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168 Cards in this Set

  • Front
  • Back
What are the deficits in dementia?
Decreased cognitive ability, memory, or function, with intact consciousness
What are the top two most common cause of dementia in the elderly?
1. Alzheimer Disease
2. Multi-infarct
What can increase your risk of getting Alzheimer Disease?
- Down syndrome (Amyloid Precursor Protein - APP is on chr 21)
- Familial forms (10% of cases)
What are the types of familial Alzheimer Disease? What is affected?
Early onset:
- Amyloid Precursor Protein (APP) - chr 21
- Presenilin-1 - chr 14
- Presenilin-2 - chr 1

Late onset:
- ApoE4 - chr 19
What can protect against Alzheimer Disease?
ApoE2 (chr 19)
What are the gross changes in Alzheimer Disease?
- Widespread cortical atrophy
- Narrowing of gyri
- Widening of sulci
What hormone changes occur in Alzheimer Disease?
↓ ACh
What are the histologic change associated with Alzheimer Disease?
- Senile Plaques
- Neurofibrillary tangles
What are the contents and effect of a senile plaque?
- Extracellular β-amyloid core
- May cause amyloid angiopathy → intracranial hemorrhage
- Aβ (Amyloid-β) synthesized by cleaving Amyloid Precursor Protein (APP)

- Associated with Alzheimer Disease
What are the contents and effect of a neurofibrillary tangle?
- Intracellular hyperphosphorylated tau protein = insoluble cytoskeletal elements
- Tangles correlate with degree of dementia

- Associated with Alzheimer Disease
What type of dementia is associated with aphasia, parkinsonian aspects, and a change in personality?
Pick Disease (Frontotemporal Dementia)
What are the gross changes in Pick Disease (Frontotemporal Dementia)?
- Frontotemporal atrophy
- Spares parietal lobe and posterior 2/3 of superior temporal gyrus
What is the histologic finding in Pick Disease (Frontotemporal Dementia)?
Pick bodies = spherical tau protein aggregates
What type of dementia is associated with visual hallucinations followed by parkinsonian features?
Lewy Body Dementia
What defect is associated with Lewy Body Dementia?
α-Synuclein defect
What type of dementia is rapidly progressive (weeks to months) and causes myoclonus ("startle myoclonus")?
Creutzfeldt-Jakob disease
What is the cause and effect of Creutzfeldt-Jakob disease?
- Prions (PrP-c → PrP-sc sheet [β-pleated sheet resistant to proteases])
- Causes a spongiform cortex
- Dementia is rapidly progressive (weeks to months) and associated with myoclonus
What other diseases can cause dementia?
- Multi-infarct (2nd most common cause of dementia in elderly)
- Syphilis
- HIV
- Vitamins B1, B3, or B12 deficiency
- Wilson disease
- NPH
What vitamin deficiencies can cause dementia?
Vitamins B1, B3, or B12
What causes Multiple Sclerosis?
Auto-immune inflammation and demyelination of the CNS (brain and spinal cord)
What are the common symptoms in a patient with Multiple Sclerosis?
- Optic neuritis (sudden loss of vision)
- Internuclear Ophthalmoplegia
- Hemiparesis
- Hemisensory symptoms
- Bladder / bowel incontinence
Who is most commonly affected by Multiple Sclerosis? Clinical course?
- Most often women in 20s and 30s
- More common in whites
- Relapsing and remitting course
What is the classic triad of symptoms in Multiple Sclerosis?
SIN:
- Scanning speech
- Intention tremor (also Incontinence and Internuclear ophthalmoplegia)
- Nystagmus
What are the lab findings associated with Multiple Sclerosis?
Increased protein (IgG) in CSF
*Oligoclonal bands are diagnostic
What is the best way to diagnose Multiple Sclerosis? What findings do you look for?
MRI is the gold standard
- Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with destruction of axons
- Multiple white matter lesions separated in space and time
How do you treat Multiple Sclerosis?
- β-interferon
- Immunosuppression
- Natalizumab
- Symptomatic treatment for neurogenic bladder (catheter, muscarinic antagonists)
- Symptomatic treatment for spasticity (baclofen, GABA receptor agonist)
- Symptomatic treatment for pain (opioids)
What drugs are given to patients with Multiple Sclerosis regardless of symptoms?
- β-interferon
- Immunosuppression
- Natalizumab
What drugs are given to patients with Multiple Sclerosis with neurogenic bladder problems?
