Neurology Flashcards
Broca aphasia
Expressive aphasia
Patients understand language but cannot generate language
Effects spoken and written language
Broken speech
Wernicke aphasia
Receptive aphasia
Patients can generate words/sentences for cannot understand what is said to them
Language generated is often incomprehensible
Conduction aphasia
Patient cannot repeat what is said to them
Language comprehension and generation are intact
Global aphasia
Combination of non-fluent speech, poor comprehension, and poor repetition
Basilar artery region supplied
Midbrain and part of the pons
Anterior inferior cerebellar artery (AICA) regions Regions supplied
Parts of the pons and cerebellum
Posterior inferior cerebellar a. (PICA) region supplied, results of stroke
Inferior cerebellum and lateral medulla
Occlusion causes Wallenberg syndrome - lateral medullary stroke
- loss of pain and temperature sensation on the contralateral side of the body
- loss of pain and temperature sensation on the ipsilateral side of the face
- cerebellar defects - ataxia, past-pointing
Lesion to nondominant parietal lobe (usually the right)
Contralateral hemispatial neglect
Lesion to dominant parietal lobe (usually the left)
Gertsmann syndrome - agraphia, acalculia, finger agnosia
Lesion to frontal lobe
Personality changes
lesion to the bilateral amygdalae
Kluver-Bucy syndrome - disinhibition, loss of fear, hyper orality/ hyperphagia, hypersexuality
Lesion to subthalamic nucleus
Hemiballismus
Cranial nerve involved in eyelid opening
CNIII
Cranial nerve involved in head turning
CN XI
Cranial nerve innervate the muscles of mastication
CNV
Cranial nerve for taste from anterior two thirds of tongue
CN VII
Cranial nerve involved in tongue movement
CN XII
Cranial nerves involved in balance
VIII
Cranial nerve monitoring carotid body and carotid sinus chemoreceptors in and baroreceptors
CN IX
Anterior spinal artery
Supplies all except dorsal columns
Dorsal column - medial lemniscus pathway
Carry sensory information - pressure, two point discrimination, vibration, proprioception
Tracks ascend ipsilaterally in the fasciculus gracilis (lower body) and fasciculus cuneatus (upper body)
decussate in the medulla
Ascend as the medial lemniscus and the brain stem to the thalamus -> sensory cortex
Spinothalamic tract
Carry sensory information - pain and temperature
Nerves enter the spinal cord and ascend 1-2 levels in Lissauer’s tract
Decussates in the anterior white commissure
Ascends contralaterally to the thalamus -> sensory cortex
Lateral corticospinal tract
Carries Motor commands for voluntary movements
Signals originate in the motor cortex
decussates in the medullary pyramids
Descends contralaterally and synapses in the anterior horn of the spinal cord -> skeletal muscles
Location of lesion and clinical findings for amyotrophic lateral sclerosis (ALS)
Lesion:
Corticospinal tracts
Anterior horn cells
Findings: Spastic paralysis (UMN) Flaccid paralysis (LMN)
Location of lesion and clinical findings for poliomyelitis
Lesion: anterior horn cells
Findings: flaccid paralysis (LMN)
Location of lesion and clinical findings for syringomyelia
Lesion:
Anterior white commissure - spinothalamic tract
+/- anterior horn cells
Most common cervical and upper thoracic
Findings:
Cape like loss of pain and temperature over shoulders and arms
+/- flaccid paralysis in the arms and hands
Location of lesion and clinical findings for tabes dorsalis
Lesion: dorsal column and dorsal roots
Findings:
Impaired proprioception
Gait/balance problems
Location of lesion and clinical findings for Brown-Sequard syndrome
