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Paraneoplastic Neurologic Syndromes Flashcards

1
Q

tumors associated with anti Yo

A

Breast

ovary

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1
Q

Tumors associated with Anti-AMPAR

A

SCLC, thymoma, breast

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1
Q

Encephalitis with N-methyl-d-aspartate (NMDA) receptor antibodies is common in

A

young women and children

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1
Q

Peripheral neuropathy in sclerotic myeloma

A

Sensorimotor with predominantly motor deficits

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2
Q

Tumors associated with Anti-VGKC- related proteins

A

SCLC

thymoma

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3
Q

Anti-Tr

A

Cerebellar degeneration

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4
Q

anti AMPA encephalitis is common in

A

middle aged women

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5
Q

Rx of vasculitis of nerve and muscle

A

glucocorticoids

cyclophosphamide

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6
Q

Diagnostically useful antibodies in paraneoplastic neuropathies

A

anti Hu

anti-CV2/CRMP5

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7
Q

opsoclonus is frequently associated with

A

myoclonus

ataxia

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8
Q

Tumor associated with anti GAD

A

Thymoma

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9
Q

neuromyotonia

A

spontaneous and continuous muscle fiber activity of peripheral nerve origin

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10
Q

Classic Paraneoplastic Neurologic Syndromes

A

Encephalomyelitis

Limbic encephalitis

Cerebellar degeneration (adults)

Opsoclonus-myoclonus

Subacute sensory neuronopathy

Gastrointestinal paresis or pseudo-obstruction

Dermatomyositis (adults)

Lambert-Eaton myasthenic syndrome

Cancer or melanoma associated retinopathy

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10
Q

Jeopardy

anti Hu

anti Ri

Anti-CV2/CRMP5
Anti-amphiphysin

Recoverin, bipolar cell antibodies

A

antibodies associated with SCLC

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11
Q

presentation of anti AMPA encephalitis

A

acute limbic dysfunction

prominent psychiatric symptoms

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11
Q

Rx of opsoclonus-myoclonus associated with neuroblastoma

A

glucocorticoids

adrenocorticotropic hormone (ACTH)

plasma exchange

IVIg

rituximab

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11
Q

Paraneoplastic autonomic neuropathy usually develops as a component of

A

LEMS

Encephalomyelitis

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12
Q

Paraneoplastic neurologic dysfunctions that respond to treatment

A

Vasculitis of nerve and muscle

plasmacytoma,sclerotic myeloma,waldenstrom macroglobulinemia

Demyelinating peripheral neuropathies

Paraneoplastic Opsoclonus-Myoclonus Syndrome

Encephalitides with Antibodies to Cell-Surface or Synaptic Proteins

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14
Q

Tumors associated with anti amphiphysin

A

Breast, SCLC

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14
Q

Malignant monoclonal gammopathies

A

multiple myeloma and sclerotic myeloma associated with IgG or IgA monoclonal proteins

Waldenström’s macroglobulinemia, B cell lymphoma, and chronic B cell lymphocytic leukemia associated with IgM monoclonal proteins

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15
Q

Anti-VGKC- related proteins(LGI1, Caspr2)

A

Neuromyotonia, limbic encephalitis

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15
Q

Anti-AMPAR

A

Limbic encephalitis with relapses

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16
Q

Prodrome of paraneoplastic cerebellar degeneration

A

dizziness

oscillopsia

double vision

nausea,vomiting

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17
Q

Paraneoplastic Stiff-Person Syndrome

A

progressive muscle rigidity, stiffness, and painful spasms triggered by auditory, sensory, or emotional stimuli

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17
Q

IVIg in stiff person syndrome

A

benefit in non paraneoplastic disorder

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18
Q

Antibodies that may be found in issac syndrome

A

Caspr2-related proteins

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19
Q

rigidity in Paraneoplastic Stiff-Person Syndrome mainly affects

A

lower trunk and legs

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20
Q

Jeopardy

Encephalomyelitis, chorea, optic neuritis, uveitis, peripheral neuropathy

A

Anti-CV2/CRMP5

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21
Q

Jeopardy

disorder of eye movement characterized by involuntary, chaotic saccades that occur in all directions of gaze

