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Chapter 097. Paraneoplastic Neurologic Syndromes (Part 4)

PND of the Central Nervous System and Dorsal Root Ganglia When symptoms involve brain, spinal cord, or dorsal root ganglia, the suspicion of PND is usually based on a combination of clinical, radiologic, and CSF findings. In these cases, a biopsy of the affected tissue is often difficult to obtain, and although useful to rule out other disorders (e.g., metastasis, infection), neuropathologic findings are not specific for PND. Furthermore, there are no specific radiologic or electrophysiologic tests that are diagnostic of PND. . | Chapter 097. Paraneoplastic Neurologic Syndromes Part 4 PND of the Central Nervous System and Dorsal Root Ganglia When symptoms involve brain spinal cord or dorsal root ganglia the suspicion of PND is usually based on a combination of clinical radiologic and CSF findings. In these cases a biopsy of the affected tissue is often difficult to obtain and although useful to rule out other disorders e.g. metastasis infection neuropathologic findings are not specific for PND. Furthermore there are no specific radiologic or electrophysiologic tests that are diagnostic of PND. The presence of antineuronal antibodies Table 97-2 may help in the diagnosis with the following caveats 1 antibodies are detected in only 60-70 of PNDs of the CNS 2 antibodies may be present in both the serum and CSF but in some patients only the CSF is positive especially with antibodies to Tr and Ma proteins 3 antibodies usually at low titer are present in a variable proportion of cancer patients without PND 4 there is an imperfect correlation between antibody titers and the course of the neurologic disorder 5 several antibodies may associate with a similar syndrome with the antibody specificity often correlating with the tumor type e.g. cerebellar degeneration is associated with anti-Tr antibodies if the tumor is Hodgkin s disease but with anti-Yo antibodies if the tumor is ovarian or breast cancer and 6 several antibodies may be present in the serum or CSF of the same patient e.g. anti-Hu and anti-CV2 CRMP5 . MRI and CSF studies are important to rule out neurologic complications due to the direct spread of cancer particularly metastatic and leptomeningeal disease. In most PNDs the MRI findings are nonspecific. Paraneoplastic limbic encephalitis is usually associated with characteristic MRI abnormalities in the mesial temporal lobes see below but similar findings can occur with other disorders e.g. nonparaneoplastic limbic encephalitis with antibodies to VGKC human herpesvirus HHV 6 encephalitis .