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Neuro > Headache, tumors, neurocutaneous > Flashcards

Headache, tumors, neurocutaneous Flashcards

1
Q

Pain information from the trigeminal nerve/nuclei is mediated by neuropeptides like _ , _ , _

A

Pain information from the trigeminal nerve/nuclei is mediated by neuropeptides like calcitonin gene-related peptide , neurokinin , substance P

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2
Q

CGRP and substance P induce mast cells to release _

A

CGRP and substance P induce mast cells to release histamine
* Histamine stimulates more CGRP and substance P release

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3
Q

Name (3) types of primary headaches

A
  1. Tension
  2. Migraine
  3. Cluster (trigeminal autonomic cephalgias)
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4
Q

Name examples of secondary headaches

A

Secondary headaches include:
* Trauma
* Vascular disorders
* Substance use
* Infections

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5
Q

Excruciating unilateral pain in the distribution of V2 and V3 is called _

A

Excruciating unilateral pain in the distribution of V2 and V3 is called trigeminal neuralgia

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6
Q

Trigeminal neuralgia can be exacerbated by _

A

Trigeminal neuralgia can be exacerbated by sensory stimulation (shaving, eating, tooth brushing)

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7
Q

The presumed cause of trigeminal neuralgia is _

A

The presumed cause of trigeminal neuralgia is blood vessel pressing on the trigeminal nerve as it exits the brainstem
* Rarely caused by tumor compression or M.S. demyelination

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8
Q

The most appropriate imaging to assess trigeminal neuralgia is _

A

The most appropriate imaging to assess trigeminal neuralgia is MRI with contrast

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9
Q

First line treatment for trigeminal neuralgia is _

A

First line treatment for trigeminal neuralgia is carbamazepine

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10
Q

Side effects of carbamazepine include:

A

Side effects of carbamazepine include:
* Agranulocytosis
* Hyponatremia
* Liver damage
* Steven Johnson syndrome

In refractory cases can do microvascular decompression to separate the blood vessel from nerve

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11
Q

Familial hemiplegic migraine is an autosomal dominant condition with variable penetrance which involves _

A

Familial hemiplegic migraine is an autosomal dominant condition with variable penetrance which involves ion channelopathy
* Migraines have a strong genetic predisposition
* 3X more likely in first degree relatives

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12
Q

_ branch of the trigeminal nerve is associated with migraines

A

Opthalmic branch (V1) of the trigeminal nerve is associated with migraines

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13
Q

The migraine generator is thought to be in the [brain region]

A

The migraine generator is thought to be in the pons

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14
Q

_ is a wave of depolarization that can trigger the “migraine generator” in the pons

A

Cortical spreading depression (CSD) is a wave of depolarization that can trigger the “migraine generator” in the pons
* Causes the migraine aura

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15
Q

Diagnostic criteria of migraines includes:

A

Diagnostic criteria of migraines includes: POUND
* Pulsatile
* Lasts hours
* Unilateral
* Nausea
* Disabling

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16
Q

Triptans are (abortive/preventative) medications for migraines

A

Triptans are abortive medications for migraines
* Sumatriptan, naratriptan, rizatriptan
* Given PO, SQ, intranasally

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17
Q

Triptan mechanism of action: they target _

A

Triptan mechanism of action: they target 5-HT1B and 5-HT1D receptors
* Serotonin agonist
* CGRP release inhibitor and vasoconstrictor

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18
Q

Triptans have _ side effect and are contraindicated in patients with _

A

Triptans temporarily raise blood pressure and are contraindicated in patients with MI or stroke history

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19
Q

Never mix a triptan with _ or _

A

Never mix a triptan with MAO inhibitor or dihydroergotamine (DHE)

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20
Q

(Vasoconstricting/Vasodilating) drugs are used to treat migraines

A

Vasoconstricting drugs are used to treat migraines

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21
Q

Triptan toxicities include “triptan sensations” ie _ or _

A

Triptan toxicities include “triptan sensations” ie paresthesia or chest tightness

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21
Q

Dihydroergotamine (DHE) is a migraine medication that acts as a [mechanism]

A

Dihydroergotamine (DHE) is a migraine medication that acts as an alpha-adrenergic blocker & CGRP release inhibitor
* Directly stimulates vascular smooth muscle and agonizes serotonin
* Usually given intravenously

