Neuroimunologia Flashcards
(122 cards)
1
Q
Amiloidose gera dano axonal ou desmielinizante
A
Axonal
2
Q
Quando STC deve pensar em amiloidose
A
Bilateral, progressiva, resistente a tratamento, com sintomas em outros órgãos
3
Q
Manifestações oculares da amiloidose
A
Floaters, flashes, glaucoma, catarata, olho seco
4
Q
Manifestações de SNC de amiloidose
A
Sangramentos, AIT, aura, epilepsia
5
Q
Como detectar amiloidose
A
Biópsia de músculo e nervo, PET scan do coração
6
Q
Amiloidose é acometimento sensitivo, motor ou misto?
A
Misto
7
Q
Clínica neuropatia amiloidose
A
Comprimento dependente, simétrica, progressiva, disautonomia
8
Q
É comum restrição a difusão e captação de contraste em encefalite autoimune?
A
Não, melhor pensar em infecção e neoplasia
9
Q
Hérpes vírus pode ser trigger para encefalite autoimune?
A
Sim
10
Q
Devemos testar anti-mog em casos típicos de EM?
A
Não, muitos podem vir positivos em baixos títulos
11
Q
Precisa retestar paciente que veio anti-mog negativo na fase aguda sem tratamento?
A
Não
12
Q
Quantos meses depois do tratamento poderia tentar testar anti-mog?
A
3 meses
13
Q
Precisa tratar MOGAD monofásico?
A
Não
14
Q
Principal fator de risco para recorrência de MOGAD?
A
Ter recorrido uma vez
15
Q
Tratamento MOGAD
A
Pulso, IGG, plasmaférese, desmame lento de corticoide em um ano
16
Q
Manifestações Sjrögen no SNC
A
Vasculite, AVC, AIT
17
Q
Achados laboratoriais de Neuro Sjrögen
A
Eosinofilia e diminuição do nível de IGG
18
Q
O que pensar quando temos vários anticorpos positivos em baixos títulos?
A
Reação cruzada
19
Q
Exame padrão ouro já vasculite
A
Angiografia
20
Q
Exames de imagem na vasculite
A
Vessel wall e angiografia
21
Q
Limitação exames de imagem na vasculite
A
Não vê bem pequenos vasos
22
Q
Anticorpo na Sd de Miller Fisher
A
Anti gq1b
23
Q
Alvo inebilizumab
A
Anti CD 19
24
Q
Significado CAR-T cell
A
Chimeric Antigen Receptor T-cell
25
Tempo de aparecimento médio de complicação autoimune de inibidor de check point
5-15 semanas
26
Cânceres mais associados a síndromes paraneoplásicas
Mama, linfoma, ginecológico, medular tireoideano, pulmão
27
Sintomas de cerebelite
Cefaleia, febre, confusão, náusea e ataxia cerebelar
28
DD cerebelite
Infarto de cerebelo, câncer cerebelar, meningoencefalite infecciosa, ADEM, PRES
29
Se inchaço de cerebelo na imagem, o que fazer e por que?
Corticoide para evitar inchaço e hérniação
30
Imagem de cerebelite
Acometimento bilateral e difuso de cerebelo
31
Mielite na NMO - características
3 ou mais segmentos contíguos intramedulares com lesão ou atrofia
32
Onde é a lesão da síndrome de área postrema
Dorso da medula
33
Lesões de tronco da NMO - características
Periependimarias
34
Lesão do nervo óptico da NMO - características
Mais de 1/2 nervo óptico ou envolvendo quiasma
35
Definição síndrome da área postrema
Nausea, vômitos e soluços
36
Red flag do tempo de progressão do surto de NMO
Menos de 4 horas ou mais de 4 semanas para o ápice
37
Presença de BOC é red flag para NMO?
Sim
38
Porcentagem de casos monofásicos de NMO
5-10%
39
O que aumenta o risco de recorrência na NMO?
Soropositividade
40
Diagnóstico NMO com anticorpo
1 core clinical + excluir outras causas
41
Diagnóstico de NMO sem anticorpo
2 core clinical, sendo um neurite ou LETM ou síndrome de área postrema
42
Core clinical da NMO
Neurite óptica, LETM, síndrome de área postrema, síndrome diencefálica ou narcolepsia, síndrome de tronco aguda, síndrome cerebral com lesões típicas
43
Relação câncer urotelial e síndromes paraneoplasicas
Pouca relação, mas possível em carcinoma urotelial
44
Qual doença desmielinizante pode ter lesões que desaparecem?
Mogad
45
O que significa PLAID?
