Motor symptoms in nonparaneoplastic CNS disorders associated with neural antibodies

Motor symptoms are common, and sometimes predominant, in almost all nonparaneoplastic CNS disorders associated with neural antibodies. These CNS disorders can be classified into five groups: (1) Autoimmune encephalitis with antibodies against synaptic receptors, (2) cerebellar ataxias associated with neuronal antibodies that mostly target intracellular antigens. (3) Stiff-person syndrome and progressive encephalomyelitis with rigidity and myoclonus which have antibodies against glutamic acid decarboxylase and glycine receptor, respectively. Both diseases have in common the presence of predominant muscle stiffness and rigidity. (4) Three diseases associated with glial antibodies. Two present motor symptoms mainly due to the involvement of the spinal cord: neuromyelitis optica spectrum disorders with aquaporin-4 antibodies and myelin oligodendrocyte glycoprotein antibody-associated disease. The third disorder is the meningoencephalitis associated with glial fibrillar acidic protein antibodies which frequently also presents a myelopathy. (5) Two antibody-related diseases which are characterized by prominent sleep dysfunction: anti-IgLON5 disease, a disorder that frequently presents a variety of movement disorders, and Morvan syndrome associated with contactin-associated protein-like 2 antibodies and clinical manifestations of peripheral nerve hyperexcitability. In this chapter, we describe the main clinical features of these five groups with particular emphasis on the presence, frequency, and types of motor symptoms.