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136 Cards in this Set
- Front
- Back
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Most common cause of neuropathy
"stocking glove type" |
Diabetic neuropathy
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Symmetrical sensorimotor syptoms, severe burning pain in 25%
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Chronic alcoholism
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Peripheral neuropathy associated with degeneration in spinal cord white matter pthwys (post columns and corticospinal tracts)
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B12 deficiency
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Severe sensory neuronopathy (lesion in dorsal root gang) resulting in severe sensory ataxia and gait disturbance
Loss of vibration, proprio and gait ataxia Seen in individuals taking excess supplements |
B6 deficiency
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Mainly sensory with symptoms of burning distal pain and later motor involvement
Slowed axonal transport associated with hair loss, muscle aches ,dry skin, constipation Diagnosis is confirmed by T4 and TSH levels |
Hypothyroid Neuropathy
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End Stage Renal Disease
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Progressive distal sensorimotor PN
CTS common |
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Chemotherapeutic Agent that inhibits microtubule polymerization and interferes with axonal transport
Stocking glove Eventually profound distal weakness foot drop |
Vincristine
(symptoms can worsen even after stopping meds) improve over time mostly |
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What causes a PN that mimics the PN caused by HIV?
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Antiretroviral agents
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Which antituberculous agent causes a sensorimotor PN?
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INH w/o B6
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Which antibiotic given over a long period of time causes a sensorimotor PN?
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Metronidazole (Flagyl)
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Which antibiotic used to treat chronic UTI's can cause sensorimotor PN w/i 6 weeks?
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Nitrofurantoin (Macrodantin)
*severe weakness can occur* |
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Which lipid lowering agents cause PN?
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Lovastatin
Simvastatin Atorvastatin (Lipitor) |
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Which antiepilectic med causes a PN that also results in loss of reflexes in 50%?
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Phenytoin (Dilantin)
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This poisoning cuases a N in adults and is often associated with encephalopathy, GI sympt, weight loss, anemia
Motor n> sensory |
Lead poisoning
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Acute exposure to poison used for suicide or homicide that causes severe GI symptoms, tachy, hypotension, encephalopathy
Motor>sensory, areflexia, resp failure similar to GBS |
Arsenic poisoning
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Mee's lines, hyperpigmentation, hyperkeratosis, alopecia, fatigue
Stocking glove burning pain in feet and hands |
Low chronic levels of arsenic
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How do organophosphates work?
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Irreversible inhibition of AChE
Causes: diarrhea, nausea/vomiting, excess salivation/sweat, tachy or brady enceph, anxiety, fatigue 12-96 hours later resp failure, neck flexor and prox>>distal muscle weakness |
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7-10 days after an OP poisoning
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Begins with sensory symptoms but mostly a motor PN with foot drop and profound distal weakness
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Asymmetrical PN caused by ischemic infarct of the vasa nervorum
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Vasculitis
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Patient had a viral illness a week ago
Experiences distal paresthesias ascending paralysis w/ weak prox muscles Facial weakness lost reflexes |
GBS
(resp failure 1-2 weeks) can involve autonomics (arrhythmias, sinus tachy, BP dysregulation) |
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Triad of Acute ophthalmoplegia
Ataxia and Areflexia Anti-GQ1b antibodies |
Miller-Fisher variant of GBS
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15 year old male comes in with bilateral foot drop and extremely atrophied lower leg muscles. He has high arches and hammer toes. Some of his family members have experienced this.
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Charcot-Marie-Tooth
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68 year old lady comes in with numbness and pain in toes and feet, Lab tests and EMG come back normal
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Idiopathic sensory PN
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30 year old male comes in with sensory loss due to intracutaneous nerve damage on the dorsum of the hands, feet, ears, lat legs, and forearms
Skin biopsy shows acid fast bacillus |
Leprous Neuropathy (Hanson's disease)
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60 year old lady comes in with painful break out on her abdomen.
