Central Nervous System Flashcards
Pathoma CNS Chp 17
Anencephaly
Pathology:
Symptoms/Signs:
Labs:
Path:
A NTD of the cranial end causing absence of a skull & brain.
Signs:
1) Frog-fetus app
2) Maternal polyhydramnios
Labs:
Low Folate levels
High AFP
Path:
A NTD of the cranial end causing absence of a skull & brain.
Signs:
1) Frog-fetus app
2) Maternal polyhydramnios
Labs:
Low Folate levels
High AFP
Anencephaly
Spina bifida occulta
Pathology:
Symptoms/Signs:
Labs:
Path:
A NTD of the caudal end causing a dimple or patch of hair over the spinal defect
Signs:
1) Dimple or hairy patch on spine
Labs:
Low Folate
High AFP
Path:
A NTD of the caudal end causing a dimple or patch of hair over the spinal defect
Signs:
1) Dimple or hairy patch on spine
Labs:
Low Folate
High AFP
Spina bifida occulta
Spina bifida
Pathology:
Symptoms/Signs:
Labs:
Patho:
A NTD of the caudal end causing a cystic protrusion along the spine
Signs:
1) Meningocele (meninges)
2) Meningomyelocele (meninges & spinal cord)
Labs:
Low Folate
High AFP
Patho:
A NTD of the caudal end causing a cystic protrusion along the spine
Signs:
1) Meningocele (meninges)
2) Meningomyelocele (meninges & spinal cord)
Labs:
Low Folate
High AFP
Spina bifida
Cerebral Aqueduct Stenosis
Pathology:
Symptoms/Signs:
Labs:
Patho:
Congenital stenosis of the aqueduct that drains the 3rd into the 4th ventricle resulting in CSF accumulation in the ventricular spaces
Signs:
1) Enlarged head circumference with dilated ventricles
Labs:
Normal CSF production from choroid plexus
Patho:
Congenital stenosis of the aqueduct that drains the 3rd into the 4th ventricle resulting in CSF accumulation in the ventricular spaces
Signs:
1) Enlarged head circumference with dilated ventricles
Labs:
Normal CSF production from choroid plexus
Cerebral Aqueduct Stenosis
Arnold-Chiari (II) Malformation
Pathology:
Symptoms/Signs:
Associated conditions:
Path:
Extension of the cerebellar tonsils into the foramen magnum obstructs CSF
Signs:
1) Hydrocephalus
Ass:
Meningomyelocele
Syringomyelia
Path:
Extension of the cerebellar tonsils into the foramen magnum obstructs CSF
Signs:
1) Hydrocephalus
Ass:
Meningomyelocele
Syringomyelia
Arnold-Chiari (II) Malformation
Dandy-Walker Malformation
Pathology:
Symptoms/Signs:
Path:
Failure of the cerebellar vermis to form (no where for CSF to go!)
Signs:
1) Massive dilation of the 4th ventricle
2) Absent cerebellum
3) Hydrocephalus
Path:
Failure of the cerebellar vermis to form (no where for CSF to go!)