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22
Q

Side effects of DHE:

A

Side effects of DHE:
* Temporary rise in BP
* Myocardial ischemia (MI)
* Stroke
* Category X for pregnancy

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23
Q

Beta blockers like _ and calcium blockers like _ can be used for migraine prophylaxis

A

Beta blockers like metoprolol, propranolol and calcium blockers like verapamil, nifedipine can be used for migraine prophylaxis

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24
Q

_ and _ are two anti-epileptic drugs that can be use for migraine prophylaxis

A

Valproic acid and topiramate are two anti-epileptic drugs that can be use for migraine prophylaxis
* Recall that valproic acid can cause neural tube defects

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25
Q

_ is a CGRP receptor antibody that can be used for migraine prophylaxis

A

Erenumab is a CGRP receptor antibody that can be used for migraine prophylaxis
* These block the vasodilation, inflammation, and transmission of the seizure

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26
Q

Beta-blockers and SNRIs are helpful for migraine prophylaxis by _

A

Beta-blockers and SNRIs are helpful for migraine prophylaxis by blocking migraine initiation at the migraine generator

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27
Q

Diffuse pain that is “like a tight head band” describes _ type headache

A

Diffuse pain that is “like a tight head band” describes tension headache
* Trigemino-vascular system plays an important role

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28
Q

Unilateral _ and _ is classic for a cluster headache

A

Unilateral ptosis and miosis (anisocoria) is classic for a cluster headache
* Partial horner’s syndrome caused by failure of the sympathetics
* Occur in clusters, at the same time every day

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29
Q

Treatment for cluster headache includes triptans, prednisone, avoiding alcohol, and _

A

Treatment for cluster headache includes triptans, prednisone, avoiding alcohol, and oxygen (100% FiO2 by non-rebreather mask)

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30
Q

Giant cell (temporal) arteritis is caused by _

A

Giant cell (temporal) arteritis is caused by granulomatous inflammation of the external carotid artery branches
* Sometimes internal carotid branches are affected like opthalmic artery
* Need temporal artery biopsy to confirm- intimal thickening, lamina fragmentation, multinucleated giant cells

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31
Q

Giant cell arteritis will present with [signs] and needs to be treated right away with [medication]

A

Giant cell arteritis will present with unilateral headache, jaw claudication, visual disturbance and needs to be treated right away with prednisone
* ESR will also be elevated

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32
Q

Name three adult-type, diffuse gliomas

A

Name three adult-type, diffuse gliomas
1. Astrocytoma
2. Oligodendroglioma
3. Glioblastoma

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33
Q

_ is a circumscribed astrocytic glioma

A

Pilocytic astrocytic glioma is a circumscribed astrocytic glioma

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34
Q

Diagnosis?

A

Oligodendroglioma

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35
Q

Oligodendroglioma most commonly occurs in [patient demographics]

A

Oligodendroglioma most commonly occurs in adults, 30s-40s

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36
Q

Oligodendroglioma is most common in [brain region]

A

Oligodendroglioma is most common in cerebral hemispheres, especially frontal

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37
Q

The classic histology feature of oligodendroglioma is _ and _

A

The classic histology feature of oligodendroglioma is fried egg appearance and chicken wire capillaries

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38
Q

The molecular hallmark of oligodendroglioma is _

A

The molecular hallmark of oligodendroglioma is 1p/19q codeletion

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39
Q

Astrocytomas are most common in [patient demographic]

A

Astrocytomas are most common in adults, 30s-40s

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40
Q

Astrocytomas are most common in [brain region]

A

Astrocytomas are most common in cerebral hemispheres

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41
Q

Diagnosis?