The pain is electrical/burning and she confirms having chicken pox as a child |
Herpes Zoster Neuropathy (Shingles)
Acyclovir shortens Gabapentin treats pain |
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35 year old lady comes in complaining of numbness of her hand that awakens her in the middle of the night. She also seems to have proximal pain in her shoulder
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Carpal tunnel syndrome
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45 year old male comes in complaining of weakness in the hand and numbness of digits 4 & 5 that worsens when he leans on elbow
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Ulnar neuropathy
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Neuropathy at the spiral groove (sat night) or in the axilla (honeymooner's) that results in wrist drop
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Radial neuropathy
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26 year old female complains of numbness in leg and foot, foot drop and weakness in 1st & 2nd toe
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Peroneal neuropathies
(compression by fibular head) |
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Patient comes in with weakness and muscle pain during exertion. Also experiences coca-cola urine
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Metabolic myopathies
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Patient comes in with eye movement problems, ptosis, retinitis pigmentosa, myoclonic epilepsy,and myopathic weakness
(KSS & PEO) |
Mitochondrial Myopathies
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2 main factors that distinguish muscular dystrophies from other myopathies
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Hereditary
Progressive muscle weakness and wasting |
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2 year old boy comes in with delayed motor milestones. He has weak neck flexors and a broad based waddling walk. He has notable lumbar lordosis.
He has hypertrophied calve for his size and uses Gower sign to get off the floor |
Duchenne Muscular Dystrophy
missing dystrophin, 2/3 X-recessive,wheelchair bound by 12, CK super high, myopathic pattern EMG |
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12 year old boy presents with neck flexor weakness, diffuse weakness esp in lower extremities and pelvis
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Beckers MD
X recessive, majority survive til 40 or 50, less mental retardation, EMG myopathic, Dystrophin detectable but altered, high CK |
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23 year old male presents with ptosis, weak and wasted temporalis muscles. Nasal voice and weak SCM. His distal muscles are weaker than proximal. He has a foot drop and trips often. He notices he can't release the handle on the subway easily. He suffers from balding and has cataracts. He mentions he has testicular atrophy
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Myotonic Dystrophy
normal CK, AD ch19 CTG repeat, some mental retardation, cardiac prob, Insulin resistant, EMG myotonic discharges, |
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19 male presents with the inability to smile, whistle or use a straw. He can't close his eyes tightly. He has weakness in his shoulder girdle and has notable scapular winging. He can't carry heavy objects above his head or participate in PE. He trips constantly due to his foot drop
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Facioscapulohumeral Dystrophy (FSH)
AD ch4, occasional sensorineural hearing loss, vascular retinal disease, CK normal |
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rRNA makes up this structure and it is only found in cell bodies and dentrites (NOT AXONS!!)
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Nissl substance
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stellate, bean shaped nucleus
BBB neuropil (scaffolding) Metabolism of extracellular neurotransmitters and metabolism of urea |
Astrocytes
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Myelinates CNS axons (mainly white matter)
Pyknotic round nucleus One can myelinate multiple axons |
Oligodendroglia
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Columnar cells w/ apical cilia lining the ventricles
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Ependyma
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glial cell that functions in secreting CSF
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choroid plexus
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false glial cell that act as garbage collectors of CNS (similar to macrophage)
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Microglia
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myelinating cells of the PNS
each cell myelinates a single axon Neural crest origin |
Schwann cells
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Cells that are vulnerable to hypoxic/ischemic damage (particularly CA1 of hippo) and Alzheimers
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Neurons
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CNS cells susceptible to neoplasia
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Astrocytes
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CNS cells susceptible to myelin injury (e.g. multiple sclerosis)
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Oligodendroglia
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Cells damaged by GBS
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Schwann cells
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Where and when does a significant amount of neural proliferation occur?
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during fetal development in the periventricular germinal matrix
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3 ways of Neuronal degen/death
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Central chromatolysis- central clearing, displaced Nissl, seen in axonal injury, vit def, possible reversal
Necrosis- red dead, cytoplasmic eosinophilia, nuclear pyknosis, shrinkage Apoptosis |
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What can cross the BBB?
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small lipid soluble molecules and molecules with spec transport channels
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Components of BBB
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non fenestrated cap w/ tight junc (most important)
Basement membrane Pericytes Astrocyte foot processes |
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CSF flow Pthwy
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Choroid--> Lat vent--> Foramen of Monroe--> 3rd vent--> Cerebral aqueduct of Sylvius--> 4th vent--> Foramina of Magendie (1 mid) and Lushka (2L)--> Subarach space--> Arachnoid granulations--> Dural venous sinus
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2 ways to differentiate CSF leak from other fluids
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High glucose levels in CSF
Beta-2 transferrin from neural tissue |
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5 types of Hydrocephalus
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Obstructive non-communicating
Obstructive communicating Non obstructive Ex-vacuo Normal Pressure |
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Cells damaged by GBS
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Schwann cells
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Signs of infant hydrocephalus
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enlarged head
downward gaze irritable seizures sleepy vomiting |
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Where and when does a significant amount of neural proliferation occur?