- Catheterization
- Muscarinic antagonists
What drugs are given to patients with Multiple Sclerosis with spasticity problems?
- Baclofen
- GABA receptor agonist
What drugs are given to patients with Multiple Sclerosis with pain problems?
Opioids
What is the most common variant of Guillain-Barré Syndrome?
Acute Inflammatory Demyelinating Polyradiculopathy
What causes Acute Inflammatory Demyelinating Polyradiculopathy?
Auto-immune condition that destroys Schwann cells → inflammation and demyelination of peripheral nerves and motor fibers
What are the consequences of the demyelination of the peripheral nerves and motor fibers in Acute Inflammatory Demyelinating Polyradiculopathy?
- Symmetric ascending muscle weakness and paralysis beginning in lower extremities
- Facial paralysis in 50% of cases
- Autonomic function may be severely affected (eg, cardiac irregularities, hypertension, or hypotension)
- Almost all survive, majority recover after weeks to months
What are the lab findings associated with Acute Inflammatory Demyelinating Polyradiculopathy?
- ↑ CSF protein
- Normal cell count (albuminocytologic dissociation)
- ↑ Protein → Papilledema
What is Acute Inflammatory Demyelinating Polyradiculopathy associated with / cause?
Infections:
- Campylobacter jejuni
- CMV

Leads to auto-immune attack of peripheral myelin due to molecular mimicry, inoculations, and stress, but no definitive link to pathogens
How do you treat a patient with Acute Inflammatory Demyelinating Polyradiculopathy?
- Respiratory support is critical until recovery
- Additional: plasmapheresis, IV immune globulins
What disease is associated with AIDS patients and destroys oligodendrocytes leading to demyelination of CNS? Cause?
Progressive Multifocal Leukoencephalopathy
- Associated with reactivation of latent JC virus infection
How common is Progressive Multifocal Leukoencephalopathy in AIDS patients?
2-4%
What is the prognosis for Progressive Multifocal Leukoencephalopathy?
- Rapidly progressive
- Usually fatal
What increases the risk of Progressive Multifocal Leukoencephalopathy?
- ↑ risk with Natalizumab
- More common in AIDS patients
What disease causes multi-focal perivenular inflammation and demyelination after infection or vaccinations?
Acute Disseminated Encephalomyelitis
What is the cause of Acute Disseminated Encephalomyelitis?
- Occurs after infection: commonly measles or VZV
- Or occurs after vaccine: rabies or smallpox
What are the consequences of Acute Disseminated Encephalomyelitis?
Multifocal perivenular inflammation and demyelination
What disease is due to an arylsulfatase A deficiency? What builds up?
Metachromatic Leukodystrophy
- Build up of sulfatides
What causes build up of sulfatides in Metachromatic Leukodystrophy? What does this lead to?
- Autosomal recessive deficiency of Arylsulfatase A
- Leads to impaired production of myelin sheath
What are the findings of a patient with Arylsulfatase A deficiency (Metachromatic Leukodystrophy)?
- Build up of sulfatides → impaired production of myelin sheath
- Central and peripheral demyelination
- Ataxia and dementia
What disease is related to defective production of proteins involved in the structure and function of peripheral nerves or myelin sheath?
Charcot-Marie-Tooth disease
What is wrong in Charcot-Marie-Tooth disease?
Hereditary motor and sensory neuropathy (HMSN)
- Progressive hereditary disorder related to defective production of proteins involved in structure and function of peripheral nerves or myelin sheath
- Typically autosomal dominant
What is Charcot-Marie-Tooth disease associated with?
- Scoliosis
- Foot deformities (high or flat arches)
What disease is associated with a deficiency of galactocerebrosidase? What builds up?
Krabbe Disease
- Build up of galactocerebroside and psychosine which destroy the myelin sheath
What are the clinical findings caused by a deficiency of galactocerebrosidase (Krabbe Disease)?
- Peripheral neuropathy
- Developmental delay
- Optic atrophy
- Globoid cells
What disease disrupts metabolism of very-long-chain fatty acids? Cause?
Adrenoleukodystrophy
- X-linked (typically affects males)
What are the clinical findings caused by a disruption of very-long-chain fatty acid metabolism / Adrenoleukodystrophy?
- Excessive build-up in nervous system, adrenal gland, ante testes
- Progressive disease that can lead to long-term coma/death and adrenal gland crisis
What is characterized by synchronized, high-frequency neuronal firing?
