Neurology Pathology 2

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Neurology Pathology 2
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  1. Horner's Syndrome
    "Sympathectomy of face"

    • Pathophysiology:
    • -associated with lesion of spinal cord above T1
    • -Pancoast tumor
    • -Brown Sequard syndrome
    • -late stage syringomyelia

    • Presentation:
    • -Ptosis (superior tarsal muscle)
    • -Anhidrosis and rubor
    • -Miosis (pupil constriction)

    "PAM is horny (Horner's). Ptosis, anhidrosis and miosis"

    • Sympathetic Physiology:
    • 1. Hypothalamus to intermediolateral column of the spinal cord
    • 2. to superior cervical (sympathetic) ganglion
    • 3. to the pupil, smooth muscle of the eyelids, sweat glands of forehead and face

  2. Parinaud Syndrome
    • Pathophysiology:
    • -lesion of superior colliculi
    • -eg: pinealoma

    • Presentation:
    • -paralysis of conjugate vertical gaze
  3. Cavernous Sinus Syndrome
    • Pathopysiology:
    • -CN III, IV, V and VI compressed (run through cavernous sinus)
    • -eg: due to mass effect, fistula, thrombosis

    • Presentation:
    • -ophthalmoplegia
    • -↓ corneal and maxillary sensation
    • -NORMAL vision
  4. Common Cranial Nerve Lesions
    • 1. CN V
    • 2. CN X
    • 3. CN XI
    • 4. CN XII (LMN)
  5. CN V motor lesion
    • Presentation:
    • -jaw deviates TOWARD side of lesion, due to unopposed force from the opposite pterygoid muscle
  6. CN X lesion
    • Presentation:
    • -uvula deviates AWAY from side of lesion
    • -weak side collapes and uvula points away
  7. CN XI lesion
    • Presentation:
    • -weakness turning head to contralateral side of lesion (SCM)
    • -shoulder droop on side of lesion (trapezius)

    *the left SCM contracts to help turn the head to the right
  8. CN XII Lesion
    • Presentation:
    • -tongue deviates TOWARD side of lesion
    • -due to weakened tongue muscles on affected side

    "lick your wounds"
  9. Hearing Loss
    -Rinne Test
    -Weber Test

    -Conductive Hearing Loss
    -Sensorineural Hearing Loss
    -Noise-Induced Hearing Loss
    • Rinne Test
    • -abnormal in ear with conductive hearing loss (bone > air)
    • -normal in ear with sensorineural hearing loss (air > bone)

    • Weber Test
    • -louder in ear with conductive hearing loss

    • Conductive Hearing Loss:
    • -problem conducting sound waves anywhere from outer ear, tympanic membrane to middle ear

    • Sensorineural Hearing Loss:
    • -problem in the vestibulocochlear nerve, inner ear or central processing in the brain

    • Noise-Induced Hearing Loss:
    • -damaged to stereociliated cells in the organ of Corti
    • -loss of high frequency hearing first
    • -sudden extremely loud noises can produced hearing loss due to tympanic membrane rupture
  10. Facial Lesions
    • 1. UMN
    • 2. LMN
    • 3. Facial nerve palsy

  11. UMN Facial Lesion
    • Lesion:
    • -motor cortex or connection between the cortex and facial motor nucleus (caudal pons)

    • Presentation:
    • -contralateral paralysis of lower face
    • ***Forehead spared due to bilateral UMN innervation
  12. LMN Facial Lesion
    • Lesion:
    • -below the pons

    • Presentation:
    • -ipsilateral paralysis of upper AND lower face
  13. Facial Nerve Palsy
    • Lesion:
    • -complete destruction of the facial nucleus itself or its branchial efferent fibers (facial nerve proper)
    • -Bell's Palsy: can occur idiopathically (gradual recovery in most cases)

    • Complication in:
    • -Lyme disease
    • -AIDS
    • -HSV

    • Less common Complication in:
    • -VZV
    • -sarcoidosis
    • -tumors
    • -diabetes