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during fetal development in the periventricular germinal matrix
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2 gross findings of cerebral edema
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Enlarged brain w/ flattened gyri and narrowed sulci
Increased brain weight |
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3 ways of Neuronal degen/death
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Central chromatolysis- central clearing, displaced Nissl, seen in axonal injury, vit def, possible reversal
Necrosis- red dead, cytoplasmic eosinophilia, nuclear pyknosis, shrinkage Apoptosis |
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What can cross the BBB?
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small lipid soluble molecules and molecules with spec transport channels
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Components of BBB
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non fenestrated cap w/ tight junc (most important)
Basement membrane Pericytes Astrocyte foot processes |
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CSF flow Pthwy
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Choroid--> Lat vent--> Foramen of Monroe--> 3rd vent--> Cerebral aqueduct of Sylvius--> 4th vent--> Foramina of Magendie (1 mid) and Lushka (2L)--> Subarach space--> Arachnoid granulations--> Dural venous sinus
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2 ways to differentiate CSF leak from other fluids
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High glucose levels in CSF
Beta-2 transferrin from neural tissue |
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5 types of Hydrocephalus
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Obstructive non-communicating
Obstructive communicating Non obstructive Ex-vacuo Normal Pressure |
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Signs of infant hydrocephalus
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enlarged head
downward gaze irritable seizures sleepy vomiting |
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2 gross findings of cerebral edema
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Enlarged brain w/ flattened gyri and narrowed sulci
Increased brain weight |
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Name and describe 3 types of cerebral edema
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Cytotoxic- intracellular swelling most often due to lack of oxygen or glucose (gray matter)
Vasogenic- intercellular edema due to leaky blood vessels (cerebral hemispheric white matter) Interstitial-increased P causes transfer of fluid to interstitial compartment (perivent white matter) |
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Describe Cushing's reflex
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Increased intracranial pressure activates hypothalamic symp ganglia--> increased BP--> activates carotid body baroreceptors--> parasymp respose--> bradycardia
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Cushing's triad
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Hypertension
Bradycardia Irregular resp (Cheyne-Stokes) |
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Name 3 types of brain herniations
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Subfalcine herniation
Transtentorial herniation Tonsillar (Transoraminal herniation) |
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Herniation of the cingulate gyrus under the dural falx
Compresses the Anterior Cerebral Artery Weakness and sensory loss in contralateral leg |
Subfalcine herniation
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Herniation of medial temporal lobe (often uncus) beneath the tentorium
Ipsi 3rd cranial n compressed (palsy) Ipsi PCA compressed (visual defect) Kernohan's notch Duret hemorrhage |
Transtentorial (uncal) herniation
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Herniation of cerebellar tonsils caudally through the foramen magnum
Affects cardiac and respiratory centers |
Cerebellar tonsillar transformational herniation
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What is the relay nucleus for dorsal spinocerebellar tract?
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Clark's
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The descending major UMN pathway whose fibers eventually terminate on anterior horn cels. Sacral fibers in outer lat portion
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Corticospinal tract
Crosses at medulla: brain lesion contralateral, spinal lesion ipsilateral hemiparesis |
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Ascending Pain and Temp
ventro lateral Small unmyelinated peri nerves that enter the dorsal horn to synapse (sub gelatinosa) after going through Lissauer's tract |
Spinothalamic Tract (STT)
crosses immediately--> always contralateral |
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Large myelinated pthwy with vibration and proprioception (f. gras, f, cune)
Uncrossed sensory fibers |
Dorsal Columns/Medial Lemniscus Pthwy
Cross in medulla: brain stem/brain lesion contralateral |
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Compression of spinal cord mainly due to?
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Spondylosis or disc herniation of Cervical
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Name the metastatic cord compression organs or spinal cord compression
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Pb-Ktl: L>T>C
Prostate Breast Kidney Thyroid Lymphoreticular |
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Lhermitte's sign
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Flexion of the neck that precipitates an electrical sensation down the spine (involves posterior columns)
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Describe when one half of the spinal cord is involved in compression ie. Brown Sequard syn
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Ipsi spasticity and weakness below lesion
Ipsi loss of V & P below Contra loss of P & T |
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What is Transverse myelitis and what frequently causes it?
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Acute inflammation of the spinal cord, often due to MS
Likely Thoracic, spactic leg weakness with UMN in legs as hallmark... may have spinal cord shock w/ loss of reflexes and flaccid weakness... Sensation diminished... tight band-like... Bowel and bladder dysfunction |
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AIDS myelopathy
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slow progressive spastic weakness
Gait instability Urinary incontinence UMN Decreased V & P |
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Signs of Anterior Spinal Artery occlusion
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Acute onset of paraplegia, UMN in legs
Loss of P & T below due to ASA supplying STT |
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Poliomyelitis
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asymmetric weakness
atrophy severe cramps fasiculations ( loss of anterior horn cells) |
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What is Pseudobulbar palsy?