PLCG2 associated antibody deficiency and immune dysregulation
46
Característica PLAID
Reação alérgica a temperaturas frias
47
Tolebrutinib class
Oral BTK inhibitor
48
Autoimmune diseases associated with vasculitis
Lupus and rheumatoid arthritis
49
Infectious diseases associated with vasculitis
Hepatites B and C
50
Cancers that can trigger vasculitis
Lymphoma and leukemia
51
Sneddon Syndrome
Slowly progressive neurocutaneous syndrome with livedo racemosa and recurrent cerebrovascular events
52
Divry Van Bogaert Syndrome
Familial juvenile-onset disorder with livedo racemosa, white matter disease, dementia, epilepsy and cerebral angiomatosis in angiography (leptomeningial and transdural anastomoses)
53
Clinical findings in vasculitis
Headache that does not go away
Confusion and forgetfulness (dementia)
Sensibility complaints
Weakness (usually arms and legs)
Pain
Vision problems
Seizures
Problems speaking or understanding
Fever, sick feeling, weight loss, rashes or skin discoloration
Multiple organ damage
54
Findings in image of vasculitis in Brain
Aneurysms
Thrombosis
Swelling of the brain
55
Vasculitis investigation
RCP, segmentation rate, auto-antibodies, CSF, biopsy, image, angiogram, USG
56
Vasculitis treatment
Steroids, azathioprine, cyclophosphamide, rituximab, aneurysms treatment
57
Giant cell arteitis affects what arteries?
Aorta and its primary branches (temporal artery and ophthalmic artery)
58
Giant cell arteitis epidemiology?
Age 50 and older
59
Giant cell arteitis symptoms?
New, severe headache. Blurred, double vision or sudden vision loss. Pain in jaw or tongue. Tendernes in temporal arteries. Fever, weight loss, neck, muscle and joint pain. Fatigue.
60
Primary angiitis of CNS symptons
slowly progression of headache, dementia, behavioral changes, pain, sensory abnormalities and tremor. Seisures. Multiple AVC, AIT
61
Primary angiitis of CNS epidemiology
any age, peak at 50, mostly males
62
Takayasu's arteritis arteries affected
Aorta
63
Takayasu's arteritis epidemiology
women age of 40
64
Takayasu's arteritis symptoms
headache, dizziness, cold and numbness in the limbs, visual disturbances, problems with memory . Strokes, heart attacts, intestine damage
65
Takayasu's arteritis image
narrowing, blockage or swelling
66
Polyarteritis nodosa epidemiology
40-60 year, more man
67
Polyarteritis nodosa symptoms
fever, abdominal pain, numbness or pain in legs and limbs, muscle aches, weakness, weight loss. Kidney failure, HPT. PNS neuropathy is more comom than CNS
68
Deficiency of adenosine deaminase 2 (DADA2) is a mutation in what gene?
CECR1 gene
69
Deficiency of adenosine deaminase 2 (DADA2) causes what disease?
Vasculitis
70
Deficiency of adenosine deaminase 2 (DADA2) symptons
fever, skin nodules, livedo reticularis, joint pain. Strokes in chielhood
71
72
Meaning ICANS?
Immune Effector Cell-Associated Neurotoxicity Syndrome
73
% patients with ICANS
20-60% patients
74
ICANs pathology
release of inflammatory cytokines secreted by macrophages and monocytes, increasing vascular permeability and endothelial activation and leading to blood–brain barrier breakdown
75
Risk factor for ICANS
high disease burden, older age, and the specific CAR-T product
76
When does ICAN appear?
5 days, but some in 3 weeks
77
Symptoms of ICANS
Tremor, confusion, agitation, seizures, cerebral oedema, hesitancy of speach, aphasia, status epilepticus, hemorrhagic
78
Score for ICANS
Immune Effector Cell Encephalopathy Score (ICE)
79
ICANS treatment
10-20mg dexamethasone 6/6hrs ou metilprednisolone 1g for at least 4 days
80
What to do in severe responses of CAR-T cell?
Anakira (IL 1 receptor antagonist) or chemotherapy to kill car t cell
81
Treatment intracranial hipertension in ICANS
Acetazolamide 1000mg IV + 250-1000 every 12 hours, elevate head bed, hyperventilation, hyperosmolar therapy with manitol
82
Anti-γ-aminobutyric acid B (GABAB)-receptor encephalitis presentation
Seizures, cognitive impairment, confusion, and personality changes
83
Anti-γ-aminobutyric acid B (GABAB)-receptor encephalitis is usually associated with what disease?
Small-cell lung cancer
84
Anti-IgLON5 autoimmune encephalitis presentation
Sleep disorder (parasomnia, insomnia, excessive daytime sleepiness, and sleep-disordered breathing), bulbar symptoms, gait abnormalities, recurrent visual and acoustic hallucinations
85
Gene ALSP
CSF1R (colony-stimulating factor-1 receptor)
86
Meaning ALSP
Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia
87
Percentage of ALSP in leukodystrophys
10-25%
88
ALSP inheritance pattern
Autosomal dominant
89
Brain CT in ALSP
Multifocal calcifications in white matter
90
ALSP clinical presentation
Behavioral changes, executive dysfunction, depression, anxiety, psychosis, progressive cognitive decline, gait disturbances, parkinsonism and epilepsy
91
ALSP typical MR imaging findings
Bilateral patchy, diffuse, or confluent T2 hyperintensities in the white matter. Frontal and parietal lobe predominance, Without enhancement. Subcortical U-fibers are generally spared. DWI persistent hyperintensities are punctate with restricted diffusion or normal intensity on ADC maps.
