnosis of MS in the Hospital
PRESENTATION in the hospital setting is usually with significant motor or visual deficit. Paraparesis, hemiparesis, diplopia, and monocular or binocular visual loss would be the most likely ED presentations.
DIAGNOSIS is more or less difficult depending on the type. Objective neurologic deficit with typical MRI lesions is most common. If the MRI findings are absent or equivocal, then diagnosis is less certain.
Optic neuritis (ON) is covered later. However, when a patient presents to the hospital with ON and is found to have historical and/or imaging evidence of MS, treatment of the acute event with high-dose corticosteroids is routine. Disease-modifying therapy is offered after acute hospitalization.
Tumefactive MS is an MS lesion that has the appearance of a tumor.2 This is a difficult diagnostic dilemma. Some possible clinical scenarios include:
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