Lesion: spinal cord hemi section
Findings:
Ipsilateral loss of vibration sense an two point discrimination
Contralateral loss of pain and temperature below lesion
Ipsilateral motor weakness or paralysis
Most common sequelae of meningitis in kids
Hearing loss
Intellectual disability
Seizure disorder
Spastic paralysis
Clinical presentation meningitis
Fever
Headache
Stiff neck (nuchal rigidity)
photophobia
Brudzinski’s sign - spontaneous hip flexion when neck is flexed passively
Kernig’s sign - pain during knee extension while hip is flexed to 90 in supine
Contraindications to lumbar puncture
Relative:
Tendency to bleed
Very low platelets
Increased intracranial pressure - risk herniation
Exam findings to consider CT before LP
Focal neurological deficits New onset seizure AMS papilledema Immunocompromised History of CNS disease
CSF analysis associated with bacterial meningitis
Glucose:
Protein:
WBCs:
Glucose: low
Protein: high
WBCs: very high - neutrophils
CSF analysis associated with viral meningitis
Glucose:
Protein:
WBCs:
Glucose: nl
Protein: nl/slight elevated
WBCs: elevated - lymphocytes 10-500
CSF analysis associated with TB meningitis
Glucose:
Protein:
WBCs:
Glucose: low
Protein: high
WBCs: high - lymphocytes 10-500
CSF analysis associated with Fungal meningitis
Glucose:
Protein:
WBCs:
Glucose: low
Protein: high
WBCs: high - lymphocytes 10-500
Most common cause and treatment of bacterial meningitis in less than one month old
Listeria monocytogenes
Group B streptococci
E. coli
Ampicillin + gentamicin +/- cefotaxime
Most common cause and treatment of bacterial meningitis in 1-3 mo
Group B strep
E. coli
S. pneumo
Neisseria meningitidis
Third-generation cephalosporin - ceftriaxone or cefotaxime
+ vancomycin
Most common cause and treatment of bacterial meningitis in 3m - 50 yo
S. pneumo
Neisseria meningitidis - dorms, barracks (petechial rash)
Unvaccinated - HIB
Third-generation cephalosporin + vancomycin
Most common cause and treatment of bacterial meningitis in over 50 yo
S. pneumo
Neisseria meningitidis
Listeria monocytogenes
Third-generation cephalosporin
+ vancomycin
+ ampicillin
Intrapartum treatment to prevent Group B strep infection to neonate
Ampicillin or penicillin during labor and delivery
Treatment for meningitis in recent neurosurgery
Vancomycin + cefepime or meropenem
Treatment for meningitis with CSF leak
Third-generation cephalosporin + vancomycin
Circumstances to add dexamethasone to treatment for meningitis
Decrease likelihood of neurologic sequelae
Children with HIB meningitis (unimmunized)
S. pneumo meningitis
+/- TB meningitis
Dexamethasone is getting before or with the first dose of antibiotics not started after the antibiotics
-continue 2-4 days
Not given if patient is less than six weeks old
Special precautions in managing the patient with Neisseria meningitidis meningitis
Chocolate isolation Antibiotics prophylaxis two close contacts -cipro -rifampin -ceftriaxone
Viral meningitis (aseptic meningitis)
Clinical presentation:
Mild illness, or severe illness similar to bacterial meningitis
Usually resolve spontaneously without complications
CSF: nl glucose, nl or slightly elevated protein, 10-500 WBCs with lymphocyte predominance
MC viruses:
Enteroviruses
HSV-2
Tx:
Symptomatic - pain control, IV fluids
HSV: acyclovir
Diagnosis and treatment of cryptococcal meningitis
Seen in advanced HIV, gradual onset over 1-2 weeks
Cryptococcal antigen in CSF (old way - India ink)
IV amphotericin B + flucytosine for 2 weeks
Followed by oral fluconazole for at least 8 weeks
