Strohm content Flashcards
Encephalopathy: [definition]
Encephalopathy: dysfunction of cerebrum
Myelopathy: [definition]
Myelopathy: dysfunction of spinal cord
Radiculopathy: [definition]
Radiculopathy: dysfunction of nerve root
Plexopathy: [defintion]
Plexopathy: dysfunction of plexus
Polyneuropathy: [defintion]
Polyneuropathy: dysfunction of more than one nerve
Mononeuropathy: [definition]
Mononeuropathy: dysfunction of one nerve
* Ex: median mononeuropathy = carpal tunnel syndrome
Myopathy: [definition]
Myopathy: dysfunction of muscle
Examples of bulbar weakness:
Bulbar weakness involves a bilateral LMN lesion of CN IX, X, XI, XII
* Dysarthria: speech poorly articulated
* Dysphagia: difficulty swallowing
* Dysphonia: voice weak
Biceps and brachioradialis reflex involves [spinal level]
Biceps and brachioradialis reflex involves C5, C6
* C5, C6 pick up sticks
Triceps reflex involves [spinal level]
Triceps reflex involves C6,C7
* C7 not 8 lay them straight
Cremasteric reflex involves [spinal levels]
Cremasteric reflex involves L1, L2
Patellar reflex involves [spinal levels]
Patellar reflex involves L2-L4
* L2-L4 kick the door
Achilles reflex involves [spinal levels]
Achilles reflex involves S1, S2
* S1, S2 buckle my shoe
Anal wink reflex involves [spinal levels]
Anal wink reflex involves S3, S4
Increased muscle stretch is detected by muscle spindle receptors via [type axons]
Increased muscle stretch is detected by muscle spindle receptors via type Ia and II sensory axons
Muscle spindle afferent fibers travel to the spinal cord via _ and synapse with _ and _
Muscle spindle afferent fibers travel to the spinal cord via DRG and synapse with alpha motor neuron and inhibitory interneuron
Increased muscle stretch will send signals to the spinal cord that result in simultaneous _ and _
Increased muscle stretch will send signals to the spinal cord that result in simultaneous inhibition of antagonist muscle (prevent overstretching) and activation of agonist muscle (contraction)
Increased muscle force is detected by golgi tendon organ via [type axons]
Increased muscle force is detected by golgi tendon organ via type Ib sensory axons
Explain the golgi tendon pathway:
- Type Ib fibers sense increased muscle tension
- Fiber travel to spinal cord via DRG
- Activation of inhibitory interneuron
- Inhibition of agonist muscle (to reduce tension)
_ is an immune-mediated or inflammatory PNS demyelinating disease
Guillain-Barre syndrome is an immune-mediated or inflammatory PNS demyelinating disease
_ is a hereditary PNS demyelinating disease
Charcot Marie Tooth disease is a hereditary PNS demyelinating disease
Most cases of Guillain-Barre are _ , meaning they are monophasic and acute; cases that are > 8 weeks are called _
Most cases of Guillain-Barre are acute inflammatory demyelinating polyneuropathy (AIDP) , meaning they are monophasic and acute; cases that are > 8 weeks are called CIDP
Miller-fisher syndrome is a type of Guillain-Barre in which we see _ deficits due to antibodies against _
Miller-fisher syndrome is a type of Guillain-Barre in which we see oculomotor deficits due to antibodies against GQ1B
The most common pattern of multiple sclerosis is _
The most common pattern of multiple sclerosis is relapsing remitting
_ is a common presentation of multiple sclerosis that involves painful unilateral vision loss and Marcus Gunn pupil
Acute optic neuritis is a common presentation of multiple sclerosis that involves painful unilateral vision loss and Marcus Gunn pupil
Pyramidal tract demyelination in M.S. may present as [symptoms]
Pyramidal tract demyelination in M.S. may present as weakness, spasticity
M.S. patients may experience spinal cord syndromes such as _
M.S. patients may experience spinal cord syndromes such as electric shock-like sensation along cervical spine on neck flexion
* Also neurogenic bladder, paraparesis, etc
M.S. symptoms may be aggrevated by _ temperatures
