Paraneoplastic syndrome is a rare neurologic disorder caused not by the direct invasion or metastasis of cancer, but by the remote effects of cancer. The central- and peripheral-nervous system or neuromuscular junction area were involved in this syndrome. The pathogenesis was thought as the autoimmune disease, the result of an immunologic response to cancer and to cross-react with self-cells of the nervous system or of the neuromuscular junction, causing neuronal dam-age. Specific forms of this syndrome are often associated with specific paraneoplastic autoantibodies and cancer. The onset of neurological symptoms and detection of these antibodies often precede the diagnosis of the cancer; therefore, detection of these antibodies greatly assists the diagnosis of this syndrome and prompts investigations for the underly-ing cancer. Some paraneoplastic central neurological syndromes, such as cerebellar degeneration, limbic encephalitis, and necrotizing myelitis, were not improved by putative pathogenic autoantibodies, or by immunosuppresant or tumor removal, inspite of improvement in other peripheral neurologic syndrome, Lambert-Eaton myasthenic syndrome, neu-romyotonia, and Stiff-man syndrome. A more detailed understanding of the relationship between the cancer and the neural involvement from the molecular biological standpoint may lead to rational tumor therapy and elucidation of the mechanism of neural death. Here, major clinical forms with well-known antineuronal antibodies and specific cancers are reviewed.
Antibodies ; Autoantibodies ; Autoimmune Diseases ; Diagnosis ; Lambert-Eaton Myasthenic Syndrome ; Limbic Encephalitis ; Myelitis, Transverse ; Neoplasm Metastasis ; Nervous System ; Nervous System Diseases ; Neuromuscular Junction ; Neurons ; Paraneoplastic Syndromes* ; Small Cell Lung Carcinoma ; Stiff-Person Syndrome

The dizziness associated with paraneoplastic neurologic syndrome is hard to diagnose clinically because the prevalence of disease is rare, and radiologic and serologic examination result may come out normal. Opsoclonus-myoclonus symdrome is a representative of classical paraneoplastic neurologic syndromes. In this paper, we report 2 cases of paraneoplastic neurologic syndromes with negative serologic auto-antibody test and no brain lesion on MRI. Both cases were eventually diagnosed through PET. Patients with opsoclonus-myoclonus type nystagmus should be evaluated for paraneoplastic neurologic syndrome even if their radiologic and serologic findings are normal.
Brain ; Dizziness ; Humans ; Magnetic Resonance Imaging ; Myoclonus ; Ocular Motility Disorders ; Opsoclonus-Myoclonus Syndrome ; Paraneoplastic Syndromes, Nervous System ; Prevalence

Adult ; Female ; Humans ; Limbic Encephalitis ; etiology ; Ovarian Neoplasms ; complications ; Paraneoplastic Syndromes, Nervous System ; etiology ; Receptors, N-Methyl-D-Aspartate ; immunology ; Seizures ; etiology ; Teratoma ; complications

Sensory neuropathy is characterized by pure sensory dysfunction, ultimately evolving into areflexia and severe sensory ataxia in spite of the preservation of the other functions in the nervous system such as motor function. This disorder is known to be commonly found in cancer patients as a paraneoplastic syndrome, but nowadays many reports are made that sensory neuropathy is not merely related to malignancy but also with autoimmune disorders such as Sjogren's syndrome or polyclonal gammopathy, etc. Hence we report two patients who have been diagnosed as chronic idiopathic ataxic sensory neuropathy through electrophysiological and histopathological studies without any evidence of cancer or any other known cause.
Ataxia ; Humans ; Nervous System ; Paraneoplastic Syndromes ; Sjogren's Syndrome

Acupuncture Therapy ; Aged ; Female ; Humans ; Nervous System Neoplasms ; therapy ; Paraneoplastic Syndromes, Nervous System ; therapy

No abstract available.
Lambert-Eaton Myasthenic Syndrome*

Paraneoplastic encephalitis presenting with partial clonic seizure is rare. We report a 57-year-old man with clonic seizure in his left arm. He had past medical history of recurrent thymoma and thymomectomy. Magnetic resonance imaging showed multiple brain lesions, but none of them were congruent with the partial seizure. His symptoms and brain lesions resolved after steroid therapy. This is the first reported case extralimbic encephalitis presenting as partial seizure with invasive thymoma in Korea.
Arm ; Brain ; Encephalitis* ; Humans ; Korea ; Magnetic Resonance Imaging ; Middle Aged ; Nervous System ; Paraneoplastic Syndromes ; Seizures* ; Thymoma*

Paraneoplastic encephalitis presenting with partial clonic seizure is rare. We report a 57-year-old man with clonic seizure in his left arm. He had past medical history of recurrent thymoma and thymomectomy. Magnetic resonance imaging showed multiple brain lesions, but none of them were congruent with the partial seizure. His symptoms and brain lesions resolved after steroid therapy. This is the first reported case extralimbic encephalitis presenting as partial seizure with invasive thymoma in Korea.
Arm ; Brain ; Encephalitis* ; Humans ; Korea ; Magnetic Resonance Imaging ; Middle Aged ; Nervous System ; Paraneoplastic Syndromes ; Seizures* ; Thymoma*

Orofacial granulomatosis is a rare granulomatous inflammatory disease, characterized by recurrent orofacial swelling. Infectious, genetic, and immunologic etiologies are suggested, but not fully understood. Herein, we report a case of synchronous orofacial granulomatosis with brain cavernous hemangioma in a 44-year-old female patient, which may be considered paraneoplastic syndrome.
Adult ; Brain ; Female ; Granulomatosis, Orofacial ; Hemangioma, Cavernous ; Hemangioma, Cavernous, Central Nervous System ; Humans ; Paraneoplastic Syndromes

Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Motor Neuron Disease ; diagnosis ; Paraneoplastic Syndromes, Nervous System ; diagnosis