Seizures
How can you categorize seizures?
- Partial / focal vs generalized
- Simple vs complex
What is the difference between partial and generalized seizures?
- Partial / focal: affects 1 area of brain (but can generalize)
- Generalized: diffuse
What is the most common location for a partial seizure?
Medial temporal lobe
What typically precedes a partial seizure?
Seizure aura
What are the types of partial seizures? How do they differ?
- Simple Partial (consciousness intact): motor, sensory, autonomic, psychic
- Complex Partial (impaired consciousness)
What are the types of generalized seizures?
- Absence (petit mal)
- Myoclonic
- Tonic-clonic (grand mal)
- Tonic
- Atonic
What is epilepsy?
Disorder of recurrent seizures (febrile seizures are not epilepsy)
What is status epilepticus?
Continuous seizure for >30 minutes or recurrent seizures without regaining consciousness between seizures for >30 minutes
(Medical emergency)
What kind of seizure is characterized by a blank stare and causes no postictal confusion?
Absence (petit mal) - 3 Hz
What kind of seizure is characterized by quick, repetitive jerks?
Myoclonic (generalized seizure)
What kind of seizure is characterized by alternating stiffening and movement?
Tonic-Clonic (grand mal) generalized seizure
What kind of seizure is characterized by stiffening only?
Tonic generalized seizure
What kind of seizure is characterized by "dropping" (falling to floor) and is commonly mistaken for fainting?
Atonic generalized seizure
What are the common causes of seizures in a child?
- Genetic
- Infection (febrile)
- Trauma
- Congenital
- Metabolic
What are the common causes of seizures in an adult?
- Tumors
- Trauma
- Stroke
- Infection
What are the common causes of seizures in the elderly?
- Stroke
- Tumor
- Trauma
- Metabolic
- Infection
What causes a headache?
Irritation of structures such as the dura, cranial nerves, or extracranial structures
What are the types of headaches?
- Cluster
- Tension
- Migraine
How do the types of headaches differ in terms of localization?
Unilateral:
- Cluster
- Migraine

Bilateral:
- Tension
How do the types of headaches differ in terms of duration?
- Cluster: 15 min - 3 hours (repetitive)
- Tension: >30 min (typically 4-6 hours); constant
- Migraine: 4-72 hours
Which type of headache causes repetitive brief headaches with excruciating periorbital pain with lacrimation and rhinorrhea?
Cluster Headache
Which type of headache may induce Horner syndrome?
Cluster Headache
Which type of headache causes a steady pain without photophobia, phonophobia, or auras?
Tension Headache
Which type of headache causes a pulsating pain with nausea, photophobia, and/or phonophobia?
Migraine
Which type of headache is due to irritation on CN V, meninges, or blood vessels? What is released?
Migraine
- Release of substance P, CGRP, and vasoactive peptides
What do Cluster Headaches cause? Treatment?
- Repetitive brief headaches
- Excruciating periorbital pain with lacrimation and rhinorrhea
- May induce Horner syndrome
- More common in males

Treatment:
- Inhaled O2
- Sumatriptan
What do Tension Headaches cause? Treatment?
- Steady pain
- No photophobia, phonophobia, or auras

Treatment:
- Analgesics, NSAIDs, or acetaminophen
- Amitriptyline for chronic pain
What do Migraine Headaches cause? Treatment?
- Pulsating pain with nausea, photophobia, or phonophobia
- May have "aura"
- Due to irritation of CN V, meninges, or blood vessels (release of substance P, CGRP, and vasoactive peptides)

Treatment:
- Abortive therapies (eg, triptans or NSAIDs)
- Prophylactic therapies (eg, propranolol, topiramate, CCBs, or amitriptyline
What mnemonic helps you remember characteristics of migraine headaches?
POUND
- Pulsatile
- One day duration
- Unilateral
- Nausea
- Disabling
What are the abortive therapies used for migraine?
- Triptans (eg, Sumatriptan)
- NSAIDs
What are the prophylactic therapies used for migraine?
- Propranolol
- Topiramate
- Calcium channel blockers
- Amitriptyline
What are some other causes of head pain (not a headache)?
- Subarachnoid hemorrhage ("worst headache of my life")
- Meningitis
- Hydrocephalus
- Neoplasia
- Arteritis
How can you distinguish a cluster headache from trigeminal neuralgia?