    • Presentation:
    • -peripheral ipsilateral facial paralysis
    • -inability to close eye on involved side
  14. Dementia
    A decrease in cognitive ability, memory or function with INTACT CONSCIOUSNESS

    • General Pathophysiology:
    • -loss of neurons within the gray matter (often due to protein accumulation)
    • -degeneration of cortex leads to dementia
    • -degeneration of brainstem and basal ganglia leads to movement disorders

    • Diseases:
    • 1. Alzheimer's
    • 2. Pick's Disease (Frontotemporal Dementia)
    • 3. Lewy Body Dementia
    • 4. Creutzfeldt-Jakob Disease (CJD)
    • 5. Vascular Dementia
    • 6. Other causes
  15. Dementia: Alzheimer's Disease
    • Epidemiology:
    • -most common cause of dementia in the elderly
    • -Sporadic form (95%)
    • -Familial form (10%)
    • -Down Syndrome: commonly occurs by 40

    • Clinical Features:
    • -slow-onset memory loss
    • -progressive disorientation
    • -loss of learned motor skills and language
    • -changes in behaviour and personality
    • -patients become mute and bedridden
    • -infection = most common cause of death
    • -focal neurologic deficits not seen in early disease

    • Pathophysiology:
    • 1. Sporadic
    • -risk increases with age (doubles every five years after 60)
    • -ApoE4 carries increased risk
    • -ApoE2 is protective
    • 2. Familial
    • -early onset: APP (chrom 21), presenilin-1 (chrom 14), presenilin-2 (chrom 1)
    • -Late onset: ApoE4

    • Aβ Amyloid Pathophysiology:
    • -APP (chrom 21) normally undergoes α-cleavage
    • -in Alzheimer's undergoes β-cleavage resulting in Aβ amyloid

    • Pathology:
    • -cerebral atrophy
    • -Decreased ACh (loss of cholinergic neurons in nucleus basalis of Meynert)

    • Histology:
    • 1. Neuritic Plaques (senile plaques)
    • -extracellular
    • -β-amyloid core
    • -entangled neuritic processes
    • *Amyloid may also deposit around vessels → amyloid angiopathy (increased risk of hemorrhage)

    • 2. Neurofibrillary Tangles
    • -intracellular
    • -abnormally phosphorylated tau protein
    • -leads to insoluble cytoskeletal elements
    • -correlate with degree of dementia
  16. Dementia: Pick's Disease (Frontotemporal Dementia)
    • Pathophysiology:
    • -degenerative disease of the frontal and temporal cortex (vs Alzheimer's)
    • -spares parietal lobe and posterior 2/3 of superior temporal gyrus

    • Clinical Presentation:
    • -behavioural and language sx arise early, eventually progresses to dementia
    • -dementia
    • -aphasia
    • -parkinsonism aspects
    • -change in personality

    • Pathology:
    • 1. Frontotemporal atrophy
    • 2. Pick bodies
    • -spherical tau protein aggregates
    • -in neurons of cortex
  17. Dementia: Lewy Body Dementia
    • Pathophysiology:
    • -unknown
    • -defect in α-synuclein

    • Clinical Presentation:
    • -overlap with Parkinsons Disease and Alzheimers
    • -see PD and hallucinations/dementia presenting within the same year

    • Pathology:
    • -Lewy bodies in cortex (vs. Parkinsons)
  18. Dementia: Creutzfeldt-Jakob Disease (CJD)
    • Pathophysiology:
    • -Prion protein is normally expressed in CNS neurons in an α-helical conformation (PrPc)
    • -conversion to β-pleated sheet form (PrPsc)
    • -PrPsc is resistant to proteases and converts the normal form to the pathologic form
    • -PrPsc damages neurons and glial cells → vacuoles
    • -can be inherited or sporadic (exposure to prion-infected human tissue)

    • Clinical Presentation:
    • -rapidly progressive (weeks - months) dementia
    • -"startle myclonus"
    • -death within one year