92
ALSP MR imaging in pre symptomatic
Asymmetric small-sized nodular hyperintensities
93
Difference between ALSP and MS
There is no white matter lesion in callososeptal interface in ALSP and no brain calcification in MS
94
Corpus callosum morphology in ALSP
Thin
95
DD of ALSP besides MS
Adult-onset leukoencephalopathy due to autosomal recessive mutations in the mitochondrial alanyl-transfer RNA synthetase 2 gene (AARS2-L)
96
Difference in MRI between ALSP and AARS-L
Marked restricted diffusion on ADC map in AARS2-L; in ALSP, ADC values are similar to or slightly lower than normal white matter
calcifications in the frontal periventricular white matter in ALSP but not in AARS-L
97
Marchiafava-Bignami disease epidemiology
Chronic alcoholics (with middle-aged to elderly male patients 40-60 years of age
98
Marchiafava-Bignami disease clinical presentation
Motor or cognitive disturbances, a hemispheric disconnection syndrome (apraxia, hemialexia, dementia) and/or seizures.
99
Marchiafava-Bignami disease pathology
Deficiency of the vitamin B group results in necrosis and demyelination of the corpus callosum. Some reports present cases of extension into the hemispheric white matter, internal capsule and middle cerebellar peduncle. Although rare, Morel laminar sclerosis can also be seen
100
Marchiafava-Bignami disease CT changes
Hypoattenuating regions in the corpus callosum
in exceptional situations of hemorrhage, these regions may turn iso- or hyperattenuating
101
Marchiafava-Bignami disease MRI
Corpus callosum may appear edematous in the acute phase and atrophic in the chronic phase.
T1: hypointense foci in the corpus callosum in the acute phase
T2:
- acute phase: hyperintensities in the corpus callosum
- subacute phase: may show hypointense focal lesions (likely as a result of hemosiderin)
ears of the lynx sign may be seen.
102
Marchiafava-Bignami disease DD
MS
Diffuse axonal injury if preceding trauma
Callosal infarction: rare due to its rich blood supply
Transient lesions of the splenium of the corpus callosum
103
Cause of progressive multifocal leukoencephalopathy (PML)
Polyomavirus JC (JC virus)
104
Risk factors for PML
Chronic corticosteroid or immunosuppressive therapy for organ transplant, or individuals with cancer (such as Hodgkin's disease or lymphoma). Autoimmune conditions such as multiple sclerosis, rheumatoid arthritis, and systemic lupus erythematosus. HIV-1 infection.
105
Treatment of PML
Withdraw the medications causing, can use plasmapheresis to accelerate
106
Eaton Lambert antibody
Antibody against voltage-gated calcium channels
107
Cancer often associated with Eaton Lambert
Small cell lung carcinoma
108
109
Anticorpos relacionados a Miastenia Gravis
anti-receptor de acetilcolina, anti-MuSK, anti-lipoproteína de baixa densidade 4 (LRP4), anti-agrina, anti-epitotos de retrovirus endógenos humanos (HERVs), especificamente HERV-K e HERV-W
110
How IgG works?
modulation of Fc gama receptors, reducing pathogenic effect of autoantibodies. Suppress T cell. Modulate B cell, inhibit complement. Expansion of Tregs and suppress pro-inflammatory Th17 cells. Autophagy in peripheral blood mononuclear cells.
111
Rituximab posology in MG
1g + 1g after 15 days + 1g every 6 months
112
Ravulizumab action mechanism
Blocks conversion of C5a to C5b blocking the formation of MAC (membrane attack complex)
113
Tocilizumab mechanism
Interleukin-6 receptor antagonist
114
Tocilizumab uses
Cytokine release syndrome, systemic juvenile idiopathic arthritis, giant cell arteritis, RA
115
Tríade da síndrome de suzak
Encefalopatia, hipoacusia neurossensorial e alterações visuais
116
Anticorpo relacionado a neuropatia motora multifocal
IgM GM1
117
Efgartigimod alfa action mechanism
neonatal Fc receptor antagonist
118
Rozanolixizumab action mechanism
Blocks the interaction between the neonatal Fc receptor (FcRn) and IgG. This process is called Fc receptor blocking.
119
Nipocalimab action mechanism
Monoclonal antibody that binds to the neonatal Fc receptor (FcRn), which reduces the recycling of IgG antibodies. This results in a decrease in the amount of IgG antibodies in the blood, including autoantibodies
120
Batoclimab action mechanism
Inhibiting the binding of IgG to FcRn. This results in the rapid catabolism of IgG via lysosomal degradation.
121
Alentuzumab mecanismo de ação
Ligando-se ao CD52, um antígeno presente na superfície de linfócitos T e B. Isso inibe a circulação dessas células e provoca a depleção e repopulação de linfócitos e lise mediada por complemento
122
Progressive encephalomyelitis with rigidity and myoclonus (PERM) antibodies
Glutamic acid decarboxylase (GAD), glycine receptors (GlyR), N-methyl-D-aspartate receptors (NMDAR), and dipeptidyl-peptidase-like protein 6 (DPPX)