Depends on duration:
- Cluster headache: 15 min - 3 hours (repetitive)
- Trigeminal neuralgia: repetitive shooting pain in distribution of CN V that lasts typically for <1 minute
What is the sensation of spinning while actually stationary?
Vertigo
- Subtype of "dizziness" but distinct from "lightheadedness"
What are the types of vertigo? Which is more common?
- Peripheral vertigo (more common)
- Central vertigo
What causes peripheral vertigo?
Inner ear etiology (eg, semicircular canal debris, vestibular nerve infection, Ménière disease
What causes central vertigo?
Brain stem or cerebellar lesion (eg, stroke affecting vestibular nuclei or posterior fossa tumor)
What are the findings in peripheral vertigo?
Positional testing → delayed horizontal nystagmus
What are the findings in central vertigo?
- Directional change of nystagmus
- Skew deviation
- Diplopia
- Dysmetria
- Postional testing → immediate nystagmus in any direction; may change directions
- Focal neurological findings
What disease is characterized by a port-wine stain of the face, seizures/epilepsy, intellectual disability, and early-onset glaucoma?
Sturge-Weber Syndrome

STURGE:
- Sporadic port-wine Stain
- Tram track Ca2+ (opposing gyri)
- Unilateral
- Retardation
- Glaucoma and GNAQ gene
- Epilepsy
What is the cause of Sturge-Weber Syndrome?
- Congenital, non-inherited (somatic), developmental anomaly of neural crest derivatives (mesoderm/ectoderm)
- Due to activating mutation of GNAQ gene
What causes the port-wine stain seen in Sturge-Weber Syndrome?
What causes the port-wine stain seen in Sturge-Weber Syndrome?
Small (capillary-sized) blood vessels have developmental anomalies → port-wine stain of face (non-neoplastic "birthmark" in CN V1/V2 distribution)
Small (capillary-sized) blood vessels have developmental anomalies → port-wine stain of face (non-neoplastic "birthmark" in CN V1/V2 distribution)
What causes the seizures/epilepsy seen in Sturge-Weber Syndrome?
Ipsilateral leptomeningeal angioma → seizures / epilepsy
Ipsilateral leptomeningeal angioma → seizures / epilepsy
What causes the glaucoma in Sturge-Weber Syndrome?
Episcleral hemangioma → ↑ intraocular pressure → early onset glaucoma
What genetic change causes Sturge-Weber Syndrome?
Activating mutation of GNAQ gene
What disease causes hamartomas in the CNS and skin, angiofribromas, mitral regurgitation, ash-leaf spots, cardiac rhabdomyoma, mental retardation, renal angiomyolipoma, seizures, and Shagreen patches?
Tuberous Sclerosis
What mnemonic helps you remember the characteristics of Turberous Sclerosis?
HAMARTOMAS:
- Hamartomas in CNS and skin
- Angiofibromas (C)
- Mitral regurgitation
- Ash-leaf spots
- Rhabdomyoma (cardiac)
- Tuberous sclerosis
- autosomal dOminant
- Mental retardation
- Angiomyolipoma (renal) (D)
- Seizures and Shagr...
HAMARTOMAS:
- Hamartomas in CNS and skin
- Angiofibromas (C)
- Mitral regurgitation
- Ash-leaf spots
- Rhabdomyoma (cardiac)
- Tuberous sclerosis
- autosomal dOminant
- Mental retardation
- Angiomyolipoma (renal) (D)
- Seizures and Shagreen patches
What is there increased incidence of in Turberous Sclerosis?
- Subependymal astrocytomas
- Ungual fibromas
Which disease is associated with café-au-lait spots, Lisch nodules, neurofibromas in the skin, optic gliomas, and pheochromocytomas?
Neurofibromatosis Type 1 (von Recklinghausen disease)
What is the cause of Neurofibromatosis Type 1 (von Recklinghausen disease)?
- Mutated NF1 tumor suppressor gene (neurofibromin - a negative regulator of Ras)
- On chromosome 17
What does a mutated NF1 tumor suppressor gene (chr 17) cause? Clinical symptoms?