Tests and treatment for TB meningitis
Stain culture for AFB (acid-fast bacilli)
PCR
Treatment: Rifampin Isoniazid + B6 Pyrazinamide Ethambutol
Encephalitis - presentation and diagnostic testing
Inflammation of the brain parenchyma
Presentation: Similar to meningitis - nuchal rigidity, headache, photophobia, fever, altered mental status Focal neurological deficits Seizures Behavioral/personality changes
CSF: mildly elevated WBC (lymphocytes), mildly elevated protein, normal glucose
Elevated opening pressure
Viral cultures, PCR, or antibody studies maybe performed on CSF
HSV encephalitis
HSV1 - older patient, latent infection
MRI finding - temporal lobe lesion
HSV PCR on CSF
Tx:
IV acyclovir
West Nile virus
Arbovirus - MC in US
Transmission: amplifying host- bird; vector - mosquitoes
Presentation:
often asx
Flulike illness - headache, malaise, back pain, myalgia, anorexia
Neuroinvasive in 1/150 pts: meningitis, encephalitis, Flaccid paralysis (involvement of anterior horn cells)
Dx: detection of virus or WNV IgM in CSF
Tx: supportive
Varicella-Zoster virus encephalitis
Complication of herpes zoster
Altered mental status days after rash appears
VZV PCR on CSF
IV acyclovir
Rabies
Transmission:
Developing countries - dog bites
US: bats, foxes, skunks, raccoons
Incubation period 1-3 months
Presentation:
Encephalitis: agitation, hydrophobia, pharyngeal spasms, Spasticity of muscles of the head and neck
Progresses to flaccid paralysis, respiratory failure, death
Negri bodies on bx
Prevention of transmission:
-Wash fresh bites with soap and water, apply povidone-iodine
-quarantine/observe animal for 10 days
=ppx if animal developed signs of rabies or if not availabl or if not available for eval
-start ppx immediately for bites on the head or neck (shorter incubation time)
PEP:
Rabies immunoglobulin injected outside of bite and IM
Rabies vaccine - series over 3-4 weeks
100% fatal without tx
Poliomyelitis
enterovirus
Fecal – oral spread or droplet respiratory secretions
Mild or subclinical infection
May invade brain or spinal cord -> destruction motor neurons in anterior horns
Asymmetric muscle weakness, muscle atrophy, or flaccid paralysis
Tx: supportive including mechanical ventilation
Prevention:
Inactivated polio vaccine (Salk) - US
Oral poliovirus vaccine (Sabin) - other parts of the world
Toxoplasmosis
CNS infection by the protozoan Toxoplasma gondii
-infectious oocytes in infected meat or feces from infected cats
Initial infection - asx
Latent infection - reactivate during immunosuppression -> fever, headache, seizures
MRI shows 1+ ring enhancing lesions
Anti-toxoplasma IgG antibodies
Tx:
Sulfadiazine + pyrimethamine
HIV CD4 less than 100 - TMP-SMX ppx
Brain abscesses
Extension of local infection - mastoiditis or sinusitis
Hematogenous spread
Headache, fever, papilledema, seizures
MRI shows ring enhancing lesions
Bx or CT guided aspiration needed
Tx:
Vancomycin + ceftriaxone + metronidazole
Steroids if abscess causing significant mass effect
If recent brain surgery - vancomycin + ceftazidime
Neurocysticercosis
Central/South America
Fecal-oral - Taenia solium eggs
Eggs hatch in small intestine -> intestinal wall invasion -> Brain
New onset seizures
CT or MRI - cysts +/- visible scolex, cysts may calcify
Tx:
Antiepileptics - phenytoin if seizures
Antiparasitics - albendazole
Corticosteroids
Reye syndrome
Rapidly progressive encephalopathy following a viral illness (flu or chickenpox) and aspirin treatment
Vomiting, confusion, elevated LFTs, hypoglycemia
Risk factors and presentation of TIA
