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FA complete review part 3.4 Pathology

What are the 4 herniation syndromes? 1. Cingulate (subfalcine) herniation under falx cerebri 2. Central/downward transtentorial herniation 3. Uncal transtentorial herniation 4. Cerebellar tonsillar herniation into the foramen magnum
A Cingulate herniation syndrome can ---> Compress anterior cerebral artery
Which artery is at risk of compression by a Cingulate herniation under the falx cerebri? Anterior cerebral artery (ACA)
ACA may be compressed by which herniation syndrome? Cingulate herniation under the falx cerebri
What is a Central/downward transtentorial herniation? Caudal displacement of brain stem which causes rupture of Paramedian basilar artery branches causing Duret hemorrhages
Duret hemorrhages are due to rupture of which arteries? Paramedian basilar artery branches
What causes the rupture of Paramedian basilar artery branches in a Central transtentorial herniation? The caudal displacement of the brain stem
The presence of Duret hemorrhages are associated with which herniation syndrome? Central/downward transtentorial herniation
Uncus = Medial Temporal lobe
What word can be used to refer to the "Medial Temporal lobe"? Uncus
What does an early Uncal transtentorial herniation causes? Ipsilateral blown pupil and contralateral hemiparesis
What does a late Uncal Transtentorial herniation causes? Coma and Kernohan phenomenon
What is the "Kernohan phenomenon"? Misleading contralateral blown pupil and ipsilateral hemirarers due to contralateral compression against Kernohan notch
Which herniation syndrome is associated with Kernohan phenomenon? Late Uncal Transtentorial herniation
Misleading contralateral blown pupil and ipsilateral hemiparesis due to contralateral compression against Kernohan notch. Kernohan phenomenon
What are the consequences of a Cerebellar tonsillar herniation into the foramen magnum? Coma and death result when these herniations compress the brain stem
What is the most feared structure to be compressed by a Cerebellar tonsillar herniation into the foramen magnum? Brain stem
Why is does a Cerebellar tonsillar herniation into the foramen magnum so feared? If it compresses the brain stem it can cause comma and death
What are the MOTOR neurons signs? 1. Weakness 2. Atrophy 3. Fasciculations 4. Reflexes 5. Tone 6. Babinski 7. Spastic paresis 8. Flaccid paralysis 9. Clasp knife spasticity
Motor neuron lesions are divided into: Upper and Lower
Lower motor neuron injury means: Less muscle mass, decreased muscle tone, decreased reflexes, and downgoing toes
Upper motor neuron injury means: Everything up (tone, DTRs, toes)
What are fasciculations? Muscle twitching
When is a (+) Babinski sign normal? In an infant
UMN and LMN injuries, both show with a positive _______________. Weakness
Which Motor neuron signs are (+) in LMN injury? Weakness, Atrophy, Fasciculations, and Flaccid paralysis
A person with Fasciculations, most likely suffered a UMN or LMN lesion? LMN lesion
Flaccid paralysis is seen in UMN or LMN lesion? LMN lesion
Which motor neuron sings are DECREASED in a LMN lesion? Reflexes and Tone
Which are (+) motor neuron signs in a UMN lesion? Weakness, Babinski, Spastic paresis, and Clasp knife spasticity
Reflexes and tone, are increased in a __________ motor neuron lesion. UMN lesion
List of Spinal lesion diseases: 1. Spinal muscular atrophy 2. Amyotrophic lateral sclerosis 3. Complete occlusion of anterior spinal artery 4. Tabes dorsalis 5. Syringomyelia 6. Vitamin B12 deficiency 7. Cauda Equina syndrome
Congenital degeneration of anterior horns of spinal cord. Dx? Spinal muscular atrophy
What type of motor neuron deficits are seen in Spinal muscular atrophy? LMN symptoms
What mutation is associated with Spinal muscular atrophy? AR mutation in SMN1 leading to defective snRNP assembly
What is the clinical presentation of Spinal muscular atrophy? - LMN symptoms only, - Symmetric weakness - "Floppy baby" with marked hypotonia - Tongue fasciculations
What is another way to refer to Spinal Muscular Atrophy (SMA) type 1? Werdnig-Hoffmann disease
Werdnig-Hoffmann disease is the same as: Spinal muscular atrophy type 1
What part(s) of the Spinal cord is affected in Werdnig-Hoffmann disease? Anterior horns of spinal cord
What is the common name for Amyotrophic lateral sclerosis (ALS)? Lou Gehrig disease
What symttoms are seen in ALS? Combined UMN and LMN degeneration.
