Clinical manifestations of multiple sclerosis

Dr. Hesham Awn Taiz 2010

The clinical syndrome of MS is classically described as a relapsing remitting disorder that affects multiple white matter within the CNS, with usual onset in young adults.

The varied clinical features reflect the multifocal areas of CNS destruction ,although discrepancies occur between the extent of clinical and pathological findings . The high degree of variability and the difficulty in predicting the causes and severity make MS one of the most puzzling of

CNS diseases.

Cognitive impairment

Cognitive impairment has been underreported in MS . neuropsychological test results have shown that 34% to 65%of patients with MS have cognitive impairment. The most frequent abnormalities are with recent memory ,attention ,and speed of information processing.

Affecting disorders:

Cross sectional studies have shown some degree of affecting disturbance in a significant number of patients with MS. Depression is the most common manifestation and is in part secondary to the burden of having a chronic ,incurable disease, however , it is more prevalent in MS than in other chronic diseases, suggesting an organic component as well.

Affecting disorders:

Euopharia is infrequent and usually associated with moderate or sever cognitive impairment . Patients may manifest a dysphoric state with swing from depression to elation.

Cranial nerves dysfunction

Impairment of visual pathways: optic neuritis is the most frequent type of involvement of visual pathways.

Cranial nerves dysfunction

Although uncommon ,homonymous field defects caused by involvement of the optic radiations can be seen in MS.

Cranial nerves dysfunction

Impairment of ocular motor nerves is infrequent in MS , when present , the involved nerves are , in decreasing order of frequency : VI , III and IV. More frequent findings are those that reflect lesions of : vestibulo-ocular connection acquired pendular nystagmus. internuclear connection ophthalomplegia. internuclear

Cranial nerves dysfunction

Cranial nerves dysfunction

Impairment of other cranial nerves:

impairment of facial sensation, subjective or objective, is a relatively common finding in MS. Trigeminal Neuralgia in a young adult is frequently an early sign of MS. Facial myokymia , hemifecial spasm, facial palsy. Vertigo is a reported symptom in 30 to 50 %of patient with MS. Malfunction of the lower cranial nerves is usually of UMN type (pseudobulbar syndrome) and is usually a late finding in MS.

Impairment of the sensory pathways:

Sensory manifestations are a frequent initial feature of MS and are present in almost every patient at some time during the cause of disease. The sensory features can reflect spinothalamic ,posterior column ,or dorsal root entry zone lesions.
the sensory symptoms are commonly described as numbness ,tingling, pins and needle ,tightness, coldness ,itching or swelling of limb or trunk.

Impairment of the motor pathways:

Corticospinal tract dysfunction is common in MS. Paraparesis ,or parapligia ,occurs more frequently than significant weakness in the upper extremities.

Impairment of cerebellar pathways

Cerebellar pathway impairment results in gait imbalance and other cerebellar signs.

Impairment of cerebellar pathways

In sever cases complete astasia ,inability to use the arms because of a violent intention tremor , and virtually incomprehensible speech may occur.

Impairment of Bladder ,Bowel ,And sexual function

The extent of sphincter and sexual dysfunction often parallels the degree of motor impairment in the lower extremities.

Clinical features distinctive of MS

Although no clinical phenomena are entirely unique to MS ,some are highly characteristic of the disease : onset between the ages of 15 and 50 years. involvement of multiple areas of the CNS. optic neuritis. internuclear ophthalmoplegia. Fatigue. Lhermittes phenomenon. Uhthoffs phenomenon.

Diagnostic criteria:

The corner stone of the diagnosis of MS remains the neurological history and

physical examination.

Diagnostic criteria:

To improve the homogeneity of MS patient groups being studied ,the Schumacher committee on diagnostic criteria of MS elaborated six items required to diagnose clinically definite MS.

the Schumacher diagnostic criteria of MS

Objective CNS dysfunction. Involvement of white matter structures. Tow or more sites of CNS involvement. Relapsing remitting or chronic (more than 6 months). age 10-50 years at onset. No better explanation of symptoms as assessed by a competent neurologist.

Diagnostic criteria:
The criteria were modified in 1983 by Poser and colleagues expanding the age at onset to 59 years and using data derived from laboratory studies ,including analysis of the CSF, evoked potentials (EP) and neuroimaging.

Diagnostic criteria:

More recently (2005) ,McDonald and colleagues proposed new criteria that include detailed guidelines for MRI and timing intervals to determine possible or definite MS. The outcome of a diagnostic evaluation is either MS ,possible MS , or not MS. these criteria were designed for use in both practice and in clinical trial.

Diagnostic criteria:
The common thread among all MS diagnostic criteria has been the requirement for symptoms and signs that are disseminated in space and time (more than one episode involving more than one area of the CNS).

Diagnostic criteria:
The patient early course who dose not meet diagnostic criteria remains clinically challenging in the setting of a monophasic neurological illness that is clinically consistent with MS and accompanied by multifocal white matter lesions on MRI consistent with demyelinating plaques , the diagnosis of MS is almost certain , this situation is referred to as

clinically isolated syndrome.

Such patients would be classed as possible MS by the latest diagnostic criteria.

course
Four categories of disease are described: Relapsing remitting (RR) MS: clearly defined relapses with full recovery or with squelae and residual deficit on recovery. The periods between relapses are characterized by a lack of disease progression.

course
Secondary

progressive (SP) MS:

Initial relapsing remitting disease course followed by progression with or without occasional relapses , minor remissions ,and plateaus.

course
Primary

progressive (PP) MS:

Disease progression from onset with occasional plateaus and temporary minor improvement allowed.

course
Progressive

relapsing (PR) MS:

Progressive disease from onset ,with clear acute relapses ,with or without full recovery.

The periods between relapses are characterized by continuing progression.

Differential diagnosis

The differential diagnosis of MS is quite limited in the setting of a young adult with tow or more clinically distinct episodes of CNS dysfunction with at least partial resolution. Problems arise with atypical presentations, monophasic episodes , or progressive illness.

Differential diagnosis

A monophasic illness with symptoms attributable to one site of the CNS creates a large differential diagnosis that includes

neoplasm , vascular event ,and infectious

A appropriate imaging study may help clarity the situation ,depending on the site of involvement and clinical progression.

Differential diagnosis

The most trouble arise with progressive CNS dysfunction ,in which great care must be taken to exclude treatable etiologies (vit B12

deficiency ,compression spinal cord lesion , arteriovenous malformation ,cavernous angioma ,Arnold-chiari malformation ) , infectious causes , and hereditary disorders.

Differential diagnosis
A common error to over interpret multiple hyperintense lesions on MRI as equivalent to MS . Clinical symptoms must be consistent with MS.

Features that should alert the clinician to the possibility of other disease include:
family history of neurological disease. a well- demarcated spinal level in the absence of disease above the foramen magnum. prominent back pain that persist. Symptoms and signs that can be attributed to one anatomical sites. Patients who are older than age 60 years or younger than 15 years at onset . Progressive disease .

Non of this features excludes the diagnosis of MS, but these situations one should seek other etiologies before accepting the diagnosis of MS.