Risk factors: Family history Over 55 years old Hypertension Diabetes Coronary artery disease Tobacco use Hyperlipidemia Hypercoagulable state
S/S: Weakness Paresthesias Brief unilateral blindness - amaurosis fugax Other vision abnormalities Impaired coordination Vertigo
Large artery low flow TIA
Due to atherosclerosis - internal carotid artery
Recurrent, short event
Embolic TIA
Often extra cranial - atrial fibrillation
Longer duration, single event
Lacunar TIA
Occlusion of small arteries arising from the middle cerebral, basilar, or vertebral artery
Imaging for TIA
MRI - better to detect infarction CT - faster, better to detect hemorrhage Ultrasound of carotids MRA or CTA - vascular defects ECHO - evaluate for source of embolus
Treatment of TIA
Antiplatelet therapy - clopidogrel and aspirin/dipyridamole prefered; aspirin alone is alternative
Anti-lipid - high intensity Staten therapy in all - atorvastatin 80 mg
BP: treat all with >140/90
Embolic TIA: warfarin + heparin or dibigatran, rivaroxaban, or apixaban
Indications for carotid endartectomy
Symptomatic with narrowing of 70 to 99%
Symptomatic men narrowing of 50 to 69%
Asymptomatic with narrowing of 62 to 99%, with life expectancy more than five years, surgeon with perioperative complication rate less than 3%
Anterior cerebral artery stroke
Contralateral lower extremity and trunk weakness
Middle cerebral artery stroke
Most common
Face and upper extremity weakness, aphasia, neglect, and inability to perform learned actions
Posterior cerebral artery stroke
Visual abnormalities
Basilar artery stroke
Cranial nerve abnormalities - visual abnormalities, vertigo
Occlusion of one of the branches (median and paramedian branches) - contralateral weakness
Complete occlusion - bilateral long track signs, herald hemiparesis -> b/l
AMS or coma
Sensory defects
Loss of coordination
Difficulty speaking
Stroke risk
Advanced age Hypertension History of stroke or TIA Diabetes Hyperlipidemia Smoking Atrial fibrillation
Lacunar infarct - pure motor hemiparesis
Weakness of the face, arm, and leg on one side of body
Absent sensory or cortical signs - a phaser, neglect, apraxia, hemianopsia
Most common - about 50% lacunar strokes
Lacunar infarct - Pure sensory stroke
Sensory defects (numbness) a face, arm, leg on one side Of the body
Absent motor or cortical signs
Lacunar infarct - ataxic hemiparesis
Ipsilateral weakness and limb ataxia out of proportion to motor defect, Possible gait deviation to the affected side
Absent cortical signs
Lacunar infarct - sensorimotor stroke
Weakness and numbness of the face, arm, and leg on one side
Absent cortical signs
Lacunar infarct - dysarthria, clumpsy hand syndrome
Facial weakness, dysarthria, dysphasia, and slight weakness and clumsiness of one hand
Absent sensory or cortical signs
Least common
Imaging for strokes
CT - faster, better to detect hemorrhage - blood bright white
MRI -better to detect infarction
Ultrasound of carotids
Echocardiogram - check source of embolus
MRA or CT angio - identify artery occluded
If hemorrhagic - coagulation studies
Treatment of ischemic strokes
Thrombolytic therapy - Alteplase first line
-within 3 to 4.5 hours
Antiplatelet therapy
- acute - aspirin ASAP unless received thrombolytics, delay 24 hours
- Long term - clopidogrel or ASA/dipyridamole (aspirin alone if can’t tolerate)
Atorvastatin 80 mg in all
Permissive htn 220/120, treat if over or if CAD (labetalol, nicardipine)
Heparin or LWMH - if afib, mural thormbosis
- prevent further emboli
- progressive thromboembolism
- higher mortality otherwise
Contraindication for thrombolytic therapy
