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Paraneoplastic Neurologic Syndromes Flashcards

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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
IVIg in stiff person syndrome
benefit in non paraneoplastic disorder
18
Antibodies that may be found in issac syndrome
Caspr2-related proteins
19
rigidity in Paraneoplastic Stiff-Person Syndrome mainly affects
lower trunk and legs
20
# Jeopardy Encephalomyelitis, chorea, optic neuritis, uveitis, peripheral neuropathy
Anti-CV2/CRMP5
21
# Jeopardy disorder of eye movement characterized by involuntary, chaotic saccades that occur in all directions of gaze
opsoclonus
21
Tumors associted with Paraneoplastic Opsoclonus-Myoclonus Syndrome
Adults: lung,breast Children: neuroblastoma
21
Tumors associated with neuromyotonia
Usually non paraneoplastic Thymoma SCLC
22
# Jeopardy Eye movement disorders: nystagmus, opsoclonus, supranuclear or nuclear paresis cranial nerve paresis dysarthria dysphagia central autonomic dysfunction
Features of brainstem encephalitis
23
tumors associated with anti Ri
Breast, gynecologic, SCLC
23
Tumor associated with Anti-NMDAR
teratoma
24
Anti-Ma proteins
Limbic, hypothalamic, brainstem encephalitis
24
IVIg,glucocorticoids and plasma exchange are useful in paraneoplastic peripheral neuropathies if
demyelination features predominate
24
Peripheral neuropathy in sclerotic myeloma resembles
CIDP
26
# Jeopardy Cerebellar degeneration
anti Yo
27
# Jeopardy Limbic encephalitis with relapses
Anti-AMPAR
28
Antibodies pathogenic for LEMS
anti VGCC
28
# Jeopardy disinhibition of the fastigial nucleus of the cerebellum
pathology in Opsoclonus-myoclonus
29
Tumors associated with Anti-GABABR
SCLC, neuroendocrine
29
Anti Glycine receptor
Encephalomyelitis with rigidity, stiff-person syndrome
29
Presentation of myelitis
UMN or LMN signs myoclonus muscle rigidity and spasms
30
Presentation of vasculitis of nerve and muscle
painful symmetric or asymmetric distal axonal sensorimotor neuropathy with variable proximal weakness
31
# Jeopardy UMN or LMN signs myoclonus muscle rigidity and spasms
Presentation of myelitis
31
Acute necrotising myopathy
myalgias rapid progression of weakness involving the extremities and the pharyngeal and respiratory muscles
32
# Jeopardy Cerebellar degeneration
Anti-Tr
34
# Jeopardy Anti-AChR (muscle)
Myasthenia gravis
35
Encephalitis with antibodies to voltage-gated potassium channels (VGKC)-related proteins is common in
Men
36
Distinct characteristics of Encephalitides with Antibodies to Cell-Surface or Synaptic Proteins
1. May or may not be associated with tumor 2. Some occur in young individuals 3. Respond to immunotherapy
38
Anti-AChR (neuronal)
Autonomic neuropathy
38
Does Paraneoplastic Opsoclonus-Myoclonus Syndrome respond to Rx?