A

Astrocytoma: lack a fried egg appearance (no perinuclear appearance); look similar to oligodendrocytes but astrocytes have more variable nuclei shape

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42
Q

Grade 1 astrocytoma means the tumor is _

A

Grade 1 astrocytoma means the tumor is well circumscribed, potentially cured by resection
* Grade 1 and 2 are low grade
* Grade 2-4 are infiltrating and need treatment beyond resection

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43
Q

Grade 2 astrocytoma features [histologic feature]

A

Grade 2 astrocytoma features atypia
* Grade 2 = diffuse astrocytoma

44
Q

Grade 3 astrocytoma features [histologic feature]

A

Grade 3 astrocytoma features mitoses
* Grade 3 = anaplastic

45
Q

Grade 4 astrocytoma features [histologic feature]

A

Grade 4 astrocytoma features endothelial proliferation +/- necrosis
* Grade 4 = glioblastoma

46
Q

_ tumors are classically in older adults > 55yo and are found in the cerebral hemispheres, often crossing midline

A

Glioblastoma tumors are classically in older adults > 55yo and are found in the cerebral hemispheres, often crossing midline
* “Butterfly glioma” crosses midline

47
Q

The classic histologic feature of glioblastoma is _

A

The classic histologic feature of glioblastoma is pseudopalisading necrosis
* Histology can also show microvascular/endothelial proliferation

48
Q
A

Hemangioblastoma

49
Q
A

Metastatic melanoma

50
Q
A

Angiomatous meningioma

51
Q

What phenomenon is shown here?

A

Microvascular proliferation (ie endothelial cell proliferation)

52
Q

Diagnosis?

A

Oligodendroglioma
* Note that oligodendrogliomas and ependymomas max out at grade 3

53
Q

Glioblastoma with _ mutation actually has a better prognosis

A

Glioblastoma with IDH mutation actually has a better prognosis

54
Q

Diagnosis?

A

Pilocytic astrocytoma

Posterior fossa mass in child could be pilocytic astrocytoma, medulloblastoma, ependymoma, hemangioblastoma

55
Q

“Cyst with a mural nodule” is classic for [brain tumor]

A

“Cyst with a mural nodule” is classic for pilocytic astrocytoma

56
Q

Diagnosis?

A

Pilocytic astrocytoma

57
Q

The classic histologic feature of pilocytic astrocytoma is _

A

The classic histologic feature of pilocytic astrocytoma is rosenthal fibers
* They are often biphasic, meaning they have areas that are compact and areas that are loose

58
Q

The most common primary brain tumor in children is _

A

The most common primary brain tumor in children is pilocytic astrocytoma
* Occur in the cerebellum of kids but cerebrum in adults

59
Q

Kids with NF1 often get optic pathway gliomas, specifically [type]

A

Kids with NF1 often get optic pathway gliomas, specifically pilocytic astrocytoma

60
Q

_ are childhood tumors that classically sit at the floor of the 4th ventricle

A

Ependymoma are childhood tumors that classically sit at the floor of the 4th ventricle
* Because of this they can cause hydrocephalus

61
Q

Patients with NF2 can get glial tumors in the spinal cord, specifically [type]

A

Patients with NF2 can get glial tumors in the spinal cord, specifically ependymoma

62
Q

The histological hallmark associated with ependymomas is _

A

The histological hallmark associated with ependymomas is perivascular pseudorosettes
* Also see hyalinized vessels and sometimes true rosettes

63
Q

The most important prognostic predictor for a child with ependymoma is _

A

The most important prognostic predictor for a child with ependymoma is extent of resection

64
Q

The most common malignant peds brain tumor is _

A

The most common malignant peds brain tumor is medulloblastoma
* Occur in the cerebellum

65
Q

“drop metastasis to the spinal cord” should make you think [tumor]

A

“drop metastasis to the spinal cord” should make you think medulloblastoma

66
Q

Medulloblastomas feature [histologic feature]

A

Medulloblastomas feature homer-wright rosettes
* Have a central neuropil of axons, glial products, etc

67
Q

_ molecular associations have the best medulloblastoma prognosis while _ have the worst

A

WNT molecular associations have the best medulloblastoma prognosis while SHH with TP53 mutation or MYCN amplification have the worst

68
Q

Diagnosis?

