Pathology Flashcards
Difference between paralysis, paresis, plegia, palsy
Paralysis: weakness so severe that muscle cannot be contracted
Paresis: weakness or partial paralysis
Plegia: severe weakness or paralysis
(Di-, hemi-, quadri-
Lower motor neuron syndrome
- Weakness/paralysis
- Atrophy
- Hypo or areflexia
- Decreased tone/flaccid paralysis
- Fibrillations (as measured by EMG)
EMG signs of denervation when muscle at rest
Fibrillation: short duration spontaneous biphasic or triphasic potentials produced by single muscle fibers, which are indicative of denervated muscle
Fasciculations: large potentials caused by spontaneous activity in one or several motor unit; large potentials suggest denervation and reinnervation
ALS
Degenerative disease of upper and lower motor neurons
Ventral horn involvement leads to lower motor neuron syndrome, while lateral corticospinal tract involvement leads to upper motor neuron syndrome
Loss of motor neurons in ventral horn cause fasciculations
No sensory loss.
Most are sporadic, but familial cases can be caused by a zinc-copper superoxide dismutase mutation (needed to bind free radicals)
Common causes of lower motor neuron syndrome
- Lesions in peripheral, spinal, or cranial nerves
- Lesions in cauda equina
- Strokes or tumors affecting alpha motor neurons in ventral horn or brainstem
- Polio
- ALS
- Guillain Barre
- Werdnig-Hoffman
Polio
Virus that affects alpha motor neuron’s ventral horn, so can lead to lower motor neuron syndrome
Can see period where patient is neurologically stable for years followed by progressive weakness in same muscles originally affected. This is because sprouting allows for recovery and stable period, but sprouts cannot be sustained.
Spinal Muscle Atrophy
Group of diseases caused by degeneration of anterior horns due to abnormalities in chromosome 5
Progressive disease that begins in infancy
4 types; first type is Werdnig-Hoffman
Affects motor neurons in the spinal cord and cranial nerve motor nuclei
Werdnig-Hoffman disease
Spinal Muscle Atrophy, Type I, inherited degeneration of anterior motor horn
Aka floppy baby
Autosomal recessive inheritance
Deadly
Symptoms:
Weakness and wasting in limb muscles, respiratory muscles, and bulbar muscles (as shown by sucking, swallowing, and breathing)
Upper Motor Neuron Lesions
Spinal shock followed by
- Paresis
- Hyper-reflexia
- Hypertonia
- Posturing
Variations on posturing with upper motor neuron syndrome
Decorticate when lesion above midbrain (upper limbs flexed, lower limbs extended)
Decerebrate when lesion below the midbrain (all limbs extended)
Babinski reflex
Indicates corticospinal tract lesion (upper motor neuron syndrome) if toes curl up (extensor plantar response) instead of down when lateral plantar surface is stroked
Normal in babies (before myelination)
Hoffman’s sign
Hold patient’s finger loosely and flick the fingernail downward, which should cause finger to rebound into extension.
If thumb flexes and adducts, it is positive Hoffman’s, indicated upper motor neuron syndrome
Bell’s Palsy
Paralysis of half the face due to lesion in CN VII
Common causes of upper motor neuron syndrome
Trauma
Stroke
MS
ALS
Cerebral Palsy
Non-progressive neurological disorder that appears in infancy or early child
Impacts body movement and muscle coordination; may cause intellectual disability
Can be caused by ischemia at birth, hypo-perfusion, trauma, or hemorrhage
Radiculopathy
Damage to spinal nerve, most commonly caused by herniated discs but also caused by osteophytes and spinal stenosis
Symptoms:
- Burning, tingling pain that radiates from back along a dermatome
- Numbness
- worsening of symptoms with coughing, sneezing, or straining; worsening of symptoms with flexing the head (in case of cervical radiculopathy)
- muscle weakness
Horner’s Syndrome
Interruption of sympathetic pathway to the head, eye, or neck caused by a lesion at T1 or above
Symptoms:
- miosis
- anhidrosis of face
- ptosis
Spinal shock
Spinal cord injury that causes immediate flaccid paralysis below the lesion
Usually lasts 1-6 weeks
Due to loss of descending facilitation that keep spinal cord circuits in continuous state of activation/readiness
WHO Grading System for Pathohistological Features of Tumors
I: curable with complete surgical resection
II: even complete resection may not be curative (~7-10 years)
III: not surgically curable; may kill on its own or up on version to grade IV (~3-7 years)
IV: not surgically curable; chemo/rad-to can extend life, but response is typically short-lived; quickly kills (~12-18 months)
Glioma
Tumor arising from glia
Pilocytic astrocytoma
Grade I/IV
Usually in kids, arising below tentorium
Cyst with nodule
Low grade, minimally infiltrative
Elongated cells with hairlike processes; Rosenthal fibers (thick eosinophilic processes)
GFAP+
Diffuse astrocytoma
Grade II, III, or IV
Irregular, elongated, crowded nuclei
GFAP+
Infiltrates normal brain to make surgical resection very difficult
The later the onset, the higher the grade
Glioblastoma
Astrocytoma
Grade IV
Most common malignant CNS tumor
Malignant, poorly differentiated tumor of glial cells
Application of receptor tyrosine kinases, proto-oncogenes (especially EGFR) which promotes its own growth
Butterfly lesion-crosses corpus callosum
May see microvascular proliferation or palisading necrosis (living cells at the edge of the necrosis)
GFAP+
Oligodendroglioma
Neoplasm of oligodendroglial cells Calcified tumor in white matter Grade II or III Usually in cerebral (frontal lobe) hemispheres Often hemorrhagic; causing seizures Genetics: co-deletion of 1p an 19q Fried egg nuclei May see chickenwire vasculature or calcifications
Ependymoma
Tumor of ependymal cells
Grade II or III
Usually in children beneath tentorium (4th ventricle)
Most common in 4th ventricle (very aggressive in children) or spinal cord (low grade and surgically treatable)
Perivascular pseduorosettes (tumor cells line up against blood vessels with pink material inbetween)
Pituitary adenoma
Low grade neuroendocrine tumor
Most common is a null-cell adenoma (not making hormones)
Most common hormone productions adenoma=protlactinoma
If it presses against optic chiasm can cause superior quadrantonopia
Prolactinoma-amenorrhea, galactorrhea
Growth hormone-acromegaly
ACTH-Cushing’s disease
Craniopharyngioma
Pituitary tumor in young kids
Supra tentorial in child and adult
Can cause inferior quadrantonopia
Derived from remnants of Rathke’s pouch (origin of anterior pituitary)
See squamous epi, “wet keratin”, and cholesterol clefts
Look at root of word!! Crani-, pharyng-
Calcifications seen on imagining
Schwannoma
Benign tumor; Grade I
Caused by bi-allergic inactivation of NF2 gene
Symptoms caused by compression of nerves, particularly vestibular nerve, which made lead to tinnitus and deafness
Bland-spindle shaped cells
Hypercellular=Antoni A
Hypocellular=Antoni B
Verocay bodies=nuclei lining up together
especially at cerebellar pontine angle, involving CN8.
S100+
Meningioma
Dural based tumor of meningothelial cells
Will see dural tail on MRI
Grade I-III
50% have NF2 mutation
Histo shows whorls and calcifications (psammomatous)
Most common benign tumor
Tumor expresses estrogen receptor; more common in women
Hemangioblastoma
Grade I lesion
Occurs from mutation in VHL, allowing for constitutive activation of HIF
Usually occurs in cerebellum, but also seen in spine, brainstem, and retina
Histo: foamy cells and dense capillary network