yes
39
Diagnosis of CNS PNDs
Clinical radiological CSF
40
Tumor associated with Anti-AChR (muscle)
Thymoma
40
morvan syndrome
neuromyotonia+limbic encephalitis
41
\_\_\_\_\_\_\_\_ % of anti AMPA associated encephalitis have a tumor
70
42
clinical presentation of neuromyotonia
cramps muscle twitching (fasciculations or myokymia) stiffness delayed muscle relaxation (pseudomyotonia) spontaneous or evoked carpal or pedal spasms paresthesia and hyperhidrosis
43
cortical encephalitis may present as
epilepsia partialis continua
44
Paraneoplastic encephalomyelitis and focal encephalitis are usually associated with
SCLC
45
anti NMDA encephalitis is misdiagnosed as
viral or idiopathic encephalitis neuroleptic malignant syndrome encephalitis lethargica
46
Rx of neuromyotonia
Phenytoin Carbamazepine plasma exchange
46
Clinical manifestations of paraneoplastic autonomic neuropathy
Pseudoobstruction cardiac arrythmias postural hypotension abnormal pupillary responses dry mouth anhidrosis erectile dysfunction problems in sphincter control
47
Anti-GABABR
Limbic encephalitis, seizures
49
Anti-CV2/CRMP5
Encephalomyelitis, chorea, optic neuritis, uveitis, peripheral neuropathy
50
# Jeopardy Limbic encephalitis, seizures
Anti-GABABR
51
Tumor commonly associated with cancer associated retinopathy
SCLC
52
presentation of paraneoplastic myelitis
UMN or LMN symptoms segmental myoclonus rigidity
53
Imaging for detection of tumors
CT CT+PET USG,MRI for Germ Cell Tumor of testis and teratoma of ovary
54
Limbic encephalitis
55
Peripheral nerve hyperexcitability
issac syndrome
57
# Jeopardy Encephalomyelitis with rigidity, stiff-person syndrome
Anti Glycine receptor
57
Screening after diagnosis of LEMS
Chest and abdomen CT,PET If negative,periodic screening for atleast 3 years
58
# Jeopardy Limbic, hypothalamic, brainstem encephalitis
Anti-Ma proteins
59
In _______ % of pts the Paraneoplastic Neurologic Syndromes precede cancer diagnosis
60%
60
Encephalitis with antibodies to voltage-gated potassium channels (VGKC)-related proteins frequently presents as
memory loss seizures hyponatremia sleep and autonomic dysfunction
61
Response of paraneoplastic encephalomyelitis to treatment
poor
63
# Jeopardy Stiff-person syndrome, encephalomyelitis
Anti-amphiphysin
64
# Jeopardy Thymoma
Tumor associated with anti GAD
65
Nystagmus in paraneoplastic cerebellar degeneration
downbeating
67
Components of paraneoplastic encephalomyelitis
cortical encephalitis limbic encephalitis brainstem encephalitis cerebellar gait and limb ataxia myelitis autonomic dysfunction
68
antibodies associated with prominent or pure cerebellar degeneration
anti Yo anti Tr
69
Tumor associated with Anti-VGCC
SCLC
70
pathology in Opsoclonus-myoclonus
disinhibition of the fastigial nucleus of the cerebellum
72
# Jeopardy Breast ovary
tumors associated with anti Yo
73
Anti-Ri
Cerebellar degeneration opsoclonus
73
Diff btw Paraneoplastic Peripheral Neuropathies that develop in early stage and late stage cancers
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
Tumor associated with Anti Glycine receptor
Lung
75
Rx of neuropathy due to plasmacytoma or sclerotic lesions
Rx of primary disease
76
# Jeopardy Stiff-person, cerebellar syndromes
Anti-GAD
77
Tumors associated with paraneoplastic autonomic neuropathy
SCLC cancer of the pancreas or testis carcinoid tumors lymphoma
78
# Jeopardy hodgkin lymphoma
tumor associated with anti Tr
79
Presentation of cancer associated retinopathy
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
Tumors associated with anti-CV2/CRMP5
SCLC, thymoma
83
Anti-NMDAR
Anti-NMDAR encephalitis
84
Autonomic manifestations of encephalomyelitis
cardiac arrythmias postural hypotension central hypoventilation
85
presentation of paraneoplastic sensory neuronopathy
symmetric/assymetric painful dysesthesias radicular pain decreased or absent reflexes Specialized sensations such as taste and hearing can also be affected
86
MRI DD for paraneoplastic limbic encephalitis
nonparaneoplastic autoimmune limbic encephalitis human herpesvirus type 6 (HHV-6) encephalitis
88
# Jeopardy Breast, SCLC
Tumors associated with anti amphiphysin
89
# Jeopardy Autonomic neuropathy
Anti-AChR (neuronal)
91
# Jeopardy Cancer-associated retinopathy (CAR)-SCLC Melanoma-associated retinopathy (MAR)
Recoverin, bipolar cell antibodies
92
# Jeopardy Testicular (Ma2)
Tumor associated with anti Ma proteins
93
Anti-amphiphysin
Stiff-person syndrome, encephalomyelitis
95
3 key concepts in management of Paraneoplastic neurological disorders
symptoms may precede tumor Syndrome develops rapidly,produces severe deficits in short period of time prompt tumor control improves the neurologic outcome
97
presentation of antiNMDA encephalitis
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
PNDs associated with antibodies
60–70% of PNDs of the CNS
101
# Jeopardy Cerebellar degeneration opsoclonus
Anti-Ri
103
# Jeopardy neuromyotonia+limbic encephalitis
morvan syndrome
104
does Paraneoplastic Cerebellar Degeneration respond to Rx?