A

opsoclonus

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21
Q

Tumors associted with Paraneoplastic Opsoclonus-Myoclonus Syndrome

A

Adults: lung,breast

Children: neuroblastoma

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21
Q

Tumors associated with neuromyotonia

A

Usually non paraneoplastic

Thymoma

SCLC

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22
Q

Jeopardy

Eye movement disorders:

nystagmus, opsoclonus, supranuclear or nuclear paresis

cranial nerve paresis

dysarthria

dysphagia

central autonomic dysfunction

A

Features of brainstem encephalitis

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23
Q

tumors associated with anti Ri

A

Breast, gynecologic, SCLC

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23
Q

Tumor associated with Anti-NMDAR

A

teratoma

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24
Q

Anti-Ma proteins

A

Limbic, hypothalamic, brainstem encephalitis

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24
Q

IVIg,glucocorticoids and plasma exchange are useful in paraneoplastic peripheral neuropathies if

A

demyelination features predominate

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24
Q

Peripheral neuropathy in sclerotic myeloma resembles

A

CIDP

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26
Q

Jeopardy

Cerebellar degeneration

A

anti Yo

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27
Q

Jeopardy

Limbic encephalitis with relapses

A

Anti-AMPAR

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28
Q

Antibodies pathogenic for LEMS

A

anti VGCC

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28
Q

Jeopardy

disinhibition of the fastigial nucleus of the cerebellum

A

pathology in Opsoclonus-myoclonus

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29
Q

Tumors associated with Anti-GABABR

A

SCLC, neuroendocrine

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29
Q

Anti Glycine receptor

A

Encephalomyelitis with rigidity, stiff-person syndrome

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29
Q

Presentation of myelitis

A

UMN or LMN signs

myoclonus

muscle rigidity and spasms

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30
Q

Presentation of vasculitis of nerve and muscle

A

painful symmetric or asymmetric distal axonal sensorimotor neuropathy with variable proximal weakness

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31
Q

Jeopardy

UMN or LMN signs

myoclonus

muscle rigidity and spasms

A

Presentation of myelitis

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31
Q

Acute necrotising myopathy

A

myalgias

rapid progression of weakness involving the extremities and the pharyngeal and respiratory muscles

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32
Q

Jeopardy

Cerebellar degeneration

A

Anti-Tr

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34
Q

Jeopardy

Anti-AChR (muscle)

A

Myasthenia gravis

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35
Q

Encephalitis with antibodies to voltage-gated potassium channels (VGKC)-related proteins is common in

A

Men

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36
Q

Distinct characteristics of Encephalitides with Antibodies to Cell-Surface or Synaptic Proteins

A
  1. May or may not be associated with tumor
  2. Some occur in young individuals
  3. Respond to immunotherapy
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38
Q

Anti-AChR (neuronal)

A

Autonomic neuropathy

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38
Q

Does Paraneoplastic Opsoclonus-Myoclonus Syndrome respond to Rx?

A

yes

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39
Q

Diagnosis of CNS PNDs

A

Clinical

radiological

CSF

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40
Q

Tumor associated with Anti-AChR (muscle)

A

Thymoma

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40
Q

morvan syndrome

A

neuromyotonia+limbic encephalitis

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41
Q

________ % of anti AMPA associated encephalitis have a tumor

42
Q

clinical presentation of neuromyotonia

A

cramps

muscle twitching (fasciculations or myokymia)

stiffness

delayed muscle relaxation (pseudomyotonia)

spontaneous or evoked carpal or pedal spasms

paresthesia and hyperhidrosis

43
Q

cortical encephalitis may present as

A

epilepsia partialis continua

44
Q

Paraneoplastic encephalomyelitis and focal encephalitis are usually associated with

