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Pathology > Neuro pathology > Flashcards

Neuro pathology Flashcards

1
Q

Dandy-Walker malformation

A

Congenital failure of the cerebellar vermis to develop

Presents with a massively dilated 4th ventricle and an absent cerebellum

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2
Q

Syringomyelia

A

Cystic degeneration of the spinal cord, arising with trauma or in associated with type 1 Arnold-Chiari malformation; usually presents at C8-T1

Initially presents with “cape-like” loss of pain and T sensation with sparing of fine touch and position sense (due to damage to anterior white commissure)

Syprinx expansion may result in muscle atrophy and weakness (damage to anterior horns) and horner syndrome (damage to lateral horns)

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3
Q

Poliomyelitis

A

Damage to anterior motor horn due to Poliovirus

Presents with LMN signs

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4
Q

Werdnig-Hoffman disease

A

Inherited degeneration of anterior motor horns

Presents as “floppy baby”

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5
Q

Amyotropic Lateral Sclerosis (ALS)

A

Degenerative disorder of UMN and LMN Initially presents as atrophy and weakness of hands.

Lack of sensory impairment distinguishes ALS from syringomyelia.

May be caused by zinc-copper superoxide disputes mutation (SOD1)

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6
Q

Friedreich ataxia

A

Autosomal recessive degenerative disorder of the cerebellum and spinal cord, caused by expansion of GAA in the frataxin gene (mitochondrial iron regulation) on chromosome 9

Presents with ataxia, loss of vibratory sense and proprioception, muscle weakness in the LEs, loss of DTRs, and hammertoes.

Associated with HOCM

Closely resembles severe Vitamin E deficiency

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7
Q

Subfalcine hernation

A

Displacement of the cingulate gyrus under the falx cerebri

Compression of the ACA

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8
Q

Uncal herniation

A

Displacement of the temporal lobe uncus under the tentorium cerebelli

  • Compression of CNIII –> down and out eye with dilated pupil
  • Compression of PCA –> contralateral homonymous hemianopsia with macula sparing
  • Rupture of paramedian artery –> Duret (brainstem) hemorrhage
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9
Q

Metachromatic leukodystrophy

A

Demyelinating disorder caused by deficiency of arylsulfatase. Sulfatides accumulate in the lysosomes of oligodendrocytes (lysosomal storage disease)

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10
Q

Krabbe disease

A

Demyelinating disorder caused by deficiency of galactocerebrosidase. Galactocerebroside accumulates in macrophages.

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11
Q

Adrenoleukodystrophy

A

Demyelinating disorder caused by impaired addition of coenzyme A to long-chain fatty acids (X-linked).

Accumulation of fatty acids damages adrenal glands and white matter of the brain.

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12
Q

Multiple sclerosis

A

Demyelinating disorder caused by autoimmune destruction of CNS myelin and oligodendrocytes. Associated with HLA-DR2.

Relapsing and remitting neurologic deficits (e.g. scanning speech and internuclear opthalmoplegia)

MRI reveals plaques (white matter demyelination)

LP shows lymphocytes, increased Ig’s with oligoclonal IgG bands, and myelin basic protein

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13
Q

Subacute sclerosing panencephalitis

A

Demyelinating disorder caused by measles virus. Infection in infancy, slow progression, symptoms in childhood.

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14
Q

Progressive multifocal leukoencephalopathy

A

Demyelinating disorder caused by JC virus of oligodendrocytes. Reactivation of latent virus can occur with AIDS or leukemia

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15
Q

Central pontine myelinolysis

A

Demyelinating disorder caused by rapid IV correction of hyponatremia. Causes “locked in” syndrome.

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16
Q

Morphological features of Alzheimer disease

A

1) Cerebral atrophy with hydrocephalus ex vacuo
2) Extracellular neuritic plaques, composed of AB amyloid and entangled neuritic processes
3) Intracellular neurofibrillary tangles, composed of hyperphosphorylated tau protein
- unlike in Pick disease, where tau proteins form round aggregates

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17
Q

Histology of Parkinson disease

A

Loss of pigmented neurons in the SNc

Lewy bodies - round eosinophilic inclusions of alpha-synuclein in affected neurons; Lewy bodies of Lewy body dementia are located in the cortex (alpha-synuclein defect)

18
Q

Glioblastoma multiforme

A

Malignant high grade tumor of astrocytes in adults; most common primary CNS malignancy in adults

Arises in cerebrum and crosses corpus collosum to form “butterfly lesion”; characterized by regions of necrosis surrounded by “pseudopallisading” tumor cells.

Stain astrocytes for GFAP (intermediate filament in glial cells)

19
Q

Meningioma

A

Benign tumor of arachnoid cells in adults (females>males)

Tumor compresses but does not invade cortex.

May present with seizures.

Characterized by spidle cells arranged in a “whorled pattern”; psammoma bodies may be present

20
Q

Schwannoma

A

Benign tumor of schwann cells in adults

Usually arises at cerebellopontine angle and affects CNVIII

Tumor cells are S-100 positive.

Bilateral schwannomas are seen in NF type 2

21
Q

Oligodendroglioma

A

Malignant tumor of oligodendrocytes in adults, usually affecting frontal lobe.

Oligodendrocytes have a “fried-egg” appearance

22
Q

Pilocytic astrocytoma

A

Benign low-grade tumor of astrocytes in children; most common primary CNS malignance in children

Usually arises in cerebellum.