Hemorrhage on CT
Recent surgery/ hemorrhages
On anticoagulation
BP > 185/110
Hemorrhagic stroke treatment
ABCs to stabilize
Stop anticoagulants - vit K and FFP if warfarin
Keep SBP below 220, below 180 if signs of increased ICP
Control intracranial pressure -elevate head of bed 30° -analgesia and sedation -mannitol -hyperventilation to PaCO2 25-30 (temporary) Surgical decompression
Causes of seizures in infants, children, adults, elderly
Infants: hypoxic injury, metabolic defects, genetic or congenital abnormalities such as PKU, infection
Children: idiopathic, infection, fever, trauma
Adults 18-35: trauma, alcohol withdrawal, brain tumor
Adults - general: idiopathic, metabolic defects (hyponatremia, hypoglycemia), drugs, trauma, neoplasm, infection, cerebrovascular disease
-toxoplasmosis, neurocysticercosis, HSV
-hemorrhagic stroke
Elderly: same as adults but higher percentage of CVA and metabolic defects
Medications or medication withdrawals known to cause seizures
Theophylline - neuro/cardiotoxicity
Bupropion (SSRIs, TCAs)
Narcotics - fentanyl, meperidine, propoxyphene
Psychoactive stimulants - amphetamines, cocaine methylphenidate
Phencyclidine (PCP)
Neuroleptics - clozapine, phenothiazines
Abx: isoniazid (ppx against by adding B6), PCNs, metronidazole
alcohol toxicity/withdrawal
Todd paralysis
Unilateral weakness/paresthesias after generalized seizure
Treatment of status epilepticus
ABC's IV/IM benzo - diazepam, lorazepam Phenobarbital Intubate Phenytoin or fosphenytoin to prevent recurrence
First-line treatment of eclampsia
magnesium-sulfate
+/- benzo: diazepam, lorazepam
C-section after stop a seizure
Cs of Huntington disease and treatment
CAG repeat disorder on chromosome 4 (huntingtin gene)
ACh and GABA decreased
Choreiform movements
Cognitive decline (dementia)
Caudate nucleus (basal ganglia) atrophy on MRI
40 yo average onset
Tx:
Dopamine antagonists - treat choreiform - tetrabenazine
Antipsychotics - risperidol, olanzapine
Presentation of multiple sclerosis
Focal inflammatory demyelinating lesions affecting the brain and spinal cord
MC - women 20-40
Lesions separated in and both space and time
- slowly progressive, with remissions and relapses
- symptoms exacerbated by overheating
Sensory deficits
Paresthesias
-Lhermitte’s sign - neck flexion causes electric shock like tingling radiating down the back and into the extremities
Motor weakness - limbs, face, diplopia, ptosis
Bowel or bladder dysfunction - urgency, incontinence, retention
Unilateral optic neuritis
Internuclear ophthalmoplegia
Unilateral optic neuritis
Acute eye pain worse with eye movement
Central vision loss
Afferent pupillary defect (Marcus Gunn Pupil)
- no constriction of either pupil when light is shone into affected eye
- both pupils constrict when light is shone into the unaffected eye
Internuclear ophthalmoplegia
Damage to the media longitudinal fasciculus (MLF)
Ipsilateral I cannot adduct on lateral gaze - stare at side of lesion
Horizontal nystagmus in the contralateral eye during lateral gaze
Normal convergence
Evaluation and treatment of multiple sclerosis
CSF:
elevated protein
mild elevated WBC count
Oligoclonal bands - IgG antibodies seen on gel electrophoresis
MRI of brain and orbits and MRI of the spinal cord - demyelinated White matter lesions of various ages
Tx:
Acute attacks: high-dose glucocorticoids (methylprednisolone 500-1000 mg qD x5 days)
Maintenance therapy:
Interferon beta - decreases the frequency of exacerbations
Glatiramer
2/3rd line: Natalizumab - increased risk for adverse side effects
Dimethyl fumerate
Teriflunomide