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Loss of UMN control of the medulla w/ resulting dysarthria and dysphagia
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What is Bulbar palsy?
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LMN degeneration of brain stem motor nuclei, primarily in the medulla (nuc ambiguus and hypoglossal XII nucleus)
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True or False: eye movements and sensory nerves are affected in ALS
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False: higher brain stem motor nuclei are relatively preserved until late as well as Cognition, sensory, bowel and bladder
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55 year old man w/ progressive weakness, muscle cramps, weight loss over last 5 months. Bad speech and chokes on food. Twitches. Dysarthric w/ nasal voice
Tongue atrophy and fasciculation and brisk jaw jerk Moderate weakness and increased tone in limbs. Prominent atrophy of hand muscles Bilateral Babinski and sustained clonus Normal sensation |
ALS
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Central cavitation that prsents with lack of P&T in both arms and shoulders due to STT
Eventually leg UMN and upper LMN dysarthria and dysphagia of medulla involved |
Syringomyelia and central spinal cord syndroms
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Why does central spinal cord tumor show sacral sparing?
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due to the fact that the sacral fibers lie outermost in the Spinal thalamic pthwy
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60 y/o man comes in with subacute combined degeneration: gait abnormality
stocking glove pain and temp loss UMN He's also experienced cognitive changes, glossitis, and anemia |
Vit B12 deficiency
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What should you do if your elderly patient yields a value of 289 pg/ml of Vit B12?
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Measure methylmalonic acid and homocysteine levels
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A 53 y/o retired stripper presents with a severe gait abnormality and severe radicular type pain. She has a slapping gait and has trouble holding her bowels and bladder. She's admitted to having syphilis in her younger days.
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Tabes Dorsalis
(late effects of neurosyphilis) |
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A 43 y/o patient presents with a sudden onset of ataxia. He walks with a slapping/stomping gait. He shows no weakness or change in P&T on exam. He does show decreased V&P.
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Posterior spinal artery occlusion
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Patient presents with gait disturbance
lower extremity spastic weakness subtle vibration loss Urinary urgency |
Hereditary Spastic Paraparesis
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35 y/o patient presents with progressive spastic paraparesis of one leg over the past 2 years. His mother's brother had the same symptoms when he was younger. You find very long chained fatty acids in his blood
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Adrenomyeloneuropathy (AMN)
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10 y/o boy presents with progressive ataxia and gait instability. He has decreased V&P on exam and displays some peripheral neuropathy. He has bilateral extensor plantar responses and other UMN. His paternal sister had the same condition. Chromosomal analysis shows a trinucleotide repeat of GAA.
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Friedreich's Ataxia
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What is being attacked in MS?
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Myelin and myelin-forming cells in the brain and spinal cord.
Defective immune responses damage oligodendroglia |
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A 25 y/o woman presents with sporatic visual loss. Each attack lasted about 2 days then went back to normal. Now she she has fully loss color vision. She has also noted a tremor in her right hand and has experienced recent difficulty swallowing. She's also noticed that when she lays out to tan, she becomes severely weak and her strength doesn't return until she's cooled down inside. This has been on and off for the past 2 years.
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MS
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A 40 y/o man comes in with progressive gait ataxia, dysarthria, and bladder dysfunction. He's had 4 recent UTI's in the past 5 months. Each month has seemed to get worse. He's been severely depressed. He has a hemiplegia in his lower extremeties. He describes feeling an electrical shock down his back and arms when he puts his chin to chest.
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MS
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50-70% of RRMS patients merge into Secondary progressive MS after 10 years. What does this mean?
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Reduced recovery from relapses
(Relapsing-remitting MS= RRMS) |
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Where are MS plaques typically seen in an MRI?
What's important to give with MRI and why? |
Seen periventricular.
Give contrast gadolinium to distinguish old/new lesions. |
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Name the 2 first line agents used for MS
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Interferon Therapy
Copaxone (Glatiramer Acetate) |
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How much energy does your brain consume?
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20% of the body's E even though it only makes up 2%
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How is the brain "picky" regarding metabolic substrates?
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Unable to use free fatty acids for Beta oxidation. Super sensitive to fluctuations in brain tissue perfusion, O2, and blood glucose
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Highest energy demand in the brain...