A

Ganglioglioma: glial and neural components (mixed)
* Features abnormal neurons and eosinophilic granular bodies
* These occur in kids and young adults and have excellent prognosis

69
Q

Name the (5) most common sources of brain metastases in adults

A

Name the (5) most common sources of brain metastases in adults
1. Lung
2. Breast
3. Kidney
4. Melanoma
5. Colon

70
Q

Immunohistochemical marker for glial neoplasms is _

A

Immunohistochemical marker for glial neoplasms is glial fibrillary acid protein (GFAP)

71
Q

Immunohistochemical marker for carcinomas is _

A

Immunohistochemical marker for carcinomas is cytokeratin (CK)

72
Q

Immunohistochemical marker for hematologic neoplasms (ie leukemia, lymphoma) is _ ; or for B-cell neoplasms _

A

Immunohistochemical marker for hematologic neoplasms (ie leukemia, lymphoma) is CD45 ; or for B-cell neoplasms CD20

73
Q

This shows metastatic carcinoma which has metastasized to _

A

This shows metastatic carcinoma which has metastasized to cerebellum

74
Q

This is a glioma (GFAP positive) that features _ (green circle)

A

This is a glioma (GFAP positive) that features endothelial/ microvascular proliferation (green circle)

75
Q

The most common CNS neoplasm in adults is _

A

The most common CNS neoplasm in adults is metastasis
* Lung, breast, melanoma, renal cell, colorectal
* Most occur as multiple lesions

76
Q

The most common primary CNS lymphoma is _

A

The most common primary CNS lymphoma is diffuse large B-cell lymphoma
* Positive for CD20
* If EBV+ think of immunodeficiency (HIV)
* Look for fever, weight loss, night sweats, mass effect signs

77
Q

The most common germ cell tumor of the CNS is _

A

The most common germ cell tumor of the CNS is germinoma
* Same histology as testicular seminoma and ovarian dysgerminoma
* Most commonly occur in the midline and in the pineal region

78
Q

Compression of the dorsal midbrain/ pineal region (ie germinoma) can cause _ syndrome; a triad of upward gaze palsy, convergence-retraction nystagmus, light-near dissociation

A

Compression of the dorsal midbrain/ pineal region (ie germinoma) can cause Parinaud syndrome; a triad of upward gaze palsy, convergence-retraction nystagmus, light-near dissociation

79
Q

_ is the mcc supratentorial brain tumor in kids (suprasellar region); it is usually cystic with motor oil like fluid

A

Craniopharyngioma is the mcc supratentorial brain tumor in kids (suprasellar region); it is usually cystic with motor oil like fluid
* Squamous neoplasm derived from rathke pouch remnants; wet keratin, calcifications

80
Q

Neurofibromatosis type 1 is [inheritance pattern]

A

Neurofibromatosis type 1 is autosomal dominant
* It has 100% penetrance, but variable expressivity
* 50% of people with NF1 are de-novo mutations

81
Q

To make a clinical diagnosis for NF1, at least 2 features must be present:

A

To make a clinical diagnosis for NF1, at least 2 features must be present:
* Multiple cafe-au-lait macules
* Skinfold freckling
* Iris hamartomas (lisch nodules)
* Optic pathway glioma
* Multiple neurofibromas
* Osseous lesions (severe scoliosis)
* An affected first degree relative

82
Q

What is the typical presentation of NF1?

A

For the majority of people with NF1…
* Skin fold freckling and lisch nodules present by puberty
* Cutaneous neurofibromas common
* Children have learning disabilities and ADHD
* Epilepsy more common, sometimes vision loss from optic nerve glioma

83
Q

The pathogenesis of NF1 involves a mutation in [gene] which encodes [protein] which results in _

A

The pathogenesis of NF1 involves a mutation in NF1 which encodes neurofibromin which results in Ras hyperactivation –> proto-oncogene involved in cell growth in differentiation
* Neurofibromin is a tumor suppressor gene/ negative regulator of Ras

84
Q

Identify the skin pathology

A

Cutaneous neurofibroma: polypoid, well circumscribed skin lesions

85
Q

Identify the skin pathology

A

Plexiform neurofibroma: likely congenital

86
Q

_ neurofibromas are more likely to transform into malignant peripheral nerve sheath tumors

A

Plexiform neurofibromas are more likely to transform into malignant peripheral nerve sheath tumors

87
Q

Benign or malignant neurofibroma?

A

Benign

88
Q

Benign or malignant neurofibroma?

