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what do you see in the CSF in MS?
increased IgG, oligoclonal bands
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what are two symptoms of Metachromatic Leukodystrophy?
- 1. Motor and cognitive d/f
- 2. Seizures
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what causes MLD?
arylsufatase A deficiency (a lysosomal storage dz)
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Symptoms of Progressive Multifocal Leukoencenphalopathy?
- limb weakness
- cortical speech problems
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what causes PML?
- JC virus
- lymphohistiocytosis
- demyelination
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Sx of Central Pontine Myelinolysis?
- pseudobulbar palsy
- spastic quadriparesis
- mental changes
- +/- locked-in syndrome
-
Et of CPM?
- focal demyelination
- aggressive tx of hyponatremia
- EtOHics, severely malnourished, liver dz
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Sx of Guillain-Barre?
- ascending inflammatory neuropathy of PNS myelin
- autonomic d/f
- cranial nn involvement
- sensory loss or pain
- absent or decreased rflxs
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Et of GBS?
- campy....2/3 of pts have respiratory or GI dz 1-3 wks bpc
- elevated CSF protein with normal cell count: albuminocytologic dissociation
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the primary vesicle forebrain becomes the...
- telencephalon and
- diencephalon
-
the midbrain becomes the...
mesencephalon
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the hindbrain become the...
- metencephalon and
- myelencephalon
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whats in the diencephalon?
- thalamus
- pineal gland
- neurohypophysis
- hypothalamus
- retina
-
whats in the metencephalon?
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what protein does anterograde transport use?
- kinesin
- ...its fast, at 400 mm per day
-
what protein does retrograde tpt use?
- dynein
- ...tpts lysosomes and recycled membrane
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what two features are seen in stillborn infants with oligohydramnios?
- clubfeet
- pulmonary hypoplasia
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what does the adenohypophysis derive from?
Rathke's Pouch; an outgrowth of oral ectoderm
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three sx of normal pressure hydrocephalus
- confusion
- gait apraxia
- urinary incontinence
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What is a Nissl stain/body?
RER in neuron cell bodies
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What type of fibers convey proprioception?
- 1a: spindle
- 1b: Golgi tendon organ
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What type of fibers convey touch?
II, A-beta
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What type of fibers convey sharp pain and cold?
III, A-delta
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What fibers convey dull pain and warmth?
4, C
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What is in the intermediate zone of the spinal cord from T1 to L2?
- preganglionic sympathetic neuron cell bodies
- the Clarke nucleus: sends unconscious proprio to cerebellum
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What does the corticospinal tract do?
voluntary skeletal muscle
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where does the corticospinal tract originate?
in the primary motor cortex, in the precentral gyrus of the frontal lobe
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Where does the corticospinal cross?
in the brain stem, at the pyramidal decussation or the caudal medulla-spinal cord junction
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Where is the first synapse of the CST?
in the ventral horn at the same level
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What does a lesion of the corticospinal tract above the pyrimidal decussation cause?
contralateral weakness
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what does the knee stretch reflex test?
-
what does the ankle stretch reflex test?
-
what does the biceps stretch reflex test?
- C5-C6
- musculocutaneous nerve
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what does the triceps reflex test?
-
what is a clasp-knife reflex characteristic of?
UMN lesion
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what does the the dorsal column-medial lemniscus system do?
- sensory info for discriminative touch
- joint position
- vibration
- pressure
- ...from trunk and limbs
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From where does the DCML system originate, and how does it get into the cord?
the cell bodies are in the DRG, then they enter the cord via class 2 or A-beta fibers
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What are the two parts of the DCML?
- the fasciculus gracilis from the feet
- and the fasciculus cuneatus from the arms
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What are the 3 receptor cell types of the DCML?
- pacinian corpuscle
- meissner corpuscle
- muscle spindle
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Where does the DCML decussate?
it synapses in the medulla, becomes the medial lemniscus and decussates?
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Where does the medial lemniscus synapse?
in the ventroposterior-lateral nucleus of the thalamus, and then on to the post-central gyrus
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What do lesions of the dorsal columns cause?
- loss of:
- joint position sense
- vibration
- pressure
- two-point discrimination
- astereognosis: inability to identify characteristics of an object
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what does the spinothalamic tract do?
pain, temperature, crude touch
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What is the route of the spinothalamic tract?
receptor - DRG - tract of lissauer - synapse in dorsal horn - cross in spinal cord at the ventral commissure - synapse in the VPL -
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How does a lesion of the spinothalamic tract present?
Contralateral loss of pain and temperature
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What does the spinocerebellar tract do?
mostly unconscious proprioception from muscle spindles and GTOs to the cerebellum
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What does the dorsal spinocerebellar tract do?
input from the lower extremities and lower trunk
-
what does the cuneocerebellar tract do?
proprioception from upper extremities and trunk to the cerebellumj
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What is the lesion and presentation of Polio?
- deg'n of LMN of both ventral horns
- flaccid paralysis
- muscle atrophy
- fasciculations
- areflexia
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lesion and presentation of Tabes Dorsalis?
- dorsal columns, pretectal nucleus
- bilateral dorsal column signs
- broad gait
- pos Romberg: sways with eyes closed
- Progressive Parasthesias Pain and Polyuria (or oligouria...)
- Argyle Robinson pupils
-
Pres and lesion location of ALS?
- progressive atrophy of ventral horn - muscle atrophy
- primary lateral sclerosis (corticospinal tract):
spastic paralysis in lower limbs - increased tone and reflexes
- flaccid paralysis in upper limbs
-
Pres and lesion in Anterior Spinal Artery occlusion?
- all but the dorsal column
- bilateral signs
- mid-thoracic, weakness, absence of pain and temperature
- BUT vibration and pressure remain
-
What is the cause and lesion in Subacute Combined Deg'n?
- Demyelination of the dorsal columns
- spinocerebellar tracts
- corticocpinal tracts
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What is the cause, lesion, and presentation of Syringomyelia?
- cavitation of the cord, usually cervical
- bilateral loss of pain and temp at the level of the lesion
- eventually, muscle weakness, then flaccid paralysis and atrophy of the upper limb muscles due to destruction of ventral horn cells
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