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Neuron
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Differentiate between hypoxia and ischemia
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Hypoxia: inadequate O2 sat w/ intact circulation
Ischemia: inadequate tissue perfusion regardless of O2 |
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Why is ischemia worse than hypoxia
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Accumulation of Toxic metabolites
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Patient comes in confused and suffering memory loss and dies a few hours later. The autopsy reveals Red deads in the basal ganglia, hippocampi, watershed neocortex, and brainstem. What happened?
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Brain Energy deprivation
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How does the brain look after brain dead individual has been on a respirator for a while?
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Hypoperfusion of dead brain: Edematous, dusky, friable brain w/ blurred gray-white junction
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Although hypoglycemeia manifests similarly to hypoxia, what is the one main difference?
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Seizures are much more common w/ hypoglycemia
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Describe Kwashiorkor PEM
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Protein deprivation w/o overall E deprivation (has carbs and lipid)
More prominent CNS abnormalities Reversible small brains in children when given adequate proteins |
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Describe Marasmus PEM
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Protein deprivation of overall energy deprivation
Fetuses from moms with marasmus have developmental abnormalities |
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Although folate deficiency has no effect on adults and children, how does it affect preggo moms?
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Increased risk of neural tube defects (spina bifida) in the fetus
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When do you see Thiamine (B1) deficiency?
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In chronic alcoholics, starvation, or any other nutrient deprived person.
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Dry beriberi vs. Wet beriberi
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Dry: Peripheral neuropathy + Wernicke's encephalopathy
Wet: High output caridovascular failure |
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3 clinical features of Wernicke's encephalopathy
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Acute: Confusion
Opthalmoplegia Ataxia |
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How does Wernicke's encephalopathy present on autopsy?
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Damaged capillaries/ hemorrhages
Neuronal necrosis of Mammillary bodies CN nuclei PAG Cerebellar vermis |
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Why must one always check thiamine levels before administering glucose to a chronic alcoholic?
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Influx of glucose rapidly depletes defiecient thiamine in the Krebs cycle
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Dori from Finding Nemo exhibits Korsakoff's amnestic syndrome. Describe her symptoms and where the lesion is
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Anterograde memory loss
Confabulation Lesions in the Dorsomedial nucleus of the thalamus |
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Classic triad of pellagra?
Deficiency? |
Dermatitis (Sun)
Diarrhea Dementia Niacin (B3) deficiency |
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What are the causes of Niacin Deficiency?
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Alcoholic
Dietary inadequacy Meds (Chemo/ INH) Hartnup's |
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How can Cyanocobalamin (B12) deficiency present?
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Reversible dementia
or Subacute Combined Degeneration: both ascending & decending myelinated tracts |
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Symptoms of B12 deficiency
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sensory loss (posterior columns)
weakness ( CST) ataxia (SCT) hporeflexia |
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Describe a brain on autopsy from CO poisoning
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Cherry red
Necrosis of the bilateral globus pallidi Widespread petechial hemorrhages in the subcortical white matter |
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Ethanol toxicity presentations
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Cerebral edema
Petechial hemorrhage Grontotemporal atrophy, hydrocephalus Trauma (subdural hemorrhage, contusions) |
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When would one see a "locked-in syndrome" in alcoholics usually?
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Central Pontine Myelinolysis (CPM)
rapid correction of ele ctrolyte disturbances, hyponatremia... Myelin destruction in the mid basis pontis |
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What would you expect to see in an alcoholic with truncal ataxia, gait abnormality, and nystagmus on autopsy?
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Cerebellar vermian atrophy of the anterosuperior aspect
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Seen in Fetal alcohol syndrome...
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Microcephaly
Corpus callosal agenesis |
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2 side effects of methanol poisoning
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Permanent blindness
Necrosis of putamen |
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Chemo that causes peripheral neuropathy
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vincristine
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What is Kernicterus?
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Elevated unconjugated bilirubin in babies that crosses the BBB
Hemolytic disease of newborn Lethargic, opisthonos Yellow discoloration of the eep gray nuclei (BG, thalami) |
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AR ATP7B gene
Inability to secrete copper into bile low serum ceruloplasmin Cirrhosis Liver flap-asterixis (damaged putamen/GP) Kayser-Fleischer rings in corneal limbus |
Wilson's disease
Familial hepatolenticular degen |
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Radiation causes acute damage to...
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White matter (radionecrosis)
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End stage liver disease causes hepatic encephalopathy and deteriorating mental status by...
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Cerebral edema
Alzheimer type II astrocytic change: hyperammonemia with chromatin clearing |