A

Malignant- malignant peripheral nerve sheath tumor

89
Q

The most common nerve sheath tumor is _

A

The most common nerve sheath tumor is schwannoma (acoustic neuroma)
* Often found incidentally in the 4th-5th decade of life

90
Q

Bilateral schwannomas are a feature of [condition]

A

Bilateral schwannomas are a feature of neurofibromatosis type 2

91
Q

Schwannomas are well-circumscribed masses attached to the nerve found in the _

A

Schwannomas are well-circumscribed masses attached to the nerve found in the soft tissue, cranial nerves, peripheral nerves, internal organs

92
Q

Schwannomas will exhibit biphasic nature on histology; dense areas with nuclear palisading are called _ while more hypocellular areas with perinuclear clearing are called _

A

Schwannomas will exhibit biphasic nature on histology; dense areas with nuclear palisading are called Antoni A while more hypocellular areas with perinuclear clearing are called Antoni B

93
Q

In addition to vestibular schwannoma, individuals with NF2 can have multiple [tumors]

A

In addition to vestibular schwannoma, individuals with NF2 can have multiple meningiomas

94
Q

NF2 is [inheritance]

A

NF2 is autosomal dominant
* Half of cases are sporadic/ newly acquired germline mutations

95
Q

NF2 is associated with the protein _ , which is coded by NF2

A

NF2 is associated with the protein merlin , which is coded by NF2

96
Q

NF2 is on chromosome _

A

NF2 is on chromosome 22q12.2

97
Q

The loss of merlin in NF2 causes _

A

The loss of merlin in NF2 causes loss of tumor suppression; merlin normally acts via E-cadherin-mediated contact inhibition of proliferation
* No merlin = no contact inhibition = schwann cells proliferate

97
Q

Von Hippel Lindau (VHL) disease is an autosomal dominant condition that involves _

A

Von Hippel Lindau (VHL) disease is an autosomal dominant condition that involves tumors in multiple organ systems

98
Q

Common tumors in VHL include:

A

Common tumors in VHL include:
* Hemangioblastomas
* Renal cysts and renal cell carcinoma
* Adrenal pheochromocytoma (VHL type 2)
* Pancreatic neuroendocrine tumors

99
Q

The VHL gene is a [type] found on chromosome _

A

The VHL gene is a tumor suppressor gene found on chromosome 3p25.3

100
Q

VHL protein is important for the degradation of _ ; without it, we end up with hyper-vascular tumors

A

VHL protein is important for the degradation of hypoxia-inducing factor (HIL) ; without it, we end up with hyper-vascular tumors

101
Q

Tuberous sclerosis is an autosomal dominant disorder that arises from mutations at two loci; TSC1 on [chromosome] and TSC2 on [chromosome]

A

Tuberous sclerosis is an autosomal dominant disorder that arises from mutations at two loci; TSC1 on 9q and TSC2 on 16p
* The majority of cases are caused by TSC2 (more severe phenotype)
* TSC1 = harmartin
* TSC2 = tuberin

102
Q

Common clinical manifestations of tuberous sclerosis:

A

Common clinical manifestations of tuberous sclerosis:
* Subependymal giant cell astrocytoma (SEGA)
* Epilepsy, intellectual disorder, autism
* Neuropsychiatric disorders
* Cardiac rhabdomyoma, kidney issues, lung issues, angiofibroma (skin)

103
Q

_ are cerebral cortical tubers ; aka distorted “normal” tissue extending from grey matter into white matter

A

Glioneuronal hamartomas are cerebral cortical tubers ; aka distorted “normal” tissue extending from grey matter into white matter

104
Q

Diagnosis?

A

Subependymal giant cell astrocytoma (SEGA)

105
Q

Port-wine birthmark, leptomeningeal vascular malformations, glaucoma, and neurologic deficits (stroke, seizures, atrophy) are characteristic of _

A

Port-wine birthmark, leptomeningeal vascular malformations, glaucoma, and neurologic deficits (stroke, seizures, atrophy) are characteristic of Sturge-Weber syndrome

106
Q

Sturge-weber is NOT inherited; and involves somatic mosaic variants in [gene] or [gene]

A

Sturge-weber is NOT inherited; and involves somatic mosaic variants in GNAQ or GNA11

107
Q

Sturge weber involves a _ mutation in GNAQ which alters the normal signaling between G protein coupled receptors and downstream effector proteins

A

Sturge weber involves a point mutation in GNAQ which alters the normal signaling between G protein coupled receptors and downstream effector proteins

Neuro (18 decks)

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