Syringomyelia
Cystic degeneration of the spinal cord -> expansion of the cavity compresses adjacent neural tissue
-develops months to years after cervical spinal injury
Features:
Compression of crossing fibers of the spinothalamic tract in the anterior white commissure -> loss of pain and temperature sensation 1-3 segments below the syrinx (arms)
Compression of LMNs in anterior horns -> UE weakness, flaccid paralysis, atrophy of hand muscles
Decreased DTRs
Muscle fasciculations
Tx: surgical decompression and shunting
Causes of dementia
Alzheimer's disease Dementia with Lewy bodies Vascular dementia Frontotemporal dementia (Pick Dz) Parkinson disease Huntington disease Normal pressure hydrocephalus Vit B12 deficiency Thiamine deficiency (Wernicke-Korsakoff syndrome) Niacin deficiency (pelagra) Hypothyroidism Wilson's disease Brain tumors HIV infection Creutzfeldt-Jakob disease Neurosyphillis
Pseudodementia
Day to day memory loss
Realize they have a problem (True dementia don’t know)
Causes: depression MC, others
Differentiate Alzheimers from vascular dementia
CT or MRI to distinguish - old ischemic damage in vascular dementia
Dementia workup
CMP CBC UA Vit B12 TSH PRP HIV test non cont CT or MRI - infarct, hydrocephalus
Alzheimer’s disease
Dementia characterized by amyloid plaques, neurofibrillary tingles, and neuronal atrophy
Risk factors:
Advancing age
Family history
Downs syndrome - Amyloid precursor protein (APP) gene on Chr 21 -> more amyloid plaques
Clinical features:
Progressive short-term memory loss
Loss of executive function -> difficulty completing complex tasks
Disinhibition, personality changes, and delusions possible
Dx of exclusion
MRI the brain shows cortical atrophy - nonspecific, not diagnostic
Tx: Cholinesterase inhibitors: -Donepezil -Galantamine -Rivastigmine Memantine - NMDA receptor blocker
Unique clinical features of dementia with Lewy bodies
Dementia
Parkinsonian features - bradykinesia, tremor, cogwheel rigidity, fenestrating gait
Visual hallucinations
Episodes of syncope - repeated falls
Unique clinical features of frontotemporal dementia (Pick disease)
Dementia plus inappropriate social behavior - personality change
Dementia plus Progressive aphasia - naming, comprehension, and expressing
Delirium causes
Drugs: Benzodiazepines Anticholinergics Antihistamines Glucocorticoids - agitation and psychosis Alcohol and drugs of abuse UTI - hospital and elderly CNS insults - stroke, encephalitis Hypoxia - especially in PE (tachycardia, tachypnea)
Workup and management of delirium
Dx: Electrolytes Urinalysis Pulse oximetry Thorough review of medications CT of brain if H&P or testing does not reveal dx
Tx:
Treat underlying cause
Environmental changes - reorient the patient, turn on lights, open the blinds, sitter
Avoid restraints if possible
Antipsychotics - haloperidol - prn for acute agitation
Avoid benzodiazepines and anticholinergics
Work up for syncope
Rule out orthostatic hypotension via till test:
-+ if SBP drops >20, DBP drops >10, HR increases >20
Rule out seizure by history and physical
CBC, electrolytes, BUN/Cr, glucose
Assessed value status
Pulse oximetry - r/o hypoxia
ECG - r/o arrhythmias
Review medications
Serial cardiac enzymes and ECGs r/o MI
Echocardiogram - exertional syncope, murmur, HOCM, AR/AS
Cardiac stress test if older pt
Bilateral carotid duplex
24 hour Holter monitor or event monitor
CT head without contrast and EEG
Causes of coma
AEIOU TIPS
Alcohol Epilepsy and environment (hypothermia) Insulin - hypo/hyper Overdose or opioids Uremia Trauma Infection Psychogenic Stroke