Imaging reveals cystic lesion with a mural nodule

Biopsy shows Rosenthal fibers (thick eosinophilic procesees). Astrocytes are GFAP positive

23
Q

Medulloblastoma

A

Highly malignant tumor of neuroectoderm of cerebellum in children.

Tumor grows rapidly and spreads via CSF

Histology reveals small blue cells and Homer Wright rosettes

24
Q

Ependymoma

A

Malignant tumor of ependymal cells in children.

Most commonly arises in 4th ventricle and may present with hydrocephalus.

Characteristic perivascular pseudorosettes

25
Q

Craniopharyngioma

A

Benign childhood tumor arising from epithelial remants of Rathke’s pouch

May present with bitemporal hemianopsia

26
Q

Charcot-Marie-Tooth disease

A

Progressive hereditary nerve disorder caused by the defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath.

Associated with scoliosis and foot deformities

27
Q

Sturge-Weber syndrome

A

Congenital somatic developmental anomaly of neural crest derivatives due to activating mutation of GNAQ gene.

Characterized by port-wine stain, retardation, early-onset glaucoma, and epilepsy.

28
Q

NF Type 1 (von Recklinghausen disaese)

A

Caused by mutated NF1 gene on chromosome 17 (negative regular of RAS).

Characterized by cafe-au-lait spots, Lisch nodules, pheochromocytomas, and neurofibromas (derived from neural crest cells)

29
Q

von Hippel-Lindau disease

A

AD, caused by mutated VHL on chromosome 3 (negative regulator of HIF and angiogenic growth factors).

Characterized by cavernous hemangiomas, bilateral renal cell carcinomas, hemangioblastomas, and pheochromocytomas

30
Q

Neurotransmitter changes with Alzheimer disease

A

Decreased ACh

31
Q

Neurotransmitter changes with anxiety

A

Increased norepinephrine

Decreased GABA and 5-HT

32
Q

Neurotransmitter changes with Huntington disease

A

Decreased norepinephrine and 5-HT

Increased dopamine

33
Q

Neurotransmitter changes with Parkinson disease

A

Decreased dopamine

Increased ACh and 5-HT

34
Q

Neurotransmitter changes with Schizophrenia

A

Increased dopamine

35
Q

Neurological manifestations of Vit B12 deficiency

A

Subacute combined degeneration. Degeneration of dorsal columns, lateral corticospinal tracts, and peripheral nerves.

Characterized by loss of position and vibration sense, ataxia, and spastic paresis.

36
Q

Medial medullary syndrome

A

Occlusion of anterior spinal artery (branch of veretebral artery)

  • Pyramid - contralateral spastic paresis
  • Medial lemniscus -contralateral loss of tactile, vibration and conscious proprioception
  • XII nucleus - ipsilateral flaccid paralysis of tongue
37
Q

Lateral medullary (Wallenberg) syndrome

A

Occlusion of posterior inferior cerebellar artery (PICA; a branch of veretebral artery)

  • Inferior cerebellar peduncle - ipsilateral limb ataxia
  • Vestibular nuclei - vertigo, contralateral nystagmus
  • Nucleus ambiguus (IX, X) - ipsilateral paralysis of larynx, pharynx, palate (dysarthria, dysphagia, loss of gag reflex)
  • Spinal V (V) - ipsilateral facial pain/T loss
  • Spinothalamic tract - contralateral body pain/T loss
  • Descending hypothalamics - ipsilateral Horner syndrome
38
Q

Medial pontine syndrome

A

Occlusion of paramedian artery (branch of basilar)

  • Corticospinal tract - contralateral spastic hemiparesis
  • Medial lemniscus - contralateral loss of tactile/position/vibration sensation
  • Fibers of VI - medial strabismus (diplopia on lateral gaze to affected side)
39
Q

Lateral pontine syndrome

A

Occlusion of anterior inferior cerebellar artery (branch of basilar)

  • Middle cerebellar peduncle - ipsilateral ataxia
  • Vestibular nuclei - vertigo, nausea, contralateral nystagmus
  • Facial nucleus - ipsilateral facial paralysis, loss of taste in anterior 2/3 tongue, lacrimation, salivation, corneal reflex, hyperacusis
  • Spinal trigeminal nucleus - ipsilateral facial pain/T loss
  • Spinothalamic tract - contralateral body pain/T loss
  • Cochlear nucleus - ipsilateral hearing loss
  • Descending hypothalamus - ipsilateral Horner syndrome
40
Q

Pontocerebellar angle syndrome

A

Usually caused by acoustic neuroma (schwannoma) of CN VIII

May involves CN VII and V

41
Q

Medial midbrain (Weber) syndrome

A

Occlusion of posterior cerebral artery

  • CN III - ipsilateral ocluomotor palsy (lateral strabismus, dilated pupil, ptosis, no accomodation or convergence)
  • Corticospinal tract - contralateral spastic hemiparesis
  • Corticobulbar tract - contralateral hemiparesis of lower face (upper face is innervated bilaterally)
42
Q

Dorsal midbrain (Parinaud) syndrome

A

Usually caused by pineal tumor compressing superior colliculi

  • **Superior colliculus/pretectal area - **paralysis of upward gaze
  • Cerebral aqueduct - noncommunicating hydrocephalus

Pathology (17 decks)

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