M.S. symptoms may be aggrevated by hot temperatures
* Ex: exercise, hot bath
The classic demographic for MS is _
The classic demographic for MS is 20-30 yo female
[Imaging] showing _ is diagnostic for MS
MRI showing multiple lesions, separated by time and space is diagnostic for MS
Multiple sclerosis
Osmotic demyelination syndrome
Progressive multifocal leukoencephalopathy (PML)
_ is a disorder of beta-oxidation due to a mutation in ABCD1 gene that causes buildup of VLCFA in the adrenal glands and white matter of the brain
Adrenoleukodystrophy is a disorder of beta-oxidation due to a mutation in ABCD1 gene that causes buildup of VLCFA in the adrenal glands and white matter of the brain
* X-linked recessive
* Progressive disease that leads to adrenal gland crisis, coma, death
Another name for osmotic demyelination syndrome is _
Another name for osmotic demyelination syndrome is central pontine myelinolysis
Osmotic demyelination syndrome is massive axonal demyelination in the _ secondary to rapid osmotic change
Osmotic demyelination syndrome is massive axonal demyelination in the pontine white matter secondary to rapid osmotic change
The most common cause of osmotic demyelination syndrome is _
The most common cause of osmotic demyelination syndrome is iatrogenic- correction of hyponatremia
Signs of osmotic demyelination syndrome include _
Signs of osmotic demyelination syndrome include acute paralysis, dysarthria, dysphagia, diplopia, loss of consciousness
* Can cause “locked in syndrome”
“Locked in syndrome” can be caused by _
“Locked in syndrome” can be caused by osmotic demyelination syndrome
Correcting sodium too fast:
“From high to low, _ “
“From low to high _ “
Correcting sodium too fast:
“From high to low, your brains will blow “ - cerebral edema/ herniation
“From low to high your pons will die “ - osmotic demyelination syndrome
_ is the most common subtype of Guillain-Barre syndrome
Acute inflammatory demyelinating polyradiculopathy is the most common subtype of Guillain-Barre syndrome
AIDP is an autoimmune condition (subtype of Guillain-Barre) that destroys _
AIDP is an autoimmune condition (subtype of Guillain-Barre) that destroys Schwann cells
* Damage occurs via inflammation
* Demyelination of motor fibers, sensory fibers, peripheral nerves
Guillain Barre does not have known definitive causal link to any pathogen though assocition with _ is most common
Guillain Barre does not have known definitive causal link to any pathogen though assocition with Campylobacter jejuni is most common
* Damage is mediated by inflammation
* Likely facilitated by molecular mimicry and triggered by stress
Guillain-Barre results in [symmetric/ asymmetric] and [ascending/ descending] paralysis
Guillain-Barre results in symmetric and ascending paralysis
Gullain-Barre will often present with _ DTR
Gullain-Barre will often present with depressed or absent DTR
* Starting in the lower extremities
* Can progress to facial paralysis and respiratory failure
Guillain-Barre is associated with _ , an increase in CSF protein with normal cell count
Guillain-Barre is associated with albuminocytologic dissociation , an increase in CSF protein with normal cell count
Guillain Barre is treated via:
Guillain Barre is treated via:
* Respiratory support
* Plasmapheresis or IVIG
Acute disseminated encephalomyelitis is _
Acute disseminated encephalomyelitis is multifocal inflammation and demyelination after infection
* Can also occur after vaccination
* Presents with rapidly progressive multifocal neurologic symptoms, altered mental status
Charcot-Marie-Tooth disease is also called _
Charcot-Marie-Tooth disease is also called hereditary motor and sensory neuropathy
Charcot-Marie-Tooth is a “dysmyelinating” disease, meaning _
Charcot-Marie-Tooth is a “dysmyelinating” disease, meaning there is defective production of proteins involved in structure and function of peripheral nerves/ myelin sheath
