paraneoplastic disorders Flashcards
what are paraneoplastic disorders?
a neurological disorder which are the indirect effect of the tumour
ie in which there is no direct involvement of the tumour or its metastases or its treatment
generally thought of as a immune-mediated condition
what two diagnostic criteria are there for paraneoplastic syndromes?
definite paraneopastic syndrome
possible paraneoplastic syndrome
what are the four diagnostic criteria for a definite paraneoplastic syndrome?
1) A classical syndrome with proven cancer, with or without paraneoplastic antibodies.
2) A non-classical syndrome with proven cancer, that resolves or significantly improves after cancer treatment without concomitant immunotherapy, provided the syndrome is not susceptible to spontaneous remission.
3) A non-classical syndrome with proven cancer, with onconeural antibodies (well characterised or not)
4) A neurological syndrome (classical or not) with no cancer identified but with well characterised onconeural antibodies (anti-Hu, Yo, CV2, Ri, Ma2, or amphiphysin). L
what are the three diagnostic criteria for possible paraneoplastic syndrome?
1) A classical syndrome, no cancer identified, and no onconeural antibodies, but at high risk of having an underlying tumour (e.g. smoker or age >50).
2) A neurological syndrome (classical or not), no cancer identified, with partially characterised onconeural antibodies.
3) A non-classical syndrome, cancer proven, but no onconeural antibodies. Note: the cancer may develop within two years of the neurological diagnosis.
what are the classic paraneoplastic syndromes?
encephalomyeltis
subacute cerebellar degeneration
opsoclonus-myoclonus
lambert-eaton myasthenic syndrome
dermatomyositis
motor neurone disorders
myasthenia gravis
what is encephalomyelitis
a general term for inflammation of of the brain and spinal cord
often multifocal involvement
Used as a term principally when clinical dysfunction is at multiple levels
includes lymbic encephalitis, brainstem encephalitis, subacute sensory neuronopathy, autonomic neuropathy
what is the diagnostic criteria for limbic encephalitis?
recent onset (<5 yrs)
at least one of:
disturbances in episodic memory
temporal lobe seizures
affective disturbance
plus one of the following four:
tumour
autoantibodies (Hu Ma2m CV2/CRMP5 etc)
brain MRI (otherwise unxplained teporomedial T2/ flair signal increase
histopathology (lymphocytyic-micronodular encephalitis)
what are the four commonest symptoms of limbic encephalitis
loss of short term memory (84%)
seizures (50%)
acute cunfusional state (46%)
psychiatric abnormalities (42%)
what are three common types of limbic encephalitis?
what are their common presenations?
VGKC-related Ab LE
prodrome of dystonic seizures (20%), hyponatraemia
NMDAr Ab related LE
movement disorders & early psychiatric features
GABAb-Ab related LE
early prominent seizures
what antibodies are usually associated with paraneoplastic disorders?
Hu
Ma2
CRMP5
Amphiphysin (rare)
what antibodies are usually associated with non-paraneoplastic disorders?
NMDAr
VGKV & associated proteins
GABAb
AMPAr
{GAD]
what tests can be performed for paraneoplastic antibodies?
cell based assays ie oxford & eurimmum slides
immunoblot
what antibodies can be tested for using cell based assays?
NMDA
VGKC
GABAB
what antibodies can be tested for with immunoblots?
HuD Yo Ri CRMP5 amphiphysin MA1 MA2
if a test for Anti-Hu is present what does this indicate
highly likely cancer (98%) is presenting with PNS