    • Pathology:
    • -spongiform changes in cortex
  19. Dementia: Vascular Dementia
    • Pathophysiology:
    • -multifocal infarction and injury due to HTN, atherosclerosis or vasculitis
    • -second most common cause of dementia
    • -decreased blood flow to the brain affects specific areas first and worst

    • Susceptible Areas:
    • 1. Hippocampus
    • 2. Cortex pyramidal neurons
    • 3. Purkinje neurons of the cerebellum

    **if relieve ischemia may improve sx
  20. Dementia: Other causes
    • -Syphilis
    • -HIV
    • -Vitamins B1, B3 or B12 Deficiency
    • -Wilson's Disease
    • -Normal Pressure Hydrocephalus
  21. Multiple Sclerosis
    • Epidemiology:
    • -most often affects women in their 20-30s
    • -more common in whites
    • -most common in regions away from the equator
    • -HLA-DR2

    • Pathophysiology:
    • -autoimmune inflammation and demyelination of CNS (brain and spinal cord)
    • -destruction of myelin and oligodendrocytes

    • Clinical Presentation:
    • -"multiple lesions in time and space"
    • -relapsing and remitting
    • -optic neuritis (sudden loss of vision)
    • -MLF syndrome (internuclear ophthalmoplegia)
    • -hemiparesis
    • -hemisensory sx
    • -bladder/bowel incontinence
    • -vertigo/scanning speech (mimic EtOH intoxication)

    • "Charcot's classic triad of MS is a SIN":
    • -Scanning speech
    • -Intention tremor, Incontinence, Internuclear ophthalmoplegia
    • -Nystagmus

    • Findings:
    • -↑ IgG in CSF
    • -oligoclonal bands in CSF (diagnostic)
    • -increased lymphocytes
    • -MRI: periventricular plaques (areas of oligodendrocyte loss and reactive gliosis)

    • Treatment:
    • -β-interferon (slows progression of disease)
    • -immunosuppression (high dose steroids for acute attacks)
    • -natalizumab
    • -symptomatic tx of neurogenic bladder (cath, muscarinic antagonists)
    • -symptomatic tx of spasticity (baclofen, GABA R antagonist)
    • -symptomatic tx of pain (opioids)
  22. Acute Inflammatory Demyelinating Polyradiculopathy (Guillain-Barre)
    *Most common variant of Guillain-Barre Syndrome

    • Pathophysiology:
    • -autoimmune condition that destroys Schwann cells
    • -inflammation and demyelination of peripheral nerves and motor fibers
    • -associated with infections (Campylobacter jejuni and CMV): autoimmune attack due to molecular mimcry, inoculations and stress
    • *no definitive link to pathogens

    • Clinical Presentation:
    • -symmetric ascending muscle weakness/paralysis
    • -begins in lower extremities
    • -facial paralysis in 50%
    • -autonomic function may be affected (cardiac irregularities, hypertension/hypotension)

    • Findings:
    • -↑ CSF protein
    • -normal cell count in CSF
    • *albuminocytologic dissociation
    • -↑ protein → papilledema

    • Treatment/Prognosis:
    • -almost all patients survive
    • -majority recover after weeks to months
    • -respiratory support is critical
    • -plasmapheresis, IV Ig
  23. Other Demyelinating/Dysmyelinating Diseases
    • 1. PML
    • 2. Acute disseminated (postinfectious) encephalomyelitis
    • 3. Metachromatic leukodystrophy
    • 4. Charcot-Marie-Tooth Disease
    • 5. Krabbe's Disease
  24. Progressive Multifocal Leukoencephalopathy (PML)
    • Pathophysiology:
    • -demyelination of CNS due to destruction of oligodendrocytes (white matter)
    • -associated with JC virus

    Seen in 2-4% of AIDS patients (reactivation of latent viral infection)

    • Clinical Presentation:
    • -rapidly progressive neurologic signs (visual loss, weakness, dementia)
    • -usually fatal