Charcot-Marie-Tooth disease has [inheritance pattern]
Charcot-Marie-Tooth disease has autosomal dominant inheritance
Presenting symptoms of Charcot-Marie Tooth disease include:
Presenting symptoms of Charcot-Marie Tooth disease include:
* Foot deformities (pes cavus, hammer toe)
* Lower extremity weakness (foot drop)
* Sensory deficits
The most common type of Charcot marie tooth, CMT1A is caused by [mutation]
The most common type of Charcot marie tooth, CMT1A is caused by PMP22 gene duplication
Progressive multifocal leukoencephalopathy is a demylination disease of the (CNS/PNS)
Progressive multifocal leukoencephalopathy is a demylination disease of the CNS
* Involves destruction of oligodendrocytes
PML occurs secondary to _
PML occurs secondary to reactivation of a latent JC virus infection
* Occurs primarily in AIDS patients
* Rapidly progressive, often fatal
_ motor neuron produces muscle movement on contraction
Alpha motor neuron produces muscle movement on contraction
_ motor neuron regulates the sensitivity to stretch and contributes to fine motor control
Gamma motor neuron regulates the sensitivity to stretch and contributes to fine motor control
Name some chronic, axonal polyneuropathies
Chronic, axonal polyneuropathies:
* Diabetic neuropathy
* B12 deficiency
* Critical illness myopathy/neuropathy
Arsenic and lead poisoning cause acute (axonal/demyelinating) polyneuropathy
Arsenic and lead poisoning cause acute axonal polyneuropathy
Delayed (blocked) conduction velocity on nerve conduction study may be an indication of [type pathology]
Delayed (blocked) conduction velocity on nerve conduction study may be an indication of demyelinating neuropathy
* E.g. Guillain Barre
Reduced signal amplitude on nerve conduction study may be an indication of underlying [type pathology]
Reduced signal amplitude on nerve conduction study may be an indication of underlying axonal neuropathy
* E.g diabetic neuropathy
What disease is seen in navy?
UMN lesion will cause _ bladder
UMN lesion will cause spastic bladder
* Urgency, incontinence, increased bladder tone
* E.g. stroke, MS, spinal cord lesion
LMN lesion will cause _ bladder
LMN lesion will cause flaccid bladder
* Incontinence, retention, no anal reflex, decreased bladder tone
* E.g. cauda equina
Spinal muscular atrophy is a congenital degeneration of _ that results in _
Spinal muscular atrophy is a congenital degeneration of anterior horns of spinal cord that results in LMN symptoms, hypotonia
A “floppy baby” with marked hypotonia and tongue fasiculations is suggestive of _
A “floppy baby” with marked hypotonia and tongue fasiculations is suggestive of spinal muscular atrophy
* aka Werdnig-Hoffmann disease
Werdnig-Hoffmann disease is a [inheritance pattern] mutation in _ gene
Werdnig-Hoffmann disease is an autosomal recessive mutation in SMN1
* Causes defective snRNP
ALS is associated with _ bladder deficits
ALS is associated with no bladder deficits
Amyotrophic lateral sclerosis involves _ type degeneration
Amyotrophic lateral sclerosis involves combined UMN and LMN degeneration
* UMN: corticobulbar/corticospinal
* LMN: medullary and spinal cord degeneration
ALS can be caused by a defect in _
ALS can be caused by a defect in superoxide dismutase 1
Complete occlusion of the anterior spinal artery spares the _ and _
Complete occlusion of the anterior spinal artery spares the dorsal column and Lissauer tract
The _ regions of the anterior spinal artery is a watershed area
The mid-thoracic (T3-T8) regions of the anterior spinal artery is a watershed area
* Below T8 the artery of Adamkiewicz supplies ASA
Complete occlusion of the ASA presents with _ deficit below the lesion
Complete occlusion of the ASA presents with UMN deficit below the lesion
* Corticospinal tract
Complete occlusion of the ASA presents with _ deficit at the level of the lesion
Complete occlusion of the ASA presents with LMN deficit at the level of the lesion