Signs:
1) Massive dilation of the 4th ventricle
2) Absent cerebellum
3) Hydrocephalus
Dandy-Walker Malformation
Syringomyelia
Pathology:
Symptoms/Signs:
Associated conditions:
Path:
Cystic degeneration of the spinal cord (C8-T1) due to trauma or Arnold-Chiari (II) malformation
Signs:
1) Sensory loss or pain & temperature in cape-like distribution (anterior white commissure)
2) Spared fine touch (dorsal column)
Complication:
Syrinx expansion (compression of the upper spinal tracts) results in:
1) Damaged neurons of the anterior horn
-Muscle atrophy/weakness
- Decreased muscle tone/reflexes
2) Damaged lateral horn of hypothalamospinal tract aka Horner syndrome (ptosis, miosis, & anhidrosis)
Path:
Cystic degeneration of the spinal cord (C8-T1) due to trauma or Arnold-Chiari (II) malformation
Signs:
1) Sensory loss or pain & temperature in cape-like distribution (anterior white commissure)
2) Spared fine touch (dorsal column)
Complication:
Syrinx expansion (compression of the upper spinal tracts) results in:
1) Damaged neurons of the anterior horn
-Muscle atrophy/weakness
- Decreased muscle tone/reflexes
2) Damaged lateral horn of hypothalamospinal tract aka Horner syndrome (ptosis, miosis, & anhidrosis)
Syringomyelia
Poliomyelitis
Pathology:
Symptoms/Signs:
Path:
Damaged anterior motor horn due to poliovirus inf
Signs:
1) Lower motor neuron signs
- Flaccid paralysis,
- Muscle atrophy/fasciculations/weakness
- Impaired reflexes & -ve Babinski sign (downfacing toes)
Path:
Damaged anterior motor horn due to poliovirus inf
Signs:
1) Lower motor neuron signs
- Flaccid paralysis,
- Muscle atrophy/fasciculations/weakness
- Impaired reflexes & -ve Babinski sign (downfacing toes)
Poliomyelitis
Werdnig-Hoffman Disease
Pathology:
Symptoms/Signs:
Path:
Degenerated anterior motor horn (auto rec)
Signs:
1) Floppy baby
2) Death within a few years
Path:
Degenerated anterior motor horn (auto rec)
Signs:
1) Floppy baby
2) Death within a few years
Werdnig-Hoffman Disease
Amyotrophic Lateral Sclerosis (ALS)
Pathology:
Symptoms/Signs:
Causes:
Path:
Degeneration of the upper & lower motor neurons of the corticospinal tract that lacks sensory impairment that appears sporadically in middle aged adults
Signs:
1) Early sign (atrophy & weakness of the hands
2) Anterior horn degeneration (LMN)
- Flaccid paralysis,
- Muscle atrophy/fasciculations/weakness
- Impaired reflexes & -ve Babinski sign (downfacing toes)
3) Lateral corticospinal tract degeneration (UMN)
- Spastic paralysis & Hyperreflexia
- Increased muscle tone
- +ve Babinski
Causes:
1) Zinc-copper superoxide dismutase (SODI) in familial cases which leads to free-radical injuries in neurons
Path:
Degeneration of the upper & lower motor neurons of the corticospinal tract that lacks sensory impairment that appears sporadically in middle aged adults
Signs:
1) Early sign (atrophy & weakness of the hands
2) Anterior horn degeneration (LMN)
- Flaccid paralysis,
- Muscle atrophy/fasciculations/weakness
- Impaired reflexes & -ve Babinski sign (downfacing toes)
3) Lateral corticospinal tract degeneration (UMN)
- Spastic paralysis & Hyperreflexia
- Increased muscle tone
- +ve Babinski
Causes:
1) Zinc-copper superoxide dismutase (SODI) in familial cases which leads to free-radical injuries in neurons
Amyotrophic Lateral Sclerosis (ALS)
Amyotrophic Lateral Sclerosis (ALS)
Pathology:
Symptoms/Signs:
Associated conditions:
Path:
Degeneration of the cerebellum & spinal cord tracts due to an unstable GAA repeat in the Frataxin gene resulting in accumulation of iron in mitochondria & free radicals
Signs:
1) Cerebellum degeneration (Ataxia)
2) Spinal cord degenerations
- Loss of vibratory sense & proprioception
- Muscle weakness & loss of deep tendon reflex
Ass:
Hypertrophic cardiomyopathy
Path:
Degeneration of the cerebellum & spinal cord tracts due to an unstable GAA repeat in the Frataxin gene resulting in accumulation of iron in mitochondria & free radicals
Signs:
1) Cerebellum degeneration (Ataxia)
2) Spinal cord degenerations
- Loss of vibratory sense & proprioception
- Muscle weakness & loss of deep tendon reflex
Ass:
Hypertrophic cardiomyopathy
Amyotrophic Lateral Sclerosis (ALS)
Bacterial meningitis
Pathology:
Causes:
Diagnostic test: & Labs:
Symptoms/Signs:
Complications:
Path:
Inflammation of the leptomeninges
Causes:
1) Unvaccinated children
- Group B strep
- Listeria monocytogenes
- E. coli
2) Kids/Teens
- N. meningitis
3) Adults/Elderly
- S. pneumoniae
Tests & Labs:
1) LP (L4-L5) with gram stain & culture
2) Kernig sign
3) Neutrophils & low CSF Glucose
Signs:
1) Headache, Nuchal rigidity, & fever
2) Photophobia
3) Altered mental status
4) Vomiting
Complications:
1) Death (herniation & cerebral edema)
2) Hydrocephalus, hearing loss, & seizures
Path:
Inflammation of the leptomeninges
Causes:
1) Unvaccinated children
- Group B strep
- Listeria monocytogenes
- E. coli
2) Kids/Teens
- N. meningitis
3) Adults/Elderly
- S. pneumoniae
Tests & Labs:
1) LP (L4-L5) with gram stain & culture
2) Kernig sign
3) Neutrophils & low CSF Glucose
Signs:
1) Headache, Nuchal rigidity, & fever
2) Photophobia
3) Altered mental status
4) Vomiting
Complications:
1) Death (herniation & cerebral edema)
2) Hydrocephalus, hearing loss, & seizures
Bacterial meningitis
Pathology:
Viral meningitis
Pathology:
Causes:
Diagnostic test: & Labs:
Symptoms/Signs:
Path:
Inflammation of the leptomeninges
Causes:
1) Unvaccinated neonates
- H. influenza
2) Children via fecal-oral route
- Coxsackie virus
Tests & Lab:
LP (L4-L5)
Lymphocytes with normal CSF Glucose
Signs:
1) Headache, Nuchal rigidity, & fever
2) Photophobia
3) Altered mental status
4) Vomiting
Path:
Inflammation of the leptomeninges
Causes:
1) Unvaccinated neonates
- H. influenza
2) Children via fecal-oral route
- Coxsackie virus
Tests & Lab:
LP (L4-L5)
Lymphocytes with normal CSF Glucose
Signs:
1) Headache, Nuchal rigidity, & fever
2) Photophobia
3) Altered mental status
4) Vomiting
Viral meningitis
Fungal Meningitis
Pathology/Causes:
Diagnostic test: & Labs:
Symptoms/Signs:
Path:
Inflammation of meninges in immunocompromised patients (HIV/AIDS)
Test/Labs:
LP & Staining
Lymphocytes & low CSF Glucose
Signs:
1) Headache, Nuchal rigidity, & fever
2) Photophobia
3) Altered mental status
4) Vomiting
Path:
Inflammation of meninges in immunocompromised patients (HIV/AIDS)
Test/Labs:
LP & Staining
Lymphocytes & low CSF Glucose
Signs:
1) Headache, Nuchal rigidity, & fever
2) Photophobia
3) Altered mental status
4) Vomiting
Fungal Meningitis
Global Cerebral Ischemia
Pathology:
Causes:
Symptoms/Signs:
Path:
Ischemia of the brain
Causes:
1) Atherosclerosis (low perfusion)
2) Anemia (Chronic hypoxia)
3) Insulinoma (repeated episodes of hypoglycemia)
Signs:
1) Mild global ischemia (transient confusion with a prompt recovery)
2) Severe global ischemia (infarcts in watershed areas that damage vulnerable areas)
Path:
Ischemia of the brain
Causes:
1) Atherosclerosis (low perfusion)
2) Anemia (Chronic hypoxia)
3) Insulinoma (repeated episodes of hypoglycemia)
Signs:
1) Mild global ischemia (transient confusion with a prompt recovery)
2) Severe global ischemia (infarcts in watershed areas that damage vulnerable areas)
Global Cerebral Ischemia
Severe Global cerebral ischemia of the cerebral cortex pyramidal neurons causes what?
Laminar necrosis
Severe Global cerebral ischemia of the Hippocampus pyramidal neurons causes what?
Long term memory loss
Severe Global cerebral ischemia of the cerebellum Purkinje neurons causes what?