    *potential adverse effect of Natalizumab
  25. Acute Disseminated (postinfectious) Encephalomyelitis
    -Multifocal perivenular inflammation and demyelination after infection (commonly measles or VZV) or certain vaccinations (rabies, smallpox)
  26. Metachromatic Leukodystrophy
    • Pathophysiology:
    • -autosomal recessive lysosomal storage disease
    • -most commonly due to arlysulfatase A deficiency
    • -build up of sulfatides leads to impaired production of myelin sheath
    • -myelin cannot be degraded and accumulates in the lysosomes of oligodendrocytes
  27. Charcot-Marie-Tooth Disease
    Hereditary Motor and Sensory Neuropathy (HMSN)

    • Pathophysiology:
    • -group of progressive hereditary nerve disorders related to the defective production of proteins involved in the structure and function of peripheral nerves of the myelin sheath

    • Clinical Presentation:
    • -progressive loss of muscle tissue and touch sensation
  28. Krabbe's Disease
    • Pathophysiology:
    • -autosomal recessive lysosomal storage disease
    • -deficiency of galactocerebrosidase
    • -build up of galactocerebroside in macrophages destroys myelin sheath
  29. Seizures
    • -characterized by synchronized, high frequency neuronal firing
    • -variety of forms

    • 1. Partial (focal) seizures
    • 2. Generalized
  30. Partial (focal) Seizures
    • -affect one area of the brain
    • -most commonly originate in medial temporal lobe
    • -often preceded by aura
    • -can secondarily generalize

    • Causes of seizures by age:
    • -Children: genetic, infection (febrile), trauma, congenital, metabolic
    • -Adults: tumors, trauma, stroke, infection
    • -Elderly: stroke, tumor, trauma, metabolic, infection

    • Simple Partial
    • -consciousness intact
    • -motor, sensory, autonomic, psychic

    • Complex Partial
    • -impaired consciousness

    • Epilepsy
    • -disorder of recurrent seizures
    • *febrile seizures are NOT epileptic

    • Status Epilepticus
    • -continuous seizures for > 30 minutes or recurrent seizures without regaining consciousness between seizures for > 30 minutes
    • -medical emergency
  31. Generalized Seizures
    -Diffuse

    • Absence
    • -petit mal
    • -3 Hz
    • -no postictal confusion
    • -blank stare

    • Myoclonic
    • -quick, repetitive jerks

    • Tonic-Clonic
    • -grand mal
    • -alternating stiffening and movement

    • Tonic
    • -stiffening

    • Atonic
    • -"drop" seizures (falls to floor)
    • -commonly mistaken for fainting
  32. Headaches
    -pain due to irritation of structures such as the dura, cranial nerves, or extracranial structures

    • Types:
    • 1. Cluster
    • 2. Tension
    • 3. Migraine

    • Other Causes:
    • -subarachnoid hemorrhage ("worst headache of my life")
    • -meningitis
    • -hydrocephalus
    • -neoplasia
    • -arteritis
  33. Cluster Headaches
    • Localization:
    • -unilateral

    • Duration:
    • -15 min to 3 hours
    • -repetitive

    • Description:
    • -repetitive brief headaches
    • -excruciating periorbital pain
    • -lacrimation
    • -rhinorrhea
    • -may induce Horner's Syndrome
    • -more common in males

    • Treatment:
    • -inhaled oxygen
    • -sumatriptan

    • Differentiating from Trigeminal Neuralgia:
    • -duration
    • -TN: produces repetitive shooting pain in the distribution of CN V that lasts < 1 minute, cluster HAs last longer
  34. Tension Headache
    • Localization:
    • -bilateral

    • Duration:
    • -> 30 minutes
    • -typically last 4-6 hours
    • -constant

    • Description:
    • -steady pain
    • -no photophobia or phonophobia
    • -no aura
  35. Vertigo
    • -Sensation of spinning while actually stationary
    • -Subtype of dizziness, but distinct from lightheadedness

