Paraneoplastic/Antibody-Mediated Syndromes

Question 1

Anti-NMDA receptor

Answer 1

ovarian teratoma; causes limbic encephalitis; psychosis, dyskinesias

Question 2

Anti-AMPA receptor

Answer 2

breast cancer, lung cancer, thymoma; causes limbic encephalitis, nystagmus

Question 3

Anti-GABA-B receptor

Answer 3

SCLC; causes limbic encephalitis, orolingual dyskinesias

Question 4

Anti-LGI1

Answer 4

SCLC, thymoma; causes limbic encephalitis

Question 5

Anti-CV2; CRMP5 IgG

Answer 5

SCLS, thymoma; causes limbic encephalitis; aphasia, chorea, ataxia, myelopathy, radiculopathy, neuropathy, LEMS

Question 6

Anti-Hu (ANNA1)

Answer 6

SCLC; causes limbic encephalitis; autonomic, peripheral, sensory neuropathy.

Question 7

Anti-Ma2

Answer 7

testicular cancer; causes limbic encephalitis, hypothalamic/pituitary disorder

Question 8

Anti-GAD65

Answer 8

SCLC, thymoma, renal cell, breast, colon; causes limbic encephalitis, stiff man syndrome, ataxia, ophthalmoplegia, parkinsonism, myelopathy

Question 9

Anti-Yo (PCA1)

Answer 9

ovarian, breast cancer; causes cerebellar ataxia, brainstem encephalitis, myelopathy, radiculopathy, peripheral neuropathy

Question 10

PCA-2

Answer 10

SCLC; limbic encephalitis, LEMS, peripheral and autonomic neuropathy

Question 11

Anti-Tr (PCA-Tr)

Answer 11

Hodgkin lymphoma; cerebellar ataxia

Question 12

Anti-Ri (ANNA2)

Answer 12

SCLC, breast cancer; dementia, limbic encephalitis, myelopathy, peripheral neuropathy

Question 13

Anti-Hu (ANNA1) in peds

Answer 13

Neuroblastoma in children with opsoclonus-myoclonus syndrome

Question 14

ANNA-3

Answer 14

lung, esophageal cancer; limbic encephalitis, myelopathy, peripheral neuropathy

Question 15

AGNA

Answer 15

SCLC; limbic encephalitis, LEMS, neuropathy

Question 16

Amphiphysin IgG

Answer 16

SCLC, breast cancer; limbic encephalitis, aphasia, Stiff person, myelopathy, neuropathy

Question 17

Anti-Ma1/Ma2

Answer 17

breast, colon, testicular cancer; limbic encephalitis, hypothalamic/pituitary disorder

Question 18

Anti-VGKC complex (LGI1 or CASPR2)

Answer 18

SCLC, thymoma, breast, prostate; limbic encephalitis, hypothalamic disorder, EPS, myoclonus, peripheral and autonomic neuropathy

Question 19

Anti-P/Q, N-type Calcium channel (VGCC)

Answer 19

SCLC, breast, gyn cancer; encephalopathy, myelopathy, neuropathy, LEMS

Question 20

Anti-NMO

Answer 20

thymoma; relapsing optic neuritis, transverse myelitis, encephalopathy

Question 21

Anti-neuronal ganglionic AChR

Answer 21

SCLC, thymoma, other AC; dysautonomia

Question 22

Anti-muscle AChR

Answer 22

thymoma, lung cancer; MG

Question 23

Anti-glycine receptor

Answer 23

Stiff man syndrome

Question 24

Anti-GluR5

Answer 24

Hodgkin lymphoma; limbic encephalitis

Question 25

Painless visual loss with night blindness, light induced glare, photosensitivity, peripheral ring like scotomas; normal fundoscopy

Answer 25

retinopathy associated with CRMP-5; Anti-recoverin

Question 26

Memory problems, personality change, focal seizures, altered mood. T2 hyperintensity in hippocampi

Answer 26

limbic encephalitis

Question 27

vision disturbance, tremulousness, disturbed sleep, screaming spells. Lightning like irregular movements of the eyes and limbs

Answer 27

opsoclonus-myoclonus; Anti-Hu (ANNA1)

Question 28

diplopia, vertigo, nausea, vomiting, ataxic dysarthria, nystagmus, gait, limb incoordination

Answer 28

Anti-Yo (PCA1); Anti-P/Q type calcium channel; Anti-Tr (PCA-Tr)

Question 29

Chorea

Answer 29

CRMP-5 IgG

Question 30

Parkinsonism, cranial neuropathies, disorders of eye movement and balance

Answer 30

brainstem encephalitis, Anti-Ma1/Ma2

Question 31

Jaw dystonia, paroxsymal laryngeal spasm

Answer 31

Anti-Ri (ANNA2)

Question 32

Myelopathy with T2 signal abnormalities on MRI and oligoclonal bands

Answer 32

CRMP-5 IgG, amphiphysin Ab, NMO, MS

Question 33

stiffness and spams of lumbar and proximal lower extremities, exaggerated startle response, loss of protective reflexes while falling

Answer 33

Stiff man syndrome, Ampiphysin IgG, Anti-GAD65, Anti-Glycine receptor; often associated with T1DM and autoimmune thyroid disease

Question 34

mydriasis, dry mouth, early satiety, nausea, small bowel pseudo-obstruction, constipation, orthostatic HoTN, heat intolerance, bladder symptoms

Answer 34

dysautonomia, ANNA-1; Anti-neuronal ganglionic AChR

Question 35

subacute progressive fatigue, weakness, worsens with exertion and improves with rest

Answer 35

LEMS; Anti-P/Q, N-type Calcium channel; Anti-CV2; CRMP5 IgG; PCA-2; AGNA

Question 36

Anti-MAG

Answer 36

Waldenstrom macroglobulinemia; peripheral neuropathy