Medication Summary

The goal of pharmacotherapy is to reduce morbidity.

Class Summary

These are useful in minimizing the effects of autoimmune reactions.

Intravenous immunoglobulin (IVIg)

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Following features may be relevant to its efficacy: neutralization of circulating antibodies through anti-idiotypic antibodies; down-regulation of pro-inflammatory cytokines, including IFN-gamma; blockade of Fc receptors on macrophages; suppression of inducer T and B cells and augmentation of suppressor T cells; blockade of complement cascade.

Class Summary

These agents modify the body's immune response to diverse stimuli. Likely mechanisms of action are inhibition of synthesis/secretion of TNF-alpha, IL-6, IL-2, and IFN-gamma, and modulation of serum and leukocyte-bound levels of cell adhesion molecules.

Prednisone (Sterapred)

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Useful in treatment of inflammatory and immune reactions. By reversing increased capillary permeability and suppressing PMN activity, may decrease inflammation. Dosage and length of treatment vary depending on specific diagnosis.

Questions

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Media Gallery

A man with neuralgic amyotrophy presenting with wasting of deltoids involving the right side more than the left.
A middle-aged man with (atypical) anterior horn cell disease presenting with wasting of the right quadriceps.
EMG at rest from the right quadriceps muscle of a patient with atypical anterior horn cell disease and isolated atrophy of the right quadriceps; EMG shows spontaneous activity.
EMG on voluntary effort from the right quadriceps muscle of a patient with atypical anterior horn disease and isolated atrophy of the right quadriceps; EMG shows motor unit potentials that exhibit prolonged duration and polyphasia.
EMG on maximal effort from the right quadriceps muscle of a patient with atypical anterior horn disease and isolated atrophy of the right quadriceps; EMG shows an impaired interference pattern.
Clinical photograph of a subject with monomelic amyotrophy showing wasting of left forearm. Note the characteristic feature of oblique atrophy, where a normal brachioradialis dominates the atrophied forearm.
Wasting of right forearm and both hand muscles in a patient with Hirayama Disease. Note the oblique atrophy of right forearm.
Wasting of small muscles of the hands in a patient with Hirayama Disease.
T2-weighted cervical spine MRI of a patient with Hirayama disease showing focal cord hyperintensity at C5-C6 level.
T2-weighted cervical spine MRI of the same patient during neck flexion showing anterior displacement of the posterior dural wall with flattening and compression of the cord against the bodies of the vertebrae with prominent dorsal epidural flow voids.