    • Peripheral Vertigo
    • -most common
    • -inner ear etiology (eg: SCC debris, vestibular nerve infection, Meniere's Disesase)
    • -Positional Testing → delayed horizontal nystagmus

    • Central Vertigo
    • -brain stem or cerebellar lesion (eg: stroke affecting vestibular nuclei or posterior fossa tumor)
    • -directional change nystagmus, skew deviation, diplopia, dysmetria
    • -Positional testing → immediate nystagmus in any direction, may change direction
  36. Neurocutaneous Disorders
    • 1. Sturge-Weber Syndrome
    • 2. Tuberous Sclerosis
    • 3. Neurofibromatosis type I
    • 4. Von Hippel Lindau Disease
  37. Sturge-Weber Syndrome
    • Etiology:
    • -congenital disorder
    • -occurs sporadically
    • -abnormal blood vessels on the brain surface

    • Presentation:
    • -port-wine stains (nevus flammeus), typically in V1 ophthalmic distribution
    • -ipsilateral leptomeningeal angiomas
    • -pheochromocytomas

    • Can Cause:
    • -glaucoma
    • -seizures
    • -hemiparesis
    • -mental retardation
  38. Tuberous Sclerosis
    • "HAMARTOMAS"
    • Hamartomas in CNS and skin
    • Adenoma sebaceum (cutaneous angiofibromas)
    • Mitral regurgitation
    • Ash-leaf spots
    • cardiac Rhabdomyoma
    • (Tuberous sclerosis)
    • autosomal dOminant
    • Mental retardation
    • renal Angiomyolipoma
    • Seizures

    • Ash leaf spots

    • Cutaneous angiofibromas
  39. Neurofibromatosis type I
    "Von Recklinghausen's disease"

    • Etiology:
    • -autosomal dominant
    • -100% penetrant
    • -variable expression
    • -mutation in NF1 gene (chrom 17)

    • Presentation:
    • -cafe-au-lait spots
    • -Lisch nodules (pigmented iris hamartomas)
    • -neurofibromas in skin
    • -optic gliomas
    • -pheochromocytomas
  40. von Hippel Lindau Disease
    • Etiology:
    • -autosomal dominant
    • -mutation in VHL (chrom 3)

    • Presentation:
    • -cavernous hemangiomas in skin, mucosa, organs
    • -bilateral renal cell carcinoma
    • -hemangioblastoma in retina, brain stem, cerebellum
    • -pheochromocytomas
  41. Adult Primary Brain Tumors
    • 1. Glioblastoma multiforme (grade IV astrocytoma)
    • 2. Meningioma
    • 3. Schwannoma
    • 4. Oligodendroglioma
    • 5. Pituitary Adenoma
  42. Glioblastoma
    Grade IV Astrocytoma

    • Epidemiology:
    • -most common primary brain tumor
    • -malignant

    • Pathology:
    • -found in cerebral hemispheres
    • -can cross corpus callosum ("butterfly glioma")

    • Histology:
    • -stain astrocytes for GFAP
    • -endothelial cell proliferation
    • -pseudopallisading pleomorphic tumor cells (border central areas of necrosis and hemorrhage)

    • Prognosis:
    • -less than 1 year life expectancy
  43. Meningioma
    • Epidemiology:
    • -second most common primary brain tumor
    • -more common in women
    • -arise from arachnoid cells

    • Pathology:
    • -most often occurs in convexities of hemispheres (near surface of brain) and parasagittal region
    • -extra-axial (external to brain parenchyma)
    • -may have a dural attachment ("tail")

    • Histology:
    • -spindle cells concentrically arranged in a whorled pattern
    • -psammoma bodies (laminated calcifications)
    • Presentation:
    • -often asymptomatic
    • -tumor compresses but does not invade the cortex
    • -may present with seizures or focal signs

    • Prognosis:
    • -typically benign and resectable
  44. Schwannoma
    • Epidemiology:
    • -third most common primary brain tumor