Neurofibromatosis Type 1 (von Recklinghausen disease)
- Café-au-lait spots (E)
- Lisch nodules (pigmented iris hamartomas) (F)
- Neurofibromas in skin
- Optic gliomas
- Pheochromocytoma
Neurofibromatosis Type 1 (von Recklinghausen disease)
- Café-au-lait spots (E)
- Lisch nodules (pigmented iris hamartomas) (F)
- Neurofibromas in skin
- Optic gliomas
- Pheochromocytoma
What are skin tumors of NF-1 derived from?
Neural crest cells
What disease is associated with cavernous hemangiomas in skin, mucosa, and organs; bilateral renal cell carcinomas; hemangioblastoma in retina, brain stem, cerebellum; and pheochromocytoma?
von Hippel-Lindau Disease
What is the cause of von Hippel-Lindau Disease?
- Autosomal dominant mutation in VHL tumor suppressor gene on chr 3
- Results in constitutive expression of HIF (transcription factor) and activation of angiogenic growth factors
What disease is caused by the autosomal dominant mutation in the VHL tumor suppressor gene on chr 3? What does this mutation cause?
von Hippel-Lindau Disease
- Mutation results in constitutive expression of HIF (transcription factor) and activation of angiogenic growth factors
- Cavernous hemangiomas in skin, mucosa, and organs
- Bilateral renal cell carcinoma
- Hemangiobl...
von Hippel-Lindau Disease
- Mutation results in constitutive expression of HIF (transcription factor) and activation of angiogenic growth factors
- Cavernous hemangiomas in skin, mucosa, and organs
- Bilateral renal cell carcinoma
- Hemangioblastoma (high vascularity with hyperchromatic nuclei (G)) in retina, brainstem, and cerebellum (H)
- Pheochromocytoma
Where are there cavernous hemangiomas in von Hippel-Lindau Disease?
- Skin
- Mucosa
- Organs
Where are there hemangioblastomas in von Hippel-Lindau Disease?
- Retina
- Brainstem
- Cerebellum
What is wrong with this little girl? Associated with what disease?
What is wrong with this little girl? Associated with what disease?
Port-wine stain on face 
- Caused by Sturge Weber Syndrome
Port-wine stain on face
- Caused by Sturge Weber Syndrome
What is wrong with this brain? Associated with what disease?
What is wrong with this brain? Associated with what disease?
Ipsilateral leptomeningeal angioma → seizures / epilepsy
- Caused by Sturge Weber Syndrome
Ipsilateral leptomeningeal angioma → seizures / epilepsy
- Caused by Sturge Weber Syndrome
What is wrong with this little boy? Associated with what disease?
What is wrong with this little boy? Associated with what disease?
Angiofibromas of the face
- Caused by Tuberous Sclerosis
Angiofibromas of the face
- Caused by Tuberous Sclerosis
What aspect of Tuberous Sclerosis has this histology?
What aspect of Tuberous Sclerosis has this histology?
Renal Angiomyolipoma
Renal Angiomyolipoma
What is this skin finding? Associated with what disease?
What is this skin finding? Associated with what disease?
Café-au-lait spot
- Caused by Neurofibromatosis type I (von Recklinghausen disease)
Café-au-lait spot
- Caused by Neurofibromatosis type I (von Recklinghausen disease)
What is this eye finding? Associated with what disease?
What is this eye finding? Associated with what disease?
Lisch nodule (pigmented iris hamartoma)
- Caused by Neurofibromatosis type I (von Recklinghausen disease)
Lisch nodule (pigmented iris hamartoma)
- Caused by Neurofibromatosis type I (von Recklinghausen disease)
What aspect of von Hippel Lindau disease has this histology?
What aspect of von Hippel Lindau disease has this histology?
Hemangioblastomas (high vascularity with hyperchromatic nuclei)
Hemangioblastomas (high vascularity with hyperchromatic nuclei)
What is this brain finding? Associated with what disease?
What is this brain finding? Associated with what disease?
Hemangioblastoma in cerebellum
- Caused by von Hippel Lindau disease
Hemangioblastoma in cerebellum
- Caused by von Hippel Lindau disease
What are the adult primary brain tumors?
- Glioblastoma multiforme (grade IV astrocytoma)
- Meningioma
- Hemangioblastoma
- Schwannoma
- Oligodendroglioma
- Pituitary adenoma
What tumor is found in the cerebral hemispheres and is known for crossing the corpus callosum?
What tumor is found in the cerebral hemispheres and is known for crossing the corpus callosum?