Pupils large and nonreactive in coma patient
CN III involvement below the mid brain, supratentorial Mass effect -> uncal herniation
Pupils small and reactive in coma patient
Thalamic involvement, possible tentorial herniation
Pupils pinpoint in coma patient
Opioid overdose, organophosphate poisoning
Ocular motility in coma patient - immobile but reactive
Metabolic cause, benzodiazepine overdose
Ocular motility in coma patient - oculovestibular reflex
Conjugate deviation towards icewater, nystagmus in opposite direction of ice water - intact brainstem
No deviation of eyes - CN III or VI involvement, suggests brainstem damage
Deviation but no nystagmus - cerebral hemisphere damage or suppression
Ocular motility in coma patient - Oculocephalic maneuver (Doll’s eye test)
Present - fixed on particular point in room: intact brainstem
Reflex absent (like eyes painted on) - Brain death
Spastic paralysis in a coma patient
UMN lesion or high spinal cord injury
Decorticate posturing in a coma patient
Elbows flexed, Legs extended
Cortical lesion or lesion below cortices
Decerebrate posturing in a coma patient
Elbows and legs extended
Midbrain involvement
Responses to pain in a coma patient
No response - Pontine-medullary involvement, reticular activating system damage
Appropriate responses - superficial cause
Nightmares versus night terrors
Nightmares: during REM - Little to no muscle tone
Night terrors: during N3 - appear to wake up, and immediately fall back asleep
Narcolepsy
Excessive daytime sleepiness
When initiating sleep, straight to REM
Hallucinations and cataplexy
Dx: polysomnography - short sleep latency
Tx:
Regular sleep schedule - nap
Improve daytime sleepiness: modafinil, armodafinil, ampthetamines
Reduce REM: venlafaxine, fluoxetine, atomoxetine
Severe sx: sodium oxybate
Restless leg syndrome (RLS)
Unpleasant paresthesias -> voluntary, spontaneous, continuous movements that temporarily relieve the sensations
-creepy crawling feeling
Secondary RLS - Iron deficiency, ESRD, diabetic neuropathy, Parkinson disease, pregnancy, RA, varicose veins, caffeine
Tx: pramipexole ropinirole gabapentin pregabalin carbidopa-levodopa increase exercise decrease caffeine
Constructive sleep apnea
Repetitive collapse of the upper airway during sleep
Risk factors: older age, obesity, male
Presentation: Excessive daytime sleepiness, snoring, choking or gasping a night
Dx: polysomnography
Tx:
Weight loss
Avoid alcohol, hypnotic medicines
CPAP
Oral appliances
Uvulopalatopharyngoplasty - if refractory
Modafinil, armodafinil, amphetamines for chronic daytime fatigue
Primary CNS neoplasms presentation
Headache that worsens over weeks to months Neuro deficits Vision changes Seizures Altered mental status
Adult brain tumors
Most supratentorial
“MGM Studios”
Metastasis
Glioblastoma
Meningioma
Schwannoma
Glioblastoma
Astrocytoma
MC primary brain tumor in adults
Very Poor prognosis - 6 mo - 1 yr survival
Located in cerebral hemisphere
Irregular masses with necrotic center surrounded by edema
Tx: resection, chemo, radiation
Meningioma
Arachnoid cells
2nd MC primary brain tumor in adults
MC parasagital area
Women>Men
Slow growing tumor with good prognosis
Often asx
Located near the surfaces of brain
Tx: resection
Schwannoma
Benign
Localized to CN VIII - acoustic neuroma
Presentation: tinnitus and hearing loss
B/L - neurofibromatosis type II
Treatment: resection
Neurofibromatosis type II
B/L Schwannomas - acoustic neuromas
Intracranial meningiomas and spinal tumors
S/S: Hearing loss Tinnitus Unsteady gait Dizziness Rare trigeminal nerve/facial nerve involvement - face numbness or paralysis