    • Pathophysiology:
    • -benign tumor of Schwann cells
    • -commonly involves CNs

    • Pathology:
    • -often localizes to CNVIII at cerebellopontine angle (acoustic neuroma) → loss of hearing and tinnitus
    • **bilateral tumors seen in NF2

    • Histology:
    • -S-100 positive

    • Treatment:
    • -resectable or treated with stereotactic radiosurgery
  45. Oligodendroglioma
    Malignant tumor of oligodendrocytes

    • Epidemiology:
    • -relatively rare
    • -slow growing

    • Presentation:
    • -may present with seizures

    • Pathology:
    • -most often in frontal lobes
    • -chicken-wire capillary pattern
    • -often calcified

    • Histology:
    • -fried egg appearance (round nuclei with clear cytoplasm)
  46. Pituitary Adenoma
    Most commonly prolactinoma

    • Presentation:
    • -bitemporal hemianopia (due to pressure on optic chiasm)
    • -hyper/hypo-pituitary sequelae
  47. Childhood Primary Brain Tumors
    • 1. Pilocytic (low-grade) astrocytoma
    • 2. Medulloblastoma
    • 3. Ependymoma
    • 4. Hemangioblastoma
    • 5. Craniopharyngioma
  48. Pilocytic Astrocytoma
    Benign tumor of astrocytes

    • Epidemiology:
    • -most common CNS tumor in children

    • Pathology:
    • -usually well circumscribed
    • -most commonly found in posterior fossa (cerebellum)
    • -may be supratentorial
    • -cystic lesion with mural nodule

    • Histology:
    • -Rosenthal fibers (eosinophilic, corkscrew fibers)
    • -GFAP positive

    • Prognosis:
    • -benign
    • -good prognosis
  49. Medulloblastoma
    • Etiology:
    • -highly malignant cerebellar tumor
    • -a form of primitive neuroectodermal tumor (derived from granular cells of the cerebellum)
    • -usually arises in small children

    • Pathology:
    • -can compress 4th ventricle → hydrocephalus
    • -solid tumor

    • Histology:
    • -Homer-Wright rosettes (small round blue cells wrap around neuritic processes)
    • -small blue cells

    • Prognosis and Treatment:
    • -poor prognosis
    • -tumor grows rapidly and spreads via CSF-can send "drop metastases" to spinal cord
    • -radiosensitive
  50. Ependymoma
    • Pathology:
    • -ependymal cell tumor
    • -most commonly found in 4th ventricle
    • -can cause hydrocephalus

    • Histology:
    • -perivascular pseudorosettes (characteristic)
    • -rod shaped blepharoplasts (basal ciliary bodies) found near nucleus

    Poor prognosis
  51. Hemangioblastoma
    • Epidemiology:
    • -associated with von Hippel-Lindau syndrome when found with retinal angiomas

    • Pathology:
    • -most often cerebellar
    • -can produce Epo → secondary polycythemia

    • Histology:
    • -foamy cells and high vascularity
  52. Craniopharyngioma
    • Epidemiology:
    • -benign childhood tumor
    • -often confused with pituitary adenoma
    • -most common childhood supratentorial tumor

    • Pathophysiology:
    • -arises from remnants of Rathke's pouch (gives rise to anterior pituitary)
    • -derived from the floor of the mouth (grows up toward the base of the brain)

    • Presentation:
    • -supratentorial mass in child or young adult
    • -can cause bitemporal hemianopia

    • Pathology:
    • -calcifcations are common ("tooth-like" tissue)

    • Prognosis:
    • -benign
    • -but tends to recur after resection
  53. Herniation Syndromes


    • 1. Cingulate (subfalcine) Herniation under falx cerebri
    • -can compress ACA

    2. Downward Transtentorial (central) Herniation

    • 3. Uncal Herniation
    • -uncus = medial temporal lobe

    • 4. Cerebellar tonsillar herniation into the foramen magnum
    • -coma and death result when the herniations compress the brain stem

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