Glioblastoma Multiforme (grade IV astrocytoma)
Glioblastoma Multiforme (grade IV astrocytoma)
What is the prognosis of Glioblastoma Multiforme (grade IV astrocytoma)? How common?
- Common, in adults
- Highly malignant with ~1 year median survival
What is the appearance of a Glioblastoma Multiforme (grade IV astrocytoma)?
- Found in cerebral hemispheres
- Can cross corpus callosum ("butterfly glioma")
What adult primary brain tumor will stain positively for GFAP? Why?
Glioblastoma Multiforme (grade IV astrocytoma) - astrocytes are stained with GFAP
What is the histologic appearance of Glioblastoma Multiforme (grade IV astrocytoma)?
"Pseudopalisading" pleomorphic tumor cells - border central areas of necrosis and hemorrhage
"Pseudopalisading" pleomorphic tumor cells - border central areas of necrosis and hemorrhage
Which type of adult brain tumor occurs in the convexities of hemispheres (near the surfaces of the brain) and parasagittal region?
Meningioma
Meningioma
What kind of cells are involved in a Meningioma? Significance for location of tumor?
- Arises from arachnoid cells
- Extra-axial (external to brain parenchyma)
- May have dural attachment ("tail")
- Arises from arachnoid cells
- Extra-axial (external to brain parenchyma)
- May have dural attachment ("tail")
What are the symptoms and prognosis for a Meningioma?
- Typically benign
- Often asymptomatic
- May present with seizures or focal neurologic signs
How do you treat Meningioma?
Resection and/or radiosurgery
What is the histologic appearance of a Meningioma?
- Spindle cells, concentrically arranged in a whorled pattern
- Psammoma bodies (laminated calcifications)
- Spindle cells, concentrically arranged in a whorled pattern
- Psammoma bodies (laminated calcifications)
What type of adult brain tumor is often cerebellar and is associated with von Hippel-Lindau syndrome when found with retinal angiomas?
Hemangioblastoma
Hemangioblastoma
What brain tumor can lead to polycythemia? How?
Hemangioblastoma - can produce erythropoietin → 2° polycythemia
What is the histologic appearance of a Hemangioblastoma?
Closely arranged, thin-walled capillaries with minimal interleaving parenchyma
Closely arranged, thin-walled capillaries with minimal interleaving parenchyma
Which type of adult brain tumor is often found at the cerebellopontine angle and can be localized to CN VIII? Origin of cells?
Schwannoma (if localized to CN VIII it is an acoustic schwannoma / acoustic neuroma)
- Schwann cell origin
Schwannoma (if localized to CN VIII it is an acoustic schwannoma / acoustic neuroma)
- Schwann cell origin
Which adult brain tumor is S-100 (+)?
Schwannoma
How do you treat a Schwannoma?
Resected or treated with stereotactic radiosurgery
What should you think of if you see bilateral acoustic Schwannomas?
NF-2
Which adult brain tumor is often found in the frontal lobes?
Oligodendroglioma
Oligodendroglioma
What is the histologic appearance of an Oligodendroglioma?
- Chicken-wire capillary pattern
- Oligodendrocytes = "fried egg" cells with round nuclei and clear cytoplasm
- Often calcified
- Chicken-wire capillary pattern
- Oligodendrocytes = "fried egg" cells with round nuclei and clear cytoplasm
- Often calcified
What type of adult brain tumor can put pressure on the optic chiasm causing bitemporal hemianopia?
What type of adult brain tumor can put pressure on the optic chiasm causing bitemporal hemianopia?
Pituitary Adenoma (most commonly a prolactinoma)
Pituitary Adenoma (most commonly a prolactinoma)
What are the possible sequelae of a pituitary adenoma?
Hyper or hypo-pituitarism
What is the location of the adult brain tumors?
- Glioblastoma Multiforme: cerebral hemispheres and corpus callosum
- Meningioma: external to brain parenchyma
- Hemangioblastoma: cerebellar
- Schwannoma: cerebellopontine angle, may localize to CN VIII
- Oligodendroglia: frontal lobes
- Pituitary adenoma: pituitary / optic chiasm
What are the types of childhood primary brain tumors?
- Pilocytic (low-grade) astrocytoma
- Medulloblastoma
- Ependymoma
- Craniopharyngioma
Which type of childhood brain tumor is GFAP (+)?
Pilocytic (low-grade) Astrocytoma
Where are Pilocytic (low-grade) Astrocytoma usually found? Prognosis?