* Ventral horn
_ is an infectious cause of progressive sensory ataxia (impaired proprioception and coordination)
Tabes dorsalis is an infectious cause of progressive sensory ataxia (impaired proprioception and coordination)
* Associated with charcot joints, argyll robertson pupils, paroxysmal shooting pain
In syringomyelia, the syrinx expands and damages the _ of the spinothalamic tract
In syringomyelia, the syrinx expands and damages the anterior white commissure of the spinothalamic tract
Subacute combined degeneration involves demyelination of (3) tracts:
Subacute combined degeneration involves demyelination of (3) tracts: SCD
* Spinocerebellar tracts
* Lateral corticospinal tracts
* Dorsal columns
Subacute combined degeneration will present with _ signs
Subacute combined degeneration will present with ataxic gait, parethesia, impaired position/vibration sense, UMN symptoms
Cauda equina syndrome is associated with radicular pain, absent _ reflexes, loss of bladder and anal sphincter control, and _ anesthesia
Cauda equina syndrome is associated with radicular pain, absent knee and ankle reflexes, loss of bladder and anal sphincter control, and saddle anesthesia
Tabes dorsalis
ALS
Syringomyelia
SCD: vitamin B12 deficiency
Non-infectious cause
Spinal muscular atrophy
Complete occlusion ASA
Syringomyelia most commonly occurs at [spinal levels]
Syringomyelia most commonly occurs at C8-T1
* Caused by trauma, tumor, chiari malformation
Anterior spinal cord syndrome is associated with loss of pain and temperature _ level
Anterior spinal cord syndrome is associated with loss of pain and temperature below the lesion
Hemisection of the spinal cord causes a contralateral loss of _ two levels below the lesion
Hemisection of the spinal cord causes a contralateral loss of pain and temperature two levels below the lesion
* Spinothalamic tract
Hemisection of the spinal cord causes ipsilateral _ signs below the level of the lesion
Hemisection of the spinal cord causes ipsilateral UMN signs below the level of the lesion
Hemisection of the spinal cord causes ipsilateral _ signs at the level of the lesion
Hemisection of the spinal cord causes ipsilateral LMN signs at the level of the lesion
Hemisection of the spinal cord causes _ lateral loss of fine discrimination, vibration, proprioception below the level of lesion
Hemisection of the spinal cord causes ipsilateral loss of fine discrimination, vibration, proprioception below the level of lesion
If brown-sequard syndrome occurs above _ level, ipsilateral Horner syndrome (ptosis, miosis, anhidrosis) is possible
If brown-sequard syndrome occurs above T1 level, ipsilateral Horner syndrome (ptosis, miosis, anhidrosis) is possible
Friedreich ataxia is an autosomal recessive trinucleotide repeat GAA, involving [chromosome] and a gene that encodes _
Friedreich ataxia is an autosomal recessive trinucleotide repeat GAA, involving chromosome 9 and a gene that encodes frataxin (iron-binding protein)
Friedreich ataxia involves degeneration of [tracts]
Friedreich ataxia involves degeneration of lateral corticospinal tract, spinocerebellar tract, dorsal columns
The primary pathology in Friedreich ataxia is a loss of _ cells
The primary pathology in Friedreich ataxia is a loss of sensory ganglion cells
Poliovirus is an RNA picornavirus that causes destruction of cells in the [spinal cord region]
Poliovirus is an RNA picornavirus that causes destruction of cells in the anterior horn
* Affects LMNs
* Asymmetric weakness
Conus medullaris syndrome involves [spinal levels]
Conus medullaris syndrome involves T12-L2
Conus medullaris syndrome presents with _ reflexic DTR
Conus medullaris syndrome presents with hyperreflexic DTR
Sudden and symmetrical describes the deficits of (conus medullaris/cauda equina)
Sudden and symmetrical describes the deficits of conus medullaris
* Cauda equina is gradual and asymmetrical