45
Q

anti NMDA encephalitis is misdiagnosed as

A

viral or idiopathic encephalitis

neuroleptic malignant syndrome

encephalitis lethargica

46
Q

Rx of neuromyotonia

A

Phenytoin

Carbamazepine

plasma exchange

46
Q

Clinical manifestations of paraneoplastic autonomic neuropathy

A

Pseudoobstruction

cardiac arrythmias

postural hypotension

abnormal pupillary responses

dry mouth

anhidrosis

erectile dysfunction

problems in sphincter control

47
Q

Anti-GABABR

A

Limbic encephalitis, seizures

49
Q

Anti-CV2/CRMP5

A

Encephalomyelitis, chorea, optic neuritis, uveitis, peripheral neuropathy

50
Q

Jeopardy

Limbic encephalitis, seizures

A

Anti-GABABR

51
Q

Tumor commonly associated with cancer associated retinopathy

52
Q

presentation of paraneoplastic myelitis

A

UMN or LMN symptoms

segmental myoclonus

rigidity

53
Q

Imaging for detection of tumors

A

CT

CT+PET

USG,MRI for Germ Cell Tumor of testis and teratoma of ovary

54
Q
A

Limbic encephalitis

55
Q

Peripheral nerve hyperexcitability

A

issac syndrome

57
Q

Jeopardy

Encephalomyelitis with rigidity, stiff-person syndrome

A

Anti Glycine receptor

57
Q

Screening after diagnosis of LEMS

A

Chest and abdomen CT,PET

If negative,periodic screening for atleast 3 years

58
Q

Jeopardy

Limbic, hypothalamic, brainstem encephalitis

A

Anti-Ma proteins

59
Q

In _______ % of pts the Paraneoplastic Neurologic Syndromes precede cancer diagnosis

60
Q

Encephalitis with antibodies to voltage-gated potassium channels (VGKC)-related proteins frequently presents as

A

memory loss

seizures

hyponatremia

sleep and autonomic dysfunction

61
Q

Response of paraneoplastic encephalomyelitis to treatment

63
Q

Jeopardy

Stiff-person syndrome, encephalomyelitis

A

Anti-amphiphysin

64
Q

Jeopardy

Thymoma

A

Tumor associated with anti GAD

65
Q

Nystagmus in paraneoplastic cerebellar degeneration

A

downbeating

67
Q

Components of paraneoplastic encephalomyelitis

A

cortical encephalitis

limbic encephalitis

brainstem encephalitis

cerebellar gait and limb ataxia

myelitis

autonomic dysfunction

68
Q

antibodies associated with prominent or pure cerebellar degeneration

A

anti Yo

anti Tr

69
Q

Tumor associated with Anti-VGCC

70
Q

pathology in Opsoclonus-myoclonus

A

disinhibition of the fastigial nucleus of the cerebellum

72
Q

Jeopardy

Breast

ovary

A

tumors associated with anti Yo

73
Q

Anti-Ri

A

Cerebellar degeneration

opsoclonus

73
Q

Diff btw Paraneoplastic Peripheral Neuropathies that develop in early stage and late stage cancers

A

Late stage:

mild to moderate sensorimotor deficits

axonal degeneration of unclear etiology

masked by concurrent neurotoxicity from chemotherapy

Early stage:

rapid progression

relapsing and remitting course

axonal loss or demyelination

74
Q

Tumor associated with Anti Glycine receptor

75
Q

Rx of neuropathy due to plasmacytoma or sclerotic lesions

A

Rx of primary disease

76
Q

Jeopardy

Stiff-person, cerebellar syndromes

77
Q

Tumors associated with paraneoplastic autonomic neuropathy

A

SCLC

cancer of the pancreas or testis

carcinoid tumors

lymphoma

78
Q

Jeopardy

hodgkin lymphoma

A

tumor associated with anti Tr

79
Q

Presentation of cancer associated retinopathy

A

photosensitivity

progressive loss of vision and color perception

central or ring scotomas

night blindness

attenuation of photopic and scotopic responses in the electroretinogram (ERG).

81
Q

Tumors associated with anti-CV2/CRMP5

A

SCLC, thymoma

83
Q

Anti-NMDAR

A

Anti-NMDAR encephalitis

84
Q

Autonomic manifestations of encephalomyelitis

A

cardiac arrythmias

postural hypotension

central hypoventilation

85
Q

presentation of paraneoplastic sensory neuronopathy

A

symmetric/assymetric

painful dysesthesias

radicular pain

decreased or absent reflexes

Specialized sensations such as taste and hearing can also be affected

86
Q

MRI DD for paraneoplastic limbic encephalitis

A

nonparaneoplastic autoimmune limbic encephalitis

human herpesvirus type 6 (HHV-6) encephalitis

88
Q

Jeopardy

Breast, SCLC

A

Tumors associated with anti amphiphysin

89
Q

Jeopardy

Autonomic neuropathy

A

Anti-AChR (neuronal)

91
Q

Jeopardy

Cancer-associated retinopathy (CAR)-SCLC

Melanoma-associated retinopathy (MAR)

A

Recoverin, bipolar cell antibodies

92
Q

Jeopardy

Testicular (Ma2)