In ALS (Lou Gehrig disease) what produces the UMN deficits? Degeneration of the corticobulbar and corticospinal tracts
What tract degeneration produces the LMN deficits seen in ALS? Degeneration of medullary and spinal cord
UMN + LMN lesion deficits with no sensory or bowel/bladder deficits. Dx? Amyotrophic lateral sclerosis
What is a common cause of Lou Gehrig disease? Defect in Superoxide dismutase 1
Defective Superoxide dismutase 1. Dx? Amyotrophic lateral sclerosis
What are the common LMN deficits seen in ALS? Flaccid limb weakness, fasciculations, atrophy, bulbar palsy
What is seen in Bulbar palsy? Dysarthria, dysphagia, and tongue atrophy
Which Spinal cord disorder is associated with Bulbar palsy? Amyotrophic lateral sclerosis
What are the UMN deficits seen with ALS? Spastic limb weakness, hyperreflexia, clonus, pseudobulbar palsy
What symptoms are associated with Pseudobulbar palsy? Dysarthria, dysphagia, and emotional lability
What is the common treatment for ALS? Rilouzole
What condition is treated with Rilouzole? Amyotrophic lateral sclerosis
Which tracts (parts of spinal cord) are spared in "Complete occlusion of ASA"? Dorsal columns and Lissauer tract
Which artery supplies the ASA below T8? Artery of Adamkiewicz
What is a common cause for Complete Occlusion of Anterior Spinal artery? Aortic aneurysm repair
What are the common clinical signs or symptoms of Complete Occlusion of ASA? 1. UMN deficit below the lesion (corticospinal tract), 2. LMN deficit at the level of the lesion (anterior horn), 3. Loss of pain and temperature sensation below the lesion (spinothalamic tract).
What parts of the Spinal cord are affected by the complete occlusion of the ASA? Corticospinal tract, anterior horn, and Spinothalamic tract.
What UMN deficits are seen in Complete Occlusion of the ASA? Deficit below the level of lesion
LMN or UMN deficits are seen at the level of lesion by Complete occlusion of ASA? LMN deficits
What is the result of damage to the Spinothalamic tract in Complete occlusion of the ASA? Loss of pain and temperature sensation below the lesion
What causes Tabes dorsalis? Tertiary syphilis
What are the pathological changes or results caused by Tabes dorsalis? Degeneration/ demyelination of dorsal columns and roots leading to progressive sensory ataxia
What are the clinical features caused by Progressive sensory ataxia caused by Tabes dorsalis? Impaired proprioception with causes poor coordination
(+) Romberg sign and absent DTRs. Dx? Tabes dorsalis
What are featured signs of Tabes dorsalis? - Charcot joints, - Shooting pain - Argyll Robertson pupils
(+) Argyll Robertson pupils. Dx? Tabes dorsalis
What spinal cord condition is associated with tertiary syphilis? Tabes dorsalis
Syrinx expand and damages anterior white commissure of spinothalamic tract. Syringomyelia pathogenesis
What is the result of Syringomyelia pathogenesis? Bilateral symmetric los of pain and tempreatio nsesaton in cape-like distribution
Chiari I malformation are associated with which Spinal cord condition? Syringomyelia
What is the featured sensation loss of Syringomyelia? Cape-like distribution loss of pain and temperature
What condition is known to affect the anterior white commissure of the Spinothalamic tract? Syringomyelia
What another name given to Vitamin B12 deficiency? Subacute combined degeneration
What tracts are demyelinated in Subacute combined degeneration? - Spinocerebellar tracts - Lateral Corticospinal tracts - Dorsal columns
What are the neurological symptoms seen in Vitamin B12 deficiency? Ataxic gait, paresthesia, impaired position/vibration sense, and UMN symptoms.
Cauda equina syndrome is due to: Compression of spinal roots L2 and below, often due to intervertebral disc herniation or tumor.
The herniation if the intervertebral disc, causing compression of spinal roots L2 and below. Dx? Cauda equina syndrome
What the clinical signs of Cauda equina syndrome? Radicular pain, absent knee and ankle reflexes, loss of bladder and anal sphincter control, and saddle anesthesia
Absence of the knee and ankle reflexes could represent what spinal cord condition? Cauda equina syndrome
What is saddle anesthesia? A loss of sensation restricted to the area of the buttocks, perineum and inner surfaces of the thighs.
What type of pain is associated with Cauda equina syndrome? Radicular pain
What pathogen causes Poliomyelitis? Poliovirus
Poliomyelitis is due to: Infections causes destruction of cells in anterior horn of spinal cord (LMN death)
Where does the Poliovirus replicate to cause Poliomyelitis? Oropharynx and small intestine before spreading via bloodstream to CNS.
What are the common LMN deficits seen in Poliomyelitis? Asymmetric weakness, hypotonia, flaccid paralysis, fasciculation, hyporeflexia, and muscle atrophy.
How does the LMN deficits different in Poliomyelitis and Spinal muscular atrophy? Poliomyelitis develops ASYMMETRIC weakness, while, Spinal muscular atrophy develos symmetric weakness.
If the patient demonstrates asymmetric weakness, is it Spinal muscular atrophy or poliomyelitis? Poliomyelitis
What condition is due to hemisection of spinal cord? Brown-Sequard syndrome
What is the cause of Brown-Sequard syndrome? Hemisection of spinal cord
What are the main 5 findings of Brown-Sequard syndrome? 1. Ipsilateral loss of all sensation at level of lesion 2. Ipsilateral LMN signs at level of lesion 3. Ipsilateral UMN signs below level of lesion 4. Ipsilateral loss of proprioception, vibration, light touch, and tactile sense below level of lesion 5. Contralateral loss of pain, temperature, and crue touch below level of lesion
What is a risk or a consequence, of Brown-Sequard syndrome above (lesion) T1? Ipsilateral Horner syndrome due to damage to oculosympathetic pathway
LMN deficits in Brown-Sequard syndrome? Ipsilateral LMN signs at level of lesion
What are the contralateral deficits seen in Brown-Sequard syndrome? Loss of pain, temperature, and crude touch below level of lesion
What are the ipsilateral symptoms below the level of lesion of Brown-Sequard syndrome? 1. UMN signs 2. Impaired proprioception, vibration, light touch, and tactile sense
What are the deficits seen ipsilaterally at the level of the lesion of Brown-Sequard syndrome? 1. Loss of sensation 2. LMN signs
Contralateral or Ipsilateral signs: Impaired pain, temperature, crude touch sensation in Brown-Sequard syndrome. Contralateral
Autosomal recessive trinucleotide repeat disorder (GAA)n on chromosome 9 in gene that encodes frataxin. Dx? Friedreich ataxia
What chromosome is involved in the mutation of Friedreich ataxia? Chromosome 9
What is "frataxin"? Iron binding protein
Which tracts are degenerated in Friedreich ataxia? 1. Lateral Corticospinal tract 2. Spinocerebellar tract 3. Dorsal columns 4. Dorsal root ganglia
What is the clinical result of degeneration of the lateral corticospinal tract in Friedreich ataxia? Spastic paralysis
What produces the loss of DRTs in Friedreich ataxia? Degeneration of dorsal root ganglia
What are symptoms of Friedreich ataxia? Staggering gaint, frequent falling, nystagmus, dysarthria, pes cavus, hammer toes, diabetes mellitus, hypertrophic cardiomyopathy
Which condition is often associated with childhood with Kyphoscoliosis? Friedreich ataxia
What featured bone abnormalities in Friedreich ataxia? Pes cavus and Hammer toes
Cardiac abnormality associated with Friedreich ataxia? Hypertrophic cardiomyopathy.
