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paraneoplastic neurologic syndrome

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: 1) cortex & cerebellum (see entries) - photoreceptor retinal degeneration - limbic encephalitis - brainstem encephalitis - subacute cerebellar degeneration - opsoclonus-myoclonus 2) spinal cord a) necrotizing myelopathy - mediator: unknown - associated neoplasms: - small cell lung cancer - lymphoma - 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 b) 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 3) peripheral nerve a) 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 b) 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 c) neuropathy with paraproteinemia - mediator: auto-antibodies - associated neoplasm: myeloma - evolution over weeks to months - signs/symptoms - chronic; may be predominantly sensory or motor - pathology - segmental demyelination - inflammation of peripheral nerves d) 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 e) sensorimotor neuropathy* - mediator: auto-antibodies - associated neoplasms: - lung cancer - GI cancer - breast cancer - evolution over weeks to months - signs/symptoms - distal motor & sensory loss - pathology - axonopathy - some segmental loss of myelin * common 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 - see ARUP consult [3] Management: - surgical resection - immunotherapy [2]

Interactions

disease interactions

Related

paraneoplastic photoreceptor retinal degeneration; paraneoplastic retinopathy stiff-man syndrome

Specific

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

General

paraneoplastic syndrome

References

  1. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 624
  2. Medical Knowledge Self Assessment Program (MKSAP) 15, 18. American College of Physicians, Philadelphia 2009, 2018.
  3. 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
  4. Darnell RB, Posner JB. Paraneoplastic syndromes involving the nervous system. N Engl J Med. 2003 Oct 16;349(16):1543-54. PMID: 14561798