Chapter 097. Paraneoplastic Neurologic Syndromes (Part 5)

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Specific Paraneoplastic Neurologic Syndromes (Table 97-3) Table 97-3 Antibody-Associated Paraneoplastic and Nonparaneoplastic Syndromesa Antibodies Paraneoplastic Syndrome Frequent t Infrequen stic Nonparaneopla Limbic encephalitis Ma2, Hu, CV2/CRMP5, anti-NR1/NR2 of NMDA receptor Tr, VGKC VGKC Cerebellar degeneration Yo, Tr, mGluR1; Gliadin, GAD P/Q VGCC, Hu, MAZ Zic, CV2/CRMP5, Ma1-2 Ri, Hypothalamic, brainstem encephalitis Ma2, Hu P5 CV2/CRM Encephalomye litis Hu, Zic P5, CV2/CRM Ri, amphiphysin Chorea P5 CV2/CRM Opsoclonusmyoclonus Ri Yo, Hu, Ma2, Stiff-person syndrome sin Amphiphy Ri Gephyrin, GAD PNH (neuromyotonia) VGKC VGKC Myasthenia AChR AChR, MuSK gravis LEMS VGCC P/Q-type MysB P/Q-type VGCC Sensory neuronopathy Hu Axonal sensorimotor neuropathy ...

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Chapter 097. Paraneoplastic Neurologic Syndromes (Part 5) Specific Paraneoplastic Neurologic Syndromes (Table 97-3) Table 97-3 Antibody-Associated Paraneoplastic and Nonparaneoplastic Syndromesa Antibodies Paraneoplastic
Syndrome Frequent Infrequen Nonparaneopla t stic Limbic Ma2, Hu, Tr, VGKC VGKC encephalitis CV2/CRMP5, anti-NR1/NR2 of NMDA receptor Cerebellar Yo, Tr, mGluR1; Gliadin, GAD degeneration P/Q VGCC, Hu, MAZ Zic, Ri, CV2/CRMP5, Ma1-2 Hypothalamic, Ma2, Hu CV2/CRM brainstem P5 encephalitis
Encephalomye Hu, Zic CV2/CRM litis P5, Ri, amphiphysin Chorea CV2/CRM P5 Opsoclonus- Ri Hu, Ma2, myoclonus Yo, Stiff-person Amphiphy Gephyrin, GAD syndrome sin Ri PNH VGKC VGKC (neuromyotonia) Myasthenia AChR AChR, MuSK
gravis LEMS P/Q-type MysB P/Q-type VGCC VGCC Sensory Hu neuronopathy Axonal Hu, Monoclonal sensorimotor CV2/CRMP5 gammopathy (M neuropathy protein)b Autonomic Hu CV2/CRM Ganglionic neuropathy P5, ganglionic AChR AChR Predominant MAG, MAG, sensory ganglioside ganglioside antibodies, demyelinating antibodies: often often present with
neuropathy present with MGUS Waldenström's macroglobulinem ia Paraneoplastic Recoverin Tubby-like Anti-enolase retinopathy (CAR), anti- protein 1, PNR bipolar cell antibodies (MAR), anti- enolase a Antibodies have been validated by more than one laboratory and/or the protein sequence of the target antigen is known. b The M protein usually does not have specific antibody activity. Note: Italics indicate that commercial testing for these antibodies is not available. PNH, peripheral nerve hyperexcitability; CAR, cancer-associated retinopathy; MAR, melanoma-associated retinopathy; PNR, photoreceptor- specific nuclear receptor; MGUS, monoclonal gammopathy of uncertain significance; VGKC, voltage-gated potassium channel; GAD, glutamic acid
decarboxylase; AChR, acetylcholine receptor; LEMS, Lambert-Eaton myasthenic syndrome; VGCC, voltage-gated calcium channel; MAG, myelin-associated glycoprotein; NMDA, N-methyl-D-aspartate.