Primary lateral sclerosis
Definition / general:
- Rarely caused by breast cancer
- 50% develop lower motor neuron signs eventually
CNS nontumor
General
Paraneoplastic syndromes
Author: Nat Pernick, M.D.
Last author update: 1 January 2006
Last staff update: 10 January 2024
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: "Paraneoplastic syndromes" CNS
Table of Contents
Definition / general | Cerebellar degeneration | Lambert-Eaton myasthenic syndrome | Limbic encephalitis | Myelopathies | Opsoclonus / myoclonus (spontaneous chaotic eye movements) | Peripheral neuropathy | Primary lateral sclerosis | Retinal degeneration | Stiff man syndrome | Subacute sensory neuropathy | Subacute motor neuronopathyCite this page: Pernick N. Paraneoplastic syndromes. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorparaneoplastic.html. Accessed November 29th, 2024.
Definition / general
- Nervous system dysfunction associated with cancer but without direct tumor invasion, infection or vascular complications of neural tissue
- May precede clinical recognition of tumors
- Occurs in < 1% of cancer patients; usually small cell carcinoma of lung, breast cancer or ovarian cancer
- Often due to autoantibodies
- May respond to treatment of the underlying tumors
Cerebellar degeneration
Definition / general:
- Destruction of Purkinje cells (antibody mediated) with perivascular lymphocytes
- Causes progressive bilateral leg and arm ataxia, dysarthria, variable vertigo and diplopia
- Associated with breast, gynecologic, Hodgkin lymphoma and small cell carcinoma of lung
- Often associated with anti-Yo antibody in serum or CSF
- References: Brain 2003;126:1409
Lambert-Eaton myasthenic syndrome
Definition / general:
- Destruction of neuromuscular junction (presynaptic terminals) by small cell lung cancer; due to IgG antibody
Limbic encephalitis
Myelopathies
Definition / general:
- Necrotizing myelopathy due to leukemia, lymphoma and lung carcinoma
- Myelitis due to anti-Hu antibody associated with small cell lung carcinoma
Opsoclonus / myoclonus (spontaneous chaotic eye movements)
Definition / general:
- Affects brain stem but site unknown
- Due to breast and small cell lung cancer; also neuroblastoma
- May be associated with anti-Ri autoantibody
Peripheral neuropathy
Definition / general:
- Most common paraneoplastic syndrome
- May be associated with anti-Hu antibody in lung cancer patients
Retinal degeneration
Definition / general:
- Destruction of photoreceptors
- Due to small cell carcinoma and gynecologic tumors
Stiff man syndrome
Definition / general:
- Destruction of spinal interneurons by amphiphysin antibody
- Usually breast cancer, also lung cancer and thymoma
Subacute sensory neuropathy
Definition / general:
- May occur with limbic encephalitis
- Loss of sensory neurons from dorsal root ganglia, associated with inflammation
- Due to small cell lung cancer
- References: J Neurol Neurosurg Psychiatry 1997;63:133 (paraneoplastic encephalomyelitis)
Subacute motor neuronopathy
Definition / general:
Case reports:
Differential diagnosis:
- Degeneration of anterior horn cells
- Rare, painless lower motor neuron weakness of extremities
- Associated with lymphoma
- References: J Neurol Neurosurg Psychiatry 2004;75:ii43, eMedicine: Metabolic Neuropathy Clinical Presentation
Case reports:
- Neuropathy associated with lymphoma and IgM antibodies against disialosyl residues (Muscle Nerve 2005;32:216)
Differential diagnosis:
- Meningeal carcinomatosis
- Metabolic brain disease
- Progressive multifocal leukoencephalopathy
