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Flashcards in paraneoplastic disorders Deck (17):
1

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

2

what two diagnostic criteria are there for paraneoplastic syndromes?

definite paraneopastic syndrome

possible paraneoplastic syndrome

3

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

4

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.

5

what are the classic paraneoplastic syndromes?

encephalomyeltis

subacute cerebellar degeneration

opsoclonus-myoclonus

lambert-eaton myasthenic syndrome

dermatomyositis

motor neurone disorders

myasthenia gravis

6

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

7

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)

8

what are the four commonest symptoms of limbic encephalitis

loss of short term memory (84%)

seizures (50%)

acute cunfusional state (46%)

psychiatric abnormalities (42%)

9

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

10

what antibodies are usually associated with paraneoplastic disorders?

Hu
Ma2
CRMP5
Amphiphysin (rare)

11

what antibodies are usually associated with non-paraneoplastic disorders?

NMDAr

VGKV & associated proteins

GABAb

AMPAr

{GAD]

12

what tests can be performed for paraneoplastic antibodies?

cell based assays ie oxford & eurimmum slides

immunoblot


13

what antibodies can be tested for using cell based assays?

NMDA
VGKC
GABAB

14

what antibodies can be tested for with immunoblots?

HuD
Yo
Ri
CRMP5
amphiphysin
MA1
MA2

15

if a test for Anti-Hu is present what does this indicate

highly likely cancer (98%) is presenting with PNS

16

what tests should be done when someone presents with a limbic encephalitis that is suspected of PNS?

Send off blood for anitbody screen, arrange for immunoblot

arrange CT to check for tumours

17

what is the prognosis for PNS?

cancer develops in most patients (60%) after the PNS or at the same time

treatment for cancer helps LE symptoms in >2/3

early removal of teratoma or early immunotherapy results in better outcome