Impaired sensory perception & motor function
Thrombotic stroke
Pathology:
Histological finding:
Path:
Rupture of an atherosclerotic plaque (branch point of ICA & MCA in the circle of Willis)
Histo:
Pale infarct at the cortex’s periphery
Embolic stroke
Pathology:
Histological finding:
Path:
Thromboemboli from Afib (left heart) that obstructs the MCA
Histo:
Hemorrhagic infarct
Path:
Rupture of an atherosclerotic plaque (branch point of ICA & MCA in the circle of Willis)
Histo:
Pale infarct
Thrombotic stroke
Path:
Thromboemboli from Afib (left heart) that obstructs the MCA
Histo:
Hemorrhagic infarct
Embolic stroke
Lacunal stroke
Pathology:
Histological finding:
Path:
Secondary to hyaline arteriosclerosis due to HTN or diabetes that obstructs the lenticulostriate vessels
Histo:
Small cystic areas of infarction
1) Internal capsule causes pure motor stroke
2) Thalamus causes a pure sensory stroke
Path:
Secondary to hyaline arteriosclerosis due to HTN or diabetes that obstructs the lenticulostriate vessels
Histo:
Small cystic areas of infarction
1) Internal capsule causes pure motor stroke
2) Thalamus causes a pure sensory stroke
Lacunal stroke
Pathology:
Histological finding:
Ischemic stroke
Pathology:
Histological finding:
Path:
Liquefactive necrosis
Histo:
12hrs = eosinophilic change
24hrs = coagulative necrosis
1-3 days = neutrophils
4-7 days = microglial cells
2-3 wks = tissue granulation
Intracerebellar hemorrhage
Pathology:
Cause:
Signs:
Path:
Bleeding into parenchyma that primarily impacts the basal ganglia
Cause:
Rupture of A Charcot-Bouchard microaneurysm in the lenticulostriate vessels (a complication of HTN)
Signs:
1) Severe headache
2) N/V
3) Eventual coma
Path:
Bleeding into parenchyma that primarily impacts the basal ganglia
Cause:
Rupture of A Charcot-Bouchard microaneurysm in the lenticulostriate vessels (a complication of HTN)
Signs:
1) Severe headache
2) N/V
3) Eventual coma
Intracerebellar hemorrhage
Subarachnoid hemorrhage
Pathology:
Cause:
Signs:
Tests/Histology:
Path:
Bleeding into the subarachnoid space
Cause:
Rupture of a berry aneurysm in the ACA
Signs:
1) Worst headache of my life
2) Nuchal rigidity
Tests/Histo:
LP showing xanthochromia (yellow CSF)
Path:
Bleeding into the subarachnoid space
Cause:
Rupture of a berry aneurysm in the ACA
Signs:
1) Worst headache of my life
2) Nuchal rigidity
Tests/Histo:
LP showing xanthochromia (yellow CSF)
Subarachnoid hemorrhage
Epidural hematoma
Pathology:
Causes:
Tests:
Complication:
Path:
Bleeding separates the dura from skull
Causes:
Fractured temporal bone with ruptured middle meningeal artery (fast bleed!!!)
Tests:
CT shows a lens shaped lesion (lemon)
Complication:
Herniation (Lethal)
Path:
Bleeding separates the dura from skull
Causes:
Fractured temporal bone with ruptured middle meningeal artery (fast bleed!!!)
Tests:
CT shows a lens shaped lesion (lemon)
Complication:
Herniation (Lethal)
Epidural hematoma
Subdural hematoma
Pathology:
Causes:
Symptoms/signs:
Diagnostic tests:
Complication:
Path:
Bleeding beneath the dura
Causes:
Trauma or age-related cerebral atrophy can both tear the bridging veins (slow bleed)
Signs:
1) Progressive neurological deficits
Tests:
Crescent lesion on CT (banana)
Complication:
Herniation (lethal)
Path:
Bleeding beneath the dura
Causes:
Trauma or age-related cerebral atrophy can both tear the bridging veins (slow bleed)
Signs:
1) Progressive neurological deficits
Tests:
Crescent lesion on CT (banana)
Complication:
Herniation (lethal)
Subdural hematoma
Multiple sclerosis:
Pathology:
Causes/Etiology:
Symptoms/Signs:
Diagnostic tests/Histological findings:
Treatments:
-Acute vs Long term
Path:
Autoimmune destruction of CNS myelin & oligodendrocytes (Demyelination)