Hypertrophic, Restrictive, or Dilated cardiomyopathy, which is associated with Friedreich ataxia? Hypertrophic cardiomyopathy
A person with trinucleotide condition associated with Staggering gain and frequent falls? Friedreich ataxia
What is the most common cause of death in Friedreich ataxia? Hypertrophic cardiomyopathy
What is the trinucleotide associated with Friedreich ataxia? GAAn
What are the consequences of CN V motor lesion? Jaw deviates toward side of lesion due to unopposed force from the opposite pterygoid muscle
In a CN V motor lesion , does the jaw deviate away or toward the side of lesion? Toward
Why does a CN V motor lesion has a ipsilateral jaw deviation? Due to unopposed force from the opposite pterygoid muscle
Which muscle has not enough forced by itself to counteract the deviation toward injured side of CN V motor nerve? Pterygoid muscle
What is the consequence or clinical presentation of CN X lesion? Uvula deviates away from side of lesion
Is the uvula deviation toward or away from side of lesion in a CN X injury? Away
What causes the contralateral deviation of the uvula in a CN X lesion? Weak side collapse and uvula points away
Upon physical examination the uvula is deviated away from presented injured side. What is the suspected CN damaged? CN X
What is the clinical presentation of CN XI lesion? Weakness turning head to contralateral side of lesion
Weakness turning head to contralateral side of lesion is due to what kind of cranial nerve damage? CN XI lesion
What are the clinical results of CN XI lesion? 1. Weakness turning head to contralateral side of lesion 2. Shoulder drop on side of lesion
What ipsilateral muscle is damaged in CN XI lesion that cause the shoulder drop? Trapezius
Which muscle is affected by CN XI lesion? Contralateral Sternocleidomastoid
What is the ipsilateral sign of a CN XI lesion? Shoulder drop
The left SCM contract to help ====> Turn the head to the right
What is the consequences of a CN XII lesion? Tongue deviates toward side of lesion
Why is the tongue deviates toward of CN XII lesion? Due to weakened tongue muscles on affected side
A CN XII lesion is considered a _______________ lesion. LMN lesion
Does tongue deviate toward or away from affected side in a CN XII lesion? Toward
The expression "lick your wounds" is used to describe aht type of CN lesion? CN XII lesion
If the tongue is deviated, which cranial nerve is most likely affected? CN XII
If the Jaw is deviated, which cranial nerve is most likely affected? CN V motor
What body part is affected and by consequence deviated in a CN X? Uvula
What two parts of the body are affected in a CN XI lesion? Sternocleidomastoid muscle (neck) and Trapezius muscles (shoulder)
What is the most common cause of peripheral facial palsy? Bell palsy
What is the most common pathogen associated with the development of Bell palsy? Reactivation of HSV
What is the treatment for Bell palsy due to HSV reactivation? Corticosteroids + acyclovir
List of causes of peripheral facial palsy? 1. Reactivation of HSV infection 2. Lyme disease 3. Herpes zoster (Ramsay Hunt syndrome) 4. Sarcoidosis 5. Tumors 6. Diabetes mellitus
What is Ramsay Hunt syndrome? A complication of shingles. It is the name given to describe the symptoms of a shingles infection affecting the facial nerve
What is the location of the lesion of Facial nerve palsy that produces UMN deficits? Motor cortex, connection from motor cortex to facial nucleus in pons
If the Facial nerve palsy prices UMN deficits, will these be contralateral or ipsilateral? Contralateral
What are the muscles involved or affected in Facial nerve palsy with UMN deficits? Lower muscles of facial expression
If the forehead is spared (not involved), the facial nerve palsy is of UMN or LMN affectio? UMN
Why is the forehead spared in facial nerve palsy with UMN lesion? Due to bilateral UMN innervation
Is UMN or LMN innervation bilateral, that a facial nerve palsy does not affect the foreadhad? UMN
Lesion location of facial nerve palsy with a LMN injury? Facial nucleus, anywhere along CN VII
Which nerve is affected in LMN Facial nerve palsy? CN VII
Ipsilateral or contralateral, the effects of LMN facial nerve palsy? Ipsilateral
Which type of lesion deficits in facial nerve palsy, affect the Upper and lower muscles of facial expression? LMN Facial nerve palsy
If both, upper and lower muscles of facial expression are affected by Facial nerve palsy, it is safe to assume the forehead will be or will not be affected? Affected forehead
LMN lesion or UMN lesion if facial nerve palsy involves only lower muscles of facial expression? UMN lesion
What are associated symptoms of LMN Facial nerve palsy? - Incomplete eye closure, - Hyperacusis - Loss of taste and sensation to anterior tongue
CN VII LMN lesion ---> Peripheral; cannot wrinkle forehead
If patient present with a facial nerve palsy, and is UNABLE to wrinkle the forehead, it indicates a ______________ lesion. LMN
Created by: rakomi
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