Herniation syndromes, spinal cord lesions and UMN/LMN signs Flashcards
How is Friedreich ataxia inherited?
Aut Recessive trinucleotide repeat disorder (GAA on chr 9)
What is the trinucleotide repeat in Friedrick ataxia?
What chromosome is it on?
GAA on chr 9
- ataxic GAAit
What does the gene code for in Friedreich ataxia?
Frataxin (iron binding protein)
What tracts / nerve structures are impaired in Friedreich ataxia?
- Spinocerebellar
- Corticospinal
- Dorsal colomns
- Dorsal root ganglia
What is the cause of death in Fiedrich ataxia?
Hypertrophic cardiomyopathy
What are the symptoms of Friedreich ataxia?
- Staggering gait
- Frequent falling
- Nystagmus
- Dysartthria
- Pes cavus (high arches)
- Hammer toes
- DM
- Hypertrophic cardiomyopathy
What feature is first identified in childhood in Friedrich ataxia?
Kyphoscolioisis
Spinal muscular atrophy is due to degeneration of what part of the spinal cord?
Anterior horns
What are the symptoms of spinal muscular atrophy?
- LMN symptoms only, symmetrical
- floppy baby with marked hypotonia (flaccid paralysis) and tongue fasiculations
What mutation causes spinal muscular atrophy
What protein does this affect?
SMN1 mutation -> defective snRNP assembly
How is spinal muscular atrophy (SMN1 mutation) inherited?
Aut Recessive
What is spinal muscular atrophy type 1 called?
Werdnig-Hoffman disease
A defect in superoxide dismutase 1 can cause what spinal lesion?
Amyotrophic lateral sclerosis
WHat is ALS treated with?
Rilouzole
What do those with ALS usually die of?
Resp failure
What is ALS also known as?
Low Gehrig disease
What are the symptoms of ALS?
LMN
- Flaccid limb weakness
- Fasiculations
- Atrophy
- Bulbar palsy (dysarthria, dysphagia, tongue atrophy)
UMN
- Spastic limb weakness
- Hyperreflexia
- Clonus
- Pseudobulbar palsy (dysarthria, dysphagia, emotional liability)
What kind of spinal lesion can be caused by AAA repair?
Complete occlusion of anterior spinal artery
What artery supplies the Anterior spinal artery (ASA) below T8?
Artery of Adamkiewicz
What are the symptoms of Complete occlusion of anterior spinal artery?
Below lesion
- UMN defecit -> Corticospinal tract
- Loss of pain + temp -> Spinothalamic tract
Level of lesion:
- LMN defecit (anterior horn)
What is tabes dorsalis caused by (what disease/bacteria)?
Tertiary syphilis
What part of the spinal cord does tabes dorsalis primarily affect?
What symptoms/signs does this result in?
Dorsal columns and roots demyelinate and degenerate
- Progressive sensory ataxia -> Impaired proprioception and poor coordination
- +ve Romberg sign
- Absent DTRs
WHat other symptoms/signs is Tabes Dorsalis associated with?
- Charcot joints
- Shooting pain
- Argyll Robertson pupils
Syringomyelia causes what symptoms?
Loss of pain and temp (spinothalamic) in cape-like distribution
What expands and damages the ant white commissure in syringomyelia?
Syrinx
What embryological disorder is associated with Syringomyelia?
Chiari I malformation
What tracts are damaged by subacute combined degeneration of the cord?
- Spinocerebellar tract - ataxic gait
- Lateral corticospinal
- Dorsal columns - parasthesia, impaired vibration/position sense. +ve Romberg sign
- UMN symptoms
Caudia Equina syndrome symptoms
- Radicular pain
- Absent knee and ankle reflexes
- Loss of anal and bladder sphincter control
- Saddle anesthesia
Compression of spinal roots below what level may cause cauda equina syndrome?
L2
Cauda equina syndrome is due to compression via what?
- Intervertebral disc herniation
- Tumour
What part of the spinal cord is affected in poliomyelitis?
Anterior horn -> LMN signs (fasiculations, atrophy etc.)
What are the symptoms of poliomyelitis?
- Asymmetric weakness
LMN signs: - Hypotonia
- Flaccid paralysis
- Fasiculations
- Hyporeflexia
- Muscle atrophy
Signs of infection (malaise, fever, nausea etc.)
What will CSF show in poliomyelitis?
- Increased WBCs (lymphocytic pleocytosis)
- Slight increase in proteins
- No change in glucose
Where does polio virus replicate?
Where can it be recovered from?
Lymphoid tissue of oropharynx and SI before spreading in bloodstream to CNS
- Virus recovered from stool or throat
What are the signs/symptoms of Brown-Sequard syndrome?
- Ipsilateral loss of all sensation at level of lesion
- Ipsilateral LMN signs (eg flaccid paralysis) at level of lesion
- Ipsilateral UMN signs below level of lesion (due to corticospinal tract damage)
- Ipsilateral loss of proprioception, vibration, and light touch below level of lesion (Dorsal column)
- Contralateral loss of pain + temperature and crude touch below lesion (Spinothalamic)
What additional syndrome may develop if Brown-Sequard syndrome occurs above T1?
Ipsilateral Horner’s syndrome due to damage of oculosympathetic pathway
What cranial nerve lesions cause ddeviation towards the side of the lesion?
- CN V -> jaw deviates toward lesion
- CN XII -> Tongue deviates toward lesion
What cranial nerve lesion causes deviation away from the side of the lesion?
- CN X -> Uvula deviates away from lesion
Describe the weakened muscles in a CNXI lesion and how these affect movements?
- SCM -> Weakness turning head to contralateral side of lesion (opposite side SCM contracts to help turn head other way)
- Trapezius -> Shoulder droop on side of lesion
What is the treatment of Bell palsy (from HSV commonly)?
- Corticosteroids +/- acyclovir
What are the different causes of peripheral facial palsy?
- HSV
- Lyme’s disease
- Herpes Zoster (Ramsey Hunt)
- Sarcoidosis
- Tumours of parotid gland
- DM
Flaccid paralysis is seen in UMN or LMN lesions?
LMN
Clasp knife spasticity is seen in UMN or LMN lesions?
UMN lesiosn