Causes/Etiology:
HLA-DR2 genotypes & women (20-30yrs)
Signs:
1) Blurry vision in one eye (optic nerve)
2) Vertigo & drunk-like speech (brainstem)
3) Intranuclear ophthalmoplegia
(medial longitudinal fasciculitis “eyes can’t look to same side at the same time”)
4) Hemiparesis/Unilateral sensation loss (white matter)
5) Sensation loss/weakness in the lower extremities (spinal cord)
6) Bowel, Bladder, & Sexual dysfunction (ANS)
Tests/Histo:
1) LP
(high lymphocytes, Oligoclonal IgG bands, & Myelin basic proteins)
2) MRI (plaques in white matter)
3) Gross exam (gray plaques in white matter)
Treatment:
Acute attacks = High dose steroid
Longterm = Interferon B (slows prog)
Path:
Autoimmune destruction of CNS myelin & oligodendrocytes (Demyelination)
Causes/Etiology:
HLA-DR2 genotypes & women (20-30yrs)
Signs:
1) Blurry vision in one eye (optic nerve)
2) Vertigo & drunk-like speech (brainstem)
3) Intranuclear ophthalmoplegia
(medial longitudinal fasciculitis “eyes can’t look to same side at the same time”)
4) Hemiparesis/Unilateral sensation loss (white matter)
5) Sensation loss/weakness in the lower extremities (spinal cord)
6) Bowel, Bladder, & Sexual dysfunction (ANS)
Tests/Histo:
1) LP
(high lymphocytes, Oligoclonal IgG bands, & Myelin basic proteins)
2) MRI (plaques in white matter)
3) Gross exam (gray plaques in white matter)
Treatment:
Acute attacks = High dose steroid
Longterm = Interferon B (slows prog)
Multiple sclerosis:
Alzheimer’s
Pathology:
Causes:
Symptoms/Signs:
Histological findings:
Complication:
Path:
Degeneration of the cortex
Causes:
1) E4 Apolipoprotein E
2) Familial presenilin 1 & 2 mutations
3) Down syndrome (40yrs)
Signs:
1) Slow onset memory loss that progresses from acute to Longterm memory
2) Motor & language skill loss
3) Behavior/Personality changes
Histo:
1) AB-Plaques & Neurofibrillary tau tangles
2) Cerebral atrophy with narrowed gyri & widened sulci
3) Loss of cholinergic neurons in the nucleus of basalis of Meynert
Complication:
Infection as the main COD & Hemorrhaging (amyloid deposits in BVs)
Path:
Degeneration of the cortex
Causes:
1) E4 Apolipoprotein E
2) Familial presenilin 1 & 2 mutations
3) Down syndrome (40yrs)
Signs:
1) Slow onset memory loss that progresses from acute to Longterm memory
2) Motor & language skill loss
3) Behavior/Personality changes
Histo:
1) AB-Plaques & Neurofibrillary tau tangles
2) Cerebral atrophy with narrowed gyri & widened sulci
3) Loss of cholinergic neurons in the nucleus of basalis of Meynert
Complication:
Infection as the main COD & Hemorrhaging (amyloid deposits in BVs)
Alzheimer’s
Parkinsons disease
Pathology:
Symptoms/Signs:
Histology:
Complication:
Path:
Degeneration of the frontal & temporal cortex (sparing the parietal & occipital lobes)
Signs: “TRAP”
1) Tremor (pinwheel at rest)
2) Rigidity (cogwheel)
3) Akinesia/Bradykinesia
4) Postural instability/Shuffling gait
Histo:
1) Lewy bodies (round & eosinophilic inclusions of alpha synclein)
2) Loss of pigmented neurons in substantia nigra
Complication:
Lewy body dementia
Path:
Degeneration of the frontal & temporal cortex (sparing the parietal & occipital lobes)
Signs: “TRAP”
1) Tremor (pinwheel at rest)
2) Rigidity (cogwheel)
3) Akinesia/Bradykinesia
4) Postural instability/Shuffling gait
Histo:
1) Lewy bodies (round & eosinophilic inclusions of alpha synclein)
2) Loss of pigmented neurons in substantia nigra
Complication:
Lewy body dementia
Parkinsons disease
Lewy body dementia
Pathology:
Symptoms/Signs:
Histology:
Path:
A complication of Parkinson disease
Signs:
1) Dementia
2) Hallucinations
3) Parkinsonian features
Histo:
Cortical lewy bodies
Path:
A complication of Parkinson disease
Signs:
1) Dementia
