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: