tailieunhanh - Chapter 097. Paraneoplastic Neurologic Syndromes (Part 8)

Paraneoplastic myelitis may present with upper or lower motor neuron symptoms, segmental myoclonus, and rigidity. This syndrome can appear as the presenting manifestation of encephalomyelitis and may be associated with SCLC and serum anti-Hu, anti-CV2/CRMP5, or anti-amphiphysin antibodies. Paraneoplastic myelopathy can also produce several syndromes characterized by prominent muscle stiffness and rigidity. The spectrum ranges from focal symptoms in one or several extremities (stiff-limb syndrome or stiffperson syndrome) to a disorder that also affects the brainstem (known as encephalomyelitis with rigidity) and likely has a different pathogenesis. Paraneoplastic Stiff-Person Syndrome This disorder is characterized by progressive muscle rigidity, stiffness, and painful spasms. | Chapter 097. Paraneoplastic Neurologic Syndromes Part 8 Paraneoplastic myelitis may present with upper or lower motor neuron symptoms segmental myoclonus and rigidity. This syndrome can appear as the presenting manifestation of encephalomyelitis and may be associated with SCLC and serum anti-Hu anti-CV2 CRMP5 or anti-amphiphysin antibodies. Paraneoplastic myelopathy can also produce several syndromes characterized by prominent muscle stiffness and rigidity. The spectrum ranges from focal symptoms in one or several extremities stiff-limb syndrome or stiffperson syndrome to a disorder that also affects the brainstem known as encephalomyelitis with rigidity and likely has a different pathogenesis. Paraneoplastic Stiff-Person Syndrome This disorder is characterized by progressive muscle rigidity stiffness and painful spasms triggered by auditory sensory or emotional stimuli. Rigidity mainly involves the lower trunk and legs but it can affect the upper extremities and neck. Symptoms improve with sleep and general anesthetics. Electrophysiologic studies demonstrate continuous motor unit activity. Antibodies associated with the stiff-person syndrome target proteins glutamic acid decarboxylase GAD amphiphysin involved in the function of inhibitory synapses utilizing y-aminobutyric acid GABA or glycine as neurotransmitters. Paraneoplastic stiff-person syndrome and amphiphysin antibodies are often related to breast cancer. By contrast antibodies to GAD may occur in some cancer patients but are much more frequently present in the nonparaneoplastic disorder. Stiff-Person Syndrome Treatment Optimal treatment of stiff-person syndrome requires therapy of the underlying tumor glucocorticoids and symptomatic use of drugs that enhance GABA-ergic transmission diazepam baclofen sodium valproate tiagabine vigabatrin . A benefit of IVIg has been demonstrated for the nonparaneoplastic disorder but remains to be established for the paraneoplastic syndrome. Paraneoplastic Sensory .