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Chapter 097. Paraneoplastic Neurologic Syndromes (Part 6)

Paraneoplastic Encephalomyelitis and Focal Encephalitis The term encephalomyelitis describes an inflammatory process with multifocal involvement of the nervous system, including brain, brainstem, cerebellum, and spinal cord. It is often associated with dorsal root ganglia and autonomic dysfunction. For any given patient, the clinical manifestations are determined by the area or areas predominantly involved, but pathology almost always reveals abnormalities (inflammatory infiltrates, neuronal loss, gliosis) beyond the symptomatic regions. . | Chapter 097. Paraneoplastic Neurologic Syndromes Part 6 Paraneoplastic Encephalomyelitis and Focal Encephalitis The term encephalomyelitis describes an inflammatory process with multifocal involvement of the nervous system including brain brainstem cerebellum and spinal cord. It is often associated with dorsal root ganglia and autonomic dysfunction. For any given patient the clinical manifestations are determined by the area or areas predominantly involved but pathology almost always reveals abnormalities inflammatory infiltrates neuronal loss gliosis beyond the symptomatic regions. Several clinicopathologic syndromes may occur alone or in combination 1 cortical encephalitis which may present as epilepsia partialis continua 2 limbic encephalitis characterized by confusion depression agitation anxiety severe short-term memory deficits partial complex seizures and dementia the MRI usually shows unilateral or bilateral medial temporal lobe abnormalities best seen with T2 and fluid-attenuated inversion recovery sequences and occasionally enhancing with gadolinium 3 brainstem encephalitis resulting in eye movement disorders nystagmus opsoclonus supranuclear or nuclear paresis cranial nerve paresis dysarthria dysphagia and central autonomic dysfunction 4 cerebellar gait and limb ataxia 5 myelitis which may cause lower or upper motor neuron symptoms myoclonus muscle rigidity and spasms and 6 autonomic dysfunction as a result of involvement of the neuraxis at multiple levels including hypothalamus brainstem and autonomic nerves see autonomic neuropathy . Cardiac arrhythmias postural hypotension or central hypoventilation are frequent causes of death in patients with encephalomyelitis. Paraneoplastic encephalomyelitis and focal encephalitis are usually associated with SCLC but many other cancers have also been reported. Patients with SCLC and these syndromes usually have anti-Hu antibodies in serum and CSF. Anti-CV2 CRMP5 antibodies occur less frequently some of these .