- Most often in posterior fossa (eg, cerebellum), but can be supratentorial
- Benign with good prognosis
- Most often in posterior fossa (eg, cerebellum), but can be supratentorial
- Benign with good prognosis
Which type of childhood brain tumor is associated with Rosenthal fibers (eosinophilic, corkscrew fibers)?
Pilocytic (low-grade) Astrocytoma
Pilocytic (low-grade) Astrocytoma
What is the gross and histologic appearance of Pilocytic (low-grade) Astrocytoma?
- Usually well circumscribed
- Rosenthal fibers: eosinophilic, corkscrew fibers
- Cystic + solid
Which type of childhood brain tumor is a form of primitive neuroectodermal tumor?
Medulloblastoma
What can a Medulloblastoma cause?
- Can compress the 4th ventricle → hydrocephalus
- Can send "drop metastases" to spinal cord
What type of childhood brain tumor is associated with Homer-Wright rosettes? Prognosis?
Medulloblastoma - highly malignant
What is the gross and histologic appearance of Medulloblastoma?
- Solid cerebellar tumor 
- Homer-Wright rosettes
- Small blue cells
- Solid cerebellar tumor
- Homer-Wright rosettes
- Small blue cells
What type of childhood brain tumor is derived from ependymal cells? Prognosis?
Ependymoma - poor prognosis
What can an Ependymoma cause?
Most commonly found in 4th ventricle so it can cause hydrocephalus
What type of childhood brain tumor is associated with perivascular rosettes
Ependymoma
Ependymoma
What is the gross and histologic appearance of an Ependymoma?
- Commonly in 4th ventricle
- Perivascular rosettes
- Rod-shaped blepharoblasts (basal ciliary bodies) found near nucleus
- Commonly in 4th ventricle
- Perivascular rosettes
- Rod-shaped blepharoblasts (basal ciliary bodies) found near nucleus
What type of childhood brain tumor may be confused with a pituitary adenoma? Source?
Craniopharyngioma - derived from remnants of Rathke pouch
What is the prognosis and clinical syndrome caused by Craniopharyngioma?
- Benign tumor
- May be confused with pituitary adenoma because they both cause bitemporal hemianopia
What is the most common childhood supratentorial brain tumor?
Craniopharyngioma
What is the histologic appearance of a Craniopharyngioma?
Calcification is common (tooth-enamel like)
Calcification is common (tooth-enamel like)
What are the types of herniation syndromes?
1. Cingulate (subfalcine) herniation under falx cerebri
2. Downward transtentorial (central) herniation
3. Uncal herniation
4. Cerebellar tonsillar herniation into foramen magnum
1. Cingulate (subfalcine) herniation under falx cerebri
2. Downward transtentorial (central) herniation
3. Uncal herniation
4. Cerebellar tonsillar herniation into foramen magnum
What type of herniation can compress the anterior cerebral artery?
Cingulate (subfalcine) herniation under falx cerebri (#1)
Cingulate (subfalcine) herniation under falx cerebri (#1)
What type of herniation can compress the ipsilateral CN III causing a blown pupil and down and out gaze?
Uncal Herniation (#3) - medial temporal lobe
Uncal Herniation (#3) - medial temporal lobe
What type of herniation can compress the posterior cerebral artery causing contralateral homonymous hemianopsia)?
Uncal Herniation (#3) - medial temporal lobe
Uncal Herniation (#3) - medial temporal lobe
What type of herniation can compress the contralateral crus cerebri causing ipsilateral paralysis / false localization sign?
Uncal Herniation (#3) - medial temporal lobe
Uncal Herniation (#3) - medial temporal lobe
What type of herniation can compress the brainstem, inhibiting respiration, and possibly causing coma and death?
Cerebellar tonsillar herniation into the foramen magnum (#4)
Cerebellar tonsillar herniation into the foramen magnum (#4)
What are the potential consequences of a cingulate (subfalcine) herniation under the falx cerebri?
Can compress anterior cerebral artery
What are the potential consequences of an uncal herniation?
Compresses:
- Ipsilateral CN III → blown pupil and down and out gaze
- Ipsilateral PCA → contralateral homonymous hemianopsia
- Contralateral crus cerebri → ipsilateral paralysis, "false localization sign"
What are the potential consequences of a cerebellar tonsillar herniation into the foramen magnum?
Coma and death result when these herniations compress the brain stem (and inhibit respiration)