Neurological Diseases Flashcards
Multiple Sclerosis
80%: most common demyelinating disease
a cell-mediated (t cell) autoimmune disease of brain and spinal cord leading to central demyelination from scarring of myelin sheaths directly (Type IV autoimmune reaction)
Average onset is 29 years old
RF: low vitamin D, smoking, genetic predisposition, northern latitude, WHITE > everyone else
What are the types of MS?
Clinically Isolated Syndrome
Relapsing-Remitting: 85%
Primary Progressive: straight line (10%)
Secondary Progressive: rarest form
Progressive Relapsing: 5%
What are the ocular symptoms of MS
optic neuritis + CN palsy and Nystagmus
how do you dx MS?
2+ attacks with lesions on MRI
what is NMO
neuromyelitis optica - Devic’s Disease
autoimmune disorder where optic nerve and spinal cord is affected by an immune attack on AQ4 (Type II rxn)
IgG Antibody in 70% of NMO patients
more women than men (8:1) and onset around 40
how do you dx NMO
MRI with 3+ vertebral segments, IgG Antibody, no brain lesions on MRI
what is Guillain barre syndrome
acute and idiopathic polyneuritis
peripheral NS demyelination and progressive ASCENDING numbness/paralysis
caused by GI or respiratory infection (V/B)
what is the ocular variant of GB syndrome
miller fisher syndrome
What is myasthenia gravis?
autoimmune disorder of NMJ where antibody blocks Ach and inactivates voluntary muscle
onset around 20
Thymoma Patients: 50% develop MG
what are the ocular manifestations of MG
ptosis + fluctuating diplopia + alternating CNP
how do you dx MG
Tensilon (+) in 90%, Ice pack test (on droopy eyelid), EMG, Anti-Ach Receptor antibody
What is bell’s palsy
inflammation of CN VII (facial nerve) and most common cause of facial paralysis
usually idiopathic + rapid onset
Unilateral facial weakness, facial droop, diminished eye blink, hyperacusis, decreased lacrimation
what are the ocular manifestations of traumatic brain injuries?
VFD / Neglect, CNP, Accommodative Dysfunction, CI, Photophobia
what are the 3 locations a brain tumor can come from?
lung > breast > colorectal
what are the symptoms of a brain tumor?
HA (increased ICP), papilledema, vomit, dysphagia, personality change + VFD/neglect, loss of vision, CNP, Retinal Detachment
What is cerebral palsy
group of syndromes – permanent, nonprogressive neuronal damage of motor control
most common motor disability in children
Spastic: most common form: stiff + tight muscles
Ataxic: shaky movements
Dyskinetic: involuntary movements
what are the ocular manifestations in CP (40% of patients)
strabismus, RE, nystagmus, amblyopia, optic atrophy and VFD
what is muscular dystrophy
progressive genetic disorder where striated muscle is degenerated
what is duchenne dystrophy
x-linked disease that is most common form where absence of dystrophin protein in striated muscle and muscle fibers is replaced by fat + CT
death by 30s due to dilated cardiomyopathy
what is amyotrophic lateral sclerosis (ALS)
progressive neurodegenerative disorder of motor neurons without dementia
onset at 55 year old and idiopathic (90%)
RF: smoking, lead, military services
weakness of lower and upper extremities + tongue atrophy (speech impairment)
death by respiratory failure
DOES NOT AFFECT EOMS OR PERSONALITY
what is Huntington’s disease
progressive degeneration of basal ganglia leading to amyloid-protein related accumulation
autosomal dominant with enlarged lateral ventricles as early indicator
dx with MRI
fatal within 10-20 years of pneumonia
what is leukodystrophy and the most common type?
progressive disruption of myelin sheaths due to defective myelin production in CNS
manifests early in life and rapidly fatal
Adrenoleukodystrophy: very long FA chain buildup and disrupts myelin sheath
x linked disease and impairment of adrenal function (90%)
What is tay-sach’s disease
autosomal recessive disease and manifests by 6 mos
cherry red spot on macula with white surround
what is neimann-pick disease
autosomal recessive disease + manifests by 1 year old
cherry red spot on macula
Fabry’s disease?
x linked disease by 3-10 years old
whorl keratopathy or vortex no effect on vision
what is an EEG
electroencephalography
- graphic recording of electrical activity of superficial layers of cerebral cortex
where do we do a lumbar puncture?
insertion of spinal needle between L3-L4 into lumbar subarachnoid space to instill air, dye, meds
what is the Blood Brain Barrier and its function?
what is it made up of?
reduce capillary permeability and protects brain from hazardous substances
made up of endothelial tight junctions, thickened basement membrane, and astrocytes
Passive Transport: O2, CO2, lipid solubles
Active Transport: glucose, AA, small ions
what are the clinical manifestations of pathophysiology of CNS
Altered Mental status
Syncope
Localizing Signs
Seizures
What are the causes of AMS
drugs/alchohol, stroke, infection, syncope, seizures
not a diagnostic - just a finding
What is CSF and where is it produced and where does it go? What is its purpose?
surrounds the brain and spinal cord
produced by choroid plexus and fills up subarachnoid space
buoyancy + protection + nutrient delivery/metabolic waste removal
What is ICP and what are the normal ranges?
How is it regulated?
What can increase it?
Intracranial pressure (pressure inside skull) 7 - 15 mmHg or 95 - 200 mmH20
Regulated by CSF: production, circulation, drainage and reabsorption
Caused by brain edema/tumor, CSF obstruction, intracranial hemorrhage, idiopathic intracranial HTN (IIH)
What is IIH and what is it known for? what is the most common cause of this? how do you dx?
symptoms?
Tx?
IIH is idiopathic intracranial HTN (pseudotumor cerebri)
increased ICP idiopathically and decreases veinous drainage
caused by papilledema (OU swelling of optic nerve)
HA + VFD + Overweight (usually)
Tx: decrease weight, oral CAI, Sx, decrease CSF
what is hydrocephalus and what are the types?
Symptoms?
Tx?
abnormal accumulation of CSF (congenital more common)
Normal Pressure, Non-Communicating, Communicating
Asymptomatic to Severe (sunset eyes, bossing forehead, scalp veins, increase head size)
Tx: ventricular shunt to drain areas and decrease ICP
What is meningitis and how is It caused?
inflammation of meninges and caused by viral and bacterial infections
what are the viruses associated with meningitis
enterovirus (GI)
least damaging compared to bacterial meningitis
what is the bacteria associated with meningitis
symptoms?
ocular manifestations?
more severe than viral
S. Pneumoniae
hearing loss + brain damage
Stiff neck, sudden fever, HA, AMS
Pupil changes, CNP, Fundus Changes (papilledema and retinal hemorrhage)
What is encephalitis and what can it lead to?
caused by what?
symptoms?
inflammation of the entire brain usually with concomitant meningitis
inflammation leads to cerebral edema and neural dysfunction and brain damage
infants/elderly are more susceptible
usually idiopathic (60%) but can be viral (enterovirus)
seizures, stupor, coma + permanent brain damage + coma
what are the types of seizures?
Type 1: Partial (focal areas are discharged)
- Simple: limited + patient is aware
- Complex: Automatisms
Type 2: Generalized
- Absence: staring spell, brief, recurrent, patient is unaware
- Tonic Clonic (Grand Mal): dramatic loss of consciousness and convulsions of all extremities, incontinence, amnesia
