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32 Cards in this Set
- Front
- Back
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What is the cause of Wernicke-Korsakoff Syndrome?
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Thiamine (B1) deficiency (can cause central lesions as well as peripheral neuropathy)
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What conditions can lead to Wernicke-Korsakoff Syndrome? (6)
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Ethanol abuse
Malnutrition Pernicious vomiting of pregnancy Thyrotoxicosis Gastric carcinoma Total parenteral nutrition w/out B1 |
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What is the clinical triad seen in Wernicke's encephalopathy?
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Nystagmus
EOM weakness (especially CN 6) Ataxia |
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Describe the korsakoff psychosis part of Wernicke-Korsakoff Syndrome?
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Disordered memory function (both anterograde and retrograde)
Impaired learning abilities Confabulation (make stuff up) |
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Gross morphology of Wernicke-Korsakoff Syndrome?
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Hemorrhage and necrosis of:
**Mammillary bodies** periaqueduct region of midbrain floor of fourth ventricle dorsal medial thalamus |
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Microscopic appearance of Wernicke-Korsakoff Syndrome?
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Neuronal and myelin necrosis
Hemorrhage due to vascular permeability Gliosis (fibrosis of CNS) |
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What is the cause of Subacute Combined Degeneration of the Spinal Cord? Age of pts?
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b12 deficiency
Mostly seen in 60-80 yr olds |
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What conditions can lead to a B12 deficiency?
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Pernicious anemia
Gastrectomy (no IF) Primary hypothyroidism Malabsorption D. latum (fish tapeworm) |
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Clinical presentation of Subacute Combined Degeneration of the Spinal Cord?
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Symmetrical spastic lower extremity weakness, band-like sensations around joints, sensory ataxia (post columns affected)
Loss of proprioception (lateralcortico) +Babinski (corticospinal) psych sx ("megaloblastic madness" - end stage, irrev). |
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What causes the sx of Subacute Combined Degeneration of the Spinal Cord?
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Demyelination of posterior and lateral columns
Peripheral nerves spared |
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What's the CSF look like in Subacute Combined Degeneration of the Spinal Cord?
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Normal
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Treatment for Subacute Combined Degeneration of the Spinal Cord?
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b12 IM - prompt tx needed for complete recovery
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What's the Chiari I malformation?
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Cerebellar tonsils lie in vertebral canal (by poking through the foramen magnum)
May obstruct CSF flow |
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What are the typical Chiari II malformations? (Arnold-Chiari)
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Small posterior fossa + Misshapen midline
Cerebellar Vermis extends through foramen magnum Hydrocephalus Lumbar myelomeningocele |
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What's a lumbar myelomeningocele?
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Opening in the posterior lumbar spine = meninges and portion of spinal cord protrudes out of subQ tissue (just meninges = mengiocele)
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What are the less common Chiari II malformations?
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Caudal displacement of medulla
Malformation of tectum Aqueduct stenosis Hydromyelia Cerebral heterotopias |
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What's Dandy Walker Syndrome?
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Cerebellar vermis is hypoplastic/aplastic
Fourth ventricle dilated Posterior fossa enlarged |
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What's Spina bifida?
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Spinal canal fails to close. Can be asx or have herniation of the meninges +/- spinal cord
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What's spina bifida occulta?
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No herniation. Can be symptomatic though.
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Clinical Features of Guillain-Barre Syndrome?
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Rapidly progressive motor neuropathy (2-3 wks) = severe muscle weakness with hypotonia, areflexia, and occassional sensory deficits
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Where does Guillain-Barre Syndrome typically start?
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Typically begins in bilateral legs, sometimes progressing to total paralysis w/ need for ventilation support
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CSF seen with Guillain-Barre Syndrome?
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Markedly increase proteins (150) and few cells (1 lymphocyte) - uncommon except with GBS.
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Causes of Guillain-Barre Syndrome?
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Unknown/immunologic
Occurs spontaneously or following viral/other infection, allergic reaction, vaccination, or surgery |
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Treatment of Guillain-Barre Syndrome?
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Supportive only
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Course seen in Guillain-Barre Syndrome?
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Most recovery completely, some have residual deficits, and only a few die (mostly the old/really sick)
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What is a Schwannoma?
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Common benign tumor derived from the Schwann cell that can occur anywhere
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What's an acoustic neuroma?
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A Schwannoma that occurs on the 8th cranial nerve
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Gross appearance of a schwannoma?
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Encapsulated
Attached to nerve but does not expand it |
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Microscopic appearance of a schwannoma?
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Focally cellular with Verocay bodies
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What's a neurofibroma?
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Common benign tumor found in dermis/subq, dervied from nerve sheath (unencapsulated = expand nerves). Can be solitary/multi
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What conditions are associated with multiple neurofibromas?
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neurofibromatosis type I
Von-Recklinghausen syndrome |
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Micro appearance of a neurofibroma?
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Homogenous with oval shaped cells (no pleomorph/hyperchrome and only rare mitosis = benign)
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