A

Tumor associated with anti Ma proteins

93
Q

Anti-amphiphysin

A

Stiff-person syndrome, encephalomyelitis

95
Q

3 key concepts in management of Paraneoplastic neurological disorders

A

symptoms may precede tumor

Syndrome develops rapidly,produces severe deficits in short period of time

prompt tumor control improves the neurologic outcome

97
Q

presentation of antiNMDA encephalitis

A

prodrome resembling viral infection

severe psychiatric symptoms

memory loss

seizures

decreased level of consciousness

abnormal movements (orofacial, limb, and trunk dyskinesias, dystonic postures)

autonomic instability

frequent hypoventilation

99
Q

PNDs associated with antibodies

A

60–70% of PNDs of the CNS

101
Q

Jeopardy

Cerebellar degeneration

opsoclonus

103
Q

Jeopardy

neuromyotonia+limbic encephalitis

A

morvan syndrome

104
Q

does Paraneoplastic Cerebellar Degeneration respond to Rx?

105
Q

Anti Ma 2 associated encephalitis pts may respond to

A

treatment of primary(testicular tumor)

Immunotherapy

106
Q

Limbic encephalitis is characterised by

A

confusion

depression

agitation

anxiety

severe short-term memory deficits

partial complex seizures

dementia

107
Q

Jeopardy

Breast, gynecologic, SCLC

A

tumors associated with anti Ri

108
Q

Features of brainstem encephalitis

A

Eye movement disorders:

nystagmus, opsoclonus, supranuclear or nuclear paresis

cranial nerve paresis

dysarthria

dysphagia

central autonomic dysfunction

109
Q

When to suspect lymphoma in paraneoplastic neurological dysfunction

A

rapidly progressive motor neuron syndrome

monoclonal protein in serum or CSF

110
Q

Jeopardy

Neuromyotonia, limbic encephalitis

A

Anti-VGKC- related proteins(LGI1, Caspr2)

112
Q

Tumor associated with Anti-AChR (neuronal)

113
Q

Tumors associated with vasculitis of nerve and muscle

A

SCLC

Lymphoma

114
Q

Anti-GAD

A

Stiff-person, cerebellar syndromes

115
Q

CSF profile of PNDs involving CNS

A

mild to moderate pleocytosis (

^protein

oligoclonal bands

117
Q

anti Yo

A

Cerebellar degeneration

118
Q

Jeopardy

issac syndrome

A

Peripheral nerve hyperexcitability

119
Q

antibodies associated with SCLC

A

anti Hu

anti Ri

Anti-CV2/CRMP5
Anti-amphiphysin

Recoverin, bipolar cell antibodies

120
Q

Jeopardy

SCLC, thymoma

A

Tumors associated with anti-CV2/CRMP5

121
Q

Jeopardy

LEMS, cerebellar degeneration

122
Q

opsoclonus

A

disorder of eye movement characterized by involuntary, chaotic saccades that occur in all directions of gaze

123
Q

Jeopardy

confusion

depression

agitation

anxiety

severe short-term memory deficits

partial complex seizures

dementia

A

Limbic encephalitis is characterised by

125
Q

Tumor associated with anti Ma proteins

A

Testicular (Ma2)

127
Q

Tumors in which Paraneoplastic Neurologic Syndromes are common

A

Thymoma

sclerotic myeloma

128
Q

_________ % of antiNMDA encephalitis female pts older than 18 years have uni- or bilateral ovarian teratomas

129
Q

Jeopardy

Encephalomyelitis, subacute sensory neuronopathy

130
Q

Anti-VGCC

A

LEMS, cerebellar degeneration

131
Q

Jeopardy

demyelination features predominate

A

IVIg,glucocorticoids and plasma exchange are useful in paraneoplastic peripheral neuropathies if

132
Q

symptoms of Paraneoplastic Stiff-Person Syndrome improve with

A

sleep

general anesthetics

133
Q

Recoverin, bipolar cell antibodies

A

Cancer-associated retinopathy (CAR)-SCLC

Melanoma-associated retinopathy (MAR)

134
Q

tumor associated with anti Tr

A

hodgkin lymphoma

135
Q

Neuropathy in Waldenstrom macroglobulinemia

A

distal symmetric sensorimotor neuropathy with predominant involvement of large sensory fibers

136
Q

Anti Hu

A

Encephalomyelitis, subacute sensory neuronopathy

137
Q

Rx of stiff person syndrome

A

therapy of the underlying tumor

glucocorticoids

diazepam, baclofen, sodium valproate, tiagabine, vigabatrin

138
Q

Diagnosis of PNS PNDs

A

Clinical

Biopsy

Electrophysiologic

139
Q

Jeopardy

epilepsia partialis continua

A

cortical encephalitis may present as

Hematology (9 decks)

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