No
105
Anti Ma 2 associated encephalitis pts may respond to
treatment of primary(testicular tumor) Immunotherapy
106
Limbic encephalitis is characterised by
confusion depression agitation anxiety severe short-term memory deficits partial complex seizures dementia
107
# Jeopardy Breast, gynecologic, SCLC
tumors associated with anti Ri
108
Features of brainstem encephalitis
Eye movement disorders: nystagmus, opsoclonus, supranuclear or nuclear paresis cranial nerve paresis dysarthria dysphagia central autonomic dysfunction
109
When to suspect lymphoma in paraneoplastic neurological dysfunction
rapidly progressive motor neuron syndrome monoclonal protein in serum or CSF
110
# Jeopardy Neuromyotonia, limbic encephalitis
Anti-VGKC- related proteins(LGI1, Caspr2)
112
Tumor associated with Anti-AChR (neuronal)
SCLC
113
Tumors associated with vasculitis of nerve and muscle
SCLC Lymphoma
114
Anti-GAD
Stiff-person, cerebellar syndromes
115
CSF profile of PNDs involving CNS
mild to moderate pleocytosis ( ^protein oligoclonal bands
117
anti Yo
Cerebellar degeneration
118
# Jeopardy issac syndrome
Peripheral nerve hyperexcitability
119
antibodies associated with SCLC
anti Hu anti Ri Anti-CV2/CRMP5 Anti-amphiphysin Recoverin, bipolar cell antibodies
120
# Jeopardy SCLC, thymoma
Tumors associated with anti-CV2/CRMP5
121
# Jeopardy LEMS, cerebellar degeneration
Anti-VGCC
122
opsoclonus
disorder of eye movement characterized by involuntary, chaotic saccades that occur in all directions of gaze
123
# Jeopardy confusion depression agitation anxiety severe short-term memory deficits partial complex seizures dementia
Limbic encephalitis is characterised by
125
Tumor associated with anti Ma proteins
Testicular (Ma2)
127
Tumors in which Paraneoplastic Neurologic Syndromes are common
Thymoma sclerotic myeloma
128
\_\_\_\_\_\_\_\_\_ % of antiNMDA encephalitis female pts older than 18 years have uni- or bilateral ovarian teratomas
50
129
# Jeopardy Encephalomyelitis, subacute sensory neuronopathy
Anti Hu
130
Anti-VGCC
LEMS, cerebellar degeneration
131
# Jeopardy demyelination features predominate
IVIg,glucocorticoids and plasma exchange are useful in paraneoplastic peripheral neuropathies if
132
symptoms of Paraneoplastic Stiff-Person Syndrome improve with
sleep general anesthetics
133
Recoverin, bipolar cell antibodies
Cancer-associated retinopathy (CAR)-SCLC Melanoma-associated retinopathy (MAR)
134
tumor associated with anti Tr
hodgkin lymphoma
135
Neuropathy in Waldenstrom macroglobulinemia
distal symmetric sensorimotor neuropathy with predominant involvement of large sensory fibers
136
Anti Hu
Encephalomyelitis, subacute sensory neuronopathy
137
Rx of stiff person syndrome
therapy of the underlying tumor glucocorticoids diazepam, baclofen, sodium valproate, tiagabine, vigabatrin
138
Diagnosis of PNS PNDs
Clinical Biopsy Electrophysiologic
139
# Jeopardy epilepsia partialis continua
cortical encephalitis may present as

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