paraneoplastic neurologic syndrome
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Etiology
- paraneoplastic cerebellar degeneration
- paraneoplastic limbic encephalitis
- paraneoplastic encephalomyelitis sensory neuropathy
- paraneoplastic sensory neuropathy
- paraneoplastic oposclonus-myoclonus
- Lambert-Eaton myasthenic syndrome
- stiff person syndrome
Pathology
- cortex & cerebellum (see entries)
- spinal cord
- necrotizing myelopathy
- mediator: unknown
- associated neoplasms:
- evolution over hours to days to weeks
- signs/symptoms
- paraplegia or quadriplegia with areflexia
- sensory loss
- bladder dysfunction
- pathology
- severe necrosis of spinal cord gray & white matter
- subacute motor neuronopathy
- mediator: unknown
- associated neoplasms: non-Hodgkin's lymphoma
- evolution over weeks or months
- signs/symptoms
- flaccid weakness & muscle atrophy
- legs affected more than arms
- pathology
- inflammation of ventral horns of spinal cord
- loss of anterior horn cells
- necrotizing myelopathy
- peripheral nerve
- acute demyelinating polyneuropathy (Guillain-Barre syndrome)
- mediator: unknown
- associated neoplasms: Hodgkin's disease
- evolution over hours to days
- signs/symptoms
- ascending paralysis
- areflexia
- ascending sensory loss (variable)
- pathology
- elevated cerebrospinal fluid protein
- segmental demyelination
- inflammation of peripheral nerves
- chronic demyelinating polyneuropathy
- mediator: anti-MAG (myelin-associated glycoprotein)
- associated neoplasms:
- lymphoma
- myeloma
- lung, breast, gastric cancer (rare)
- evolution over weeks to months
- signs/symptoms
- chronic progressive or relapsing weakness with sensory loss
- pathology
- elevated cerebrospinal fluid protein
- segmental demyelination
- inflammation of peripheral nerves
- neuropathy with paraproteinemia
- mediator: auto-antibodies
- associated neoplasm: myeloma
- evolution over weeks to months
- signs/symptoms
- pathology
- segmental demyelination
- inflammation of peripheral nerves
- subacute sensory neuronopathy
- mediator: unknown
- associated neoplasms: lung, especially small cell
- evolution over weeks to months
- signs/symptoms
- severe sensory loss with areflexia & ataxia
- paresthesias
- pain
- pathology
- inflammation & dorsal root degeneration in dorsal root ganglia -] secondary axonal loss
- sensorimotor neuropathy*
- mediator: auto-antibodies
- associated neoplasms:
- evolution over weeks to months
- signs/symptoms
- pathology
- axonopathy
- some segmental loss of myelin
- acute demyelinating polyneuropathy (Guillain-Barre syndrome)
* common
Clinical manifestations
- cognitive dysfunction
- short-term memory impairment
- mood & behavioral changes
- psychosis
- seizures, new-onset status epilepticus
- acute movement disorder
- acute progressive peripheral neuropathy[2]
Laboratory
- autoantibodies
- ANNA-1 (anti-Hu)
- patients with small cell lung carcinoma
- ANNA-2 (anti-Ri)
- patients with breast cancer, small cell lung carcinoma
- Purkinje cell antibody (PCA-1, anti-Yo)
- patients with breast cancer, ovarian cancer
- Purkinje cell antibody (anti-PCA-2)
- patients with small cell lung carcinoma
- MA (anti-Ma, anti-Ma1, anti-Ma2, anti-Ma4)
- patients with small cell lung carcinoma, breast cancer, testicular cancer
- collapsin response mediator protein (anti-CRMP-5)
- patients with small cell lung carcinoma, thymoma
- anti-amphiphysin (stiff man syndrome)
- patients with breast cancer, small cell lung carcinoma
- voltage-gated calcium channel (anti-VGCC)
- patients with small cell lung carcinoma
- voltage-gated potassium channel (anti-VGKC)
- patients with small cell lung carcinoma, thymoma
- anti PNMA3
- anti-NMDA receptor antibody
- ANNA-1 (anti-Hu)
- see ARUP consult[3]
Radiology
Management
- surgical resection
- immunotherapy[2]
More general terms
More specific terms
- GABA-A receptor antibody encephalitis
- Lambert-Eaton myasthenic syndrome (LEMS)
- paraneoplastic brainstem encephalitis; paraneoplastic rhombencephalitis
- paraneoplastic encephalomyelitis sensory neuropathy
- paraneoplastic limbic encephalitis
- paraneoplastic opsoclonus myoclonus ataxia; Kinsbourne syndrome; myoclonic encephalopathy of infants; dancing eyes-dancing feet syndrome
- paraneoplastic sensory neuropathy
- paraneoplastic subacute sensory neuronopathy
Additional terms
References
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 624
- ↑ 2.0 2.1 2.2 Medical Knowledge Self Assessment Program (MKSAP) 15, 18. American College of Physicians, Philadelphia 2009, 2018.
Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025 - ↑ 3.0 3.1 ARUP Consult: Paraneoplastic Neurological Syndromes The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/paraneoplastic-neurological-syndromes-and-associated-disorders
Paraneoplastic Neurological Syndromes Testing Algorithm https://arupconsult.com/algorithm/paraneoplastic-neurological-syndromes-testing-algorithm - ↑ Darnell RB, Posner JB. Paraneoplastic syndromes involving the nervous system. N Engl J Med. 2003 Oct 16;349(16):1543-54. PMID: https://pubmed.ncbi.nlm.nih.gov/14561798
- ↑ Flanagan EP. Paraneoplastic disorders of the nervous system. Continuum (Minneap Minn). 2020;26:1602-1628. PMID: https://pubmed.ncbi.nlm.nih.gov/33273174