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Neurology
FA complete review part 3.4 Pathology
Question | Answer |
---|---|
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 |
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rakomi
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