Graus et al reported criteria for making the diagnosis of paraneoplastic neurological syndrome (PNS). The authors are from the Paraneoplastic Neurological Syndrome Euronetwork.
(2) limbic encephalitis (targeting the hippocampus and amygdala)
(3) subacute cerebellar degeneration (targeting Purkinje cells)
(4) opsiclonus myoclonus
(5) subacute sensory neuropathy (targeting dorsal root ganglia)
(6) chronic gastrointestinal pseudo-obstruction (targeting the myenteric plexus)
(7) Lambert-Eaton myasthenic syndrome
(1) brainstem encephalitis (targeting the medulla oblongata)
(2) optic neuritis
(3) cancer-associated retinopathy
(4) melanoma-associated retinopathy
(5) stiff person syndrome
(6) necrotizing myelopathy
(7) motor neuron disease (targeting motor neurons in the anterior horn)
(8) Guillain-Barre syndrome
(9) brachial neuritis
(10) subacute or chronic sensorimotor neuropathies
(11) neuropathy with paraproteinemia
(12) neuropathy with vasculitis
(13) acute pandysautonomia
(14) myasthenia gravis
(15) acquired neuromyotonia
(16) acute necrotizing myopathy
Parameters for diagnosis:
(1) classical vs nonclassical
(2) cancer diagnosis present or absent or high risk
(3) onconeural antibodies
(4) clinical change after cancer therapy
Change with Therapy
present, well characterized
present, partially characterized
• The appearance of a paraneoplastic syndrome may precede the clinical diagnosis of cancer. For a definite PNS with classical syndrome this period can be 5 years.
• Improvement after cancer therapy cannot be explained by spontaneous remission or concomitant immunotherapy.
• Well-characterized antibodies are anti-Hu, anti-Yo, anti-CV2, anti-Ri, anti-Ma2 and anti-amphiphysin.
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Specialty: Neurology, Hematology Oncology