2) Hallucinations
3) Parkinsonian features
Histo:
Cortical lewy bodies
Lewy body dementia
Huntington disease
Pathologies:
Causes:
Symptoms/Signs:
Complication:
Path:
Degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia
Causes: Avg age ~40yrs
Expanded CAG huntingtin gene
(repeats during spermatogenesis leads to anticipation)
Signs:
1) Chorea with dementia & depression
Complication:
Suicide is the main COD
Path:
Degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia
Causes: Avg age ~40yrs
Expanded CAG huntingtin gene
(repeats during spermatogenesis leads to anticipation)
Signs:
1) Chorea with dementia & depression
Complication:
Suicide is the main COD
Huntington disease
Creutzfeldt-Jakob disease
(Spongiform encephalopathy)
Pathology:
Symptoms/Signs:
Causes:
Histology:
Complications:
Path:
Conversion of normal prions to B-pleated form (PrPsc)
Signs:
1) Rapidly progressive dementia associated with ataxia & startle myoclonus
Causes:
Exposure to prion-infected human tissue (hGH or corneal transplant)
Histo:
1) Spongy degeneration (intracellular vacuoles)
2) Spike-wave complexes on EEG
Complication:
Death within a year
Path:
Conversion of normal prions to B-pleated form (PrPsc)
Signs:
1) Rapidly progressive dementia associated with ataxia & startle myoclonus
Causes:
Exposure to prion-infected human tissue (hGH or corneal transplant)
Histo:
1) Spongy degeneration (intracellular vacuoles)
2) Spike-wave complexes on EEG
Complication:
Death within a year
Creutzfeldt-Jakob disease
(Spongiform encephalopathy)
Glioblastoma Multiforme
Pathology:
Histological findings:
Outcome:
Path:
Malignant Astrocyte tumor common in adults
Histo:
1) Butterfly lesion in the cerebral hemisphere (crosses corpus callosum)
2) Pseudopalisading (necrotic regions surrounded by the tumor)
3) Endothelial proliferation
4) GFAP +ve
Outcome:
Poor prognosis
Path:
Malignant Astrocyte tumor common in adults
Histo:
1) Butterfly lesion in the cerebral hemisphere (crosses corpus callosum)
2) Pseudopalisading (necrotic regions surrounded by the tumor)
3) Endothelial proliferation
4) GFAP +ve
Outcome:
Poor prognosis
Glioblastoma Multiforme
Meningioma
Pathology:
Symptoms/Signs:
Histology:
Path:
Benign arachnoid cell tumor that compresses (NOT invades) the cortex (usually in women)
Signs:
1) Seizures
Histology:
1) Round mass attached to the dura with a whorled pattern & psammoma bodies
Path:
Benign arachnoid cell tumor that compresses (NOT invades) the cortex (usually in women)
Signs:
1) Seizures
Histology:
1) Round mass attached to the dura with a whorled pattern & psammoma bodies
Meningioma
Oligodendroglioma
Pathology:
Symptoms/Signs:
Histology:
Path:
Malignant tumor of oligodendrocytes of the frontal lobe
Signs:
1) Seizure
Histo:
Calcified tumor in the white matter “fried-egg app”
Path:
Malignant tumor of oligodendrocytes of the frontal lobe
Signs:
1) Seizure
Histo:
Calcified tumor in the white matter “fried-egg app”
Oligodendroglioma
Pilocytic Astrocytoma
Pathology:
Histology:
“C-GEAR”
Path:
Benign astrocyte tumor (most common in children)
Histo:
1) Cystic lesion with a mural nodule (cerebellum)
2) Rosenthal fibers
3) Eosinophilic granular bodies
4) GFAP +ve tumor cells
Path:
Benign astrocyte tumor (most common in children)
Histo:
1) Cystic lesion with a mural nodule (cerebellum)
2) Rosenthal fibers
3) Eosinophilic granular bodies
4) GFAP +ve tumor cells
Pilocytic Astrocytoma
Ependymoma
Pathology:
Symptoms/Signs:
Histology:
Path:
Malignant ependymal cell tumor in the 4th ventricles (children)
Signs:
1) Hydrocephalus
Histo:
Perivascular pseudorosettes
Path:
Malignant ependymal cell tumor in the 4th ventricles (children)
Signs:
1) Hydrocephalus
Histo:
Perivascular pseudorosettes
Ependymoma