Dx: MRI or CT
Tx: surgery, radiation
Observation - if small, slow growing
Pediatric brain tumors
Infratentorial
“Animal kingdom, Magic kingdom, Epcot”
Astrocytoma
Medulloblastoma
Ependymoma
Pilocytic astrocytoma
MC primary brain tumor in kids
posterior fossa
Slow-growing with good prognosis
Tx: resection
Medulloblastoma
2nd MC Brain tumor and kids
Cerebellar tumor - malignant
Presentation: headache and ataxic gait
Compression of 4th ventricle -> non-communicating hydrocephalus
Tx: resection, chemo, radiation
Ependymoma
3rd MC Brain tumor and kids - rare
Compression of 4th ventricle
Tx: resection and radiation
Metastatic CNS neoplasms
“Lots of Bad Stuff Kills Gila”
Long Breast Skin (melanoma) Kidney (renal cell carcinoma) G.I. tract (colon cancer_
Dx: pan CT - head, neck, chest, abdomen, pelvis
Bone scan
Neurofibromatosis type 1
MC
“Von Recklinghausen disease”
NF1 gene - Chr 17
Presentation:
Café au lait spots
Axillary and/or inguinal freckling
Lisch nodules - hamartomas of iris
Neurofibromas - benign peripheral nerve sheath tumors, cutaneous, risk of malignancy
Optic pathway gliomas - vision loss, color vision problems, abnormal pupillary function, proptosis
Tx: supportive
Myasthenia Gravis
autoimmune disorder - Ab against AChR
Assoc with: thymoma or neoplasms of thymus
Presentation: Muscle weakness that worsens throughout the day Ptosis, diplopia Dysarthria, dysphasia dyspnea - severe d/t diaphragm weakness
Dx: AChR Ab Edrophonium (Tensilon) test AChE-I EMG After dx: CT chest - evaluate thymoma
Tx: AChE-I - neostigmine, pyridostigmine Thymectomy Immunosuppressants (prednisone, azothioprine) Plasmapheresis IVIG if refractory
Lambert-Eaton Syndrome
Autoimmune - Ab against presynaptic voltage gated calcium channels - inhibit ACh neurotransmission
Assoc with small cell lung cancer - paraneoplastic Sn
Muscle weakness improves with use
Tx: Treat underlying causes AChE-I - pyridostigmine immunosuppressants Plasmapheresis
Guillian-Barre Sn
Acute inflammatory demyelinating polyradiculopathy
Preceded by infection - campylobacter jejuni, HIV, CMV, EBV, mycoplasma, recent immunization
Presentation:
Symmetric muscle weakness that begins in the distal legs and ascends
Respiratory paralysis
Facial muscle weakness - B/l bell’s palsy
Autonomic dysfunction - tachycardia
Absent or depressed DTRs
Minimal sensory deficits - may have paresthesias
Dx:
CSF: albuminocytologic dissociation - elevated protein, normal WBC, normal opening pressure
Nerve conduction studies and EMG - demylination
Tx: Hospitalize - respiratory failure Plasmapheresis IVIG No glucocorticoids
Prognosis:
Spontaneous regression by one year
5-10% prolonged disease
5% die
Bell’s palsy
Involving entire facial nerve resulting in facial muscle paralysis - upper and lower
Cause: reactivation of HSV, VZV, Lyme dz (b/l)
Onsets over 1-2 days
Progressive weakness for 3 wks
Recovery within 6 mo
Tx:
Eye care: prevent corneal trauma - hourly tears, patch at night
Glucocorticoids within 3 d of onset of sxs
Valacyclovir for severe diseases
motor cortex stroke vs bell’s palsy
Lesion to motor cortex - lower face, u/l paralysis
CN VII lesion - U/l - upper and lower face
essential tremor
Fixed oscillation of hands or head
Tx:
alcohol - suppresses tremor
1st line: B-blockers - propranolol
Benzos - alprazolam, clonazepam - risk for abuse
Primidone
Refractory: thalamotmy, deep brain stimulation
Chorea
Dance like movements
Assoc with: hyperthyroidism, Huntington’s disease, lupus, levodopa, rheumatic fever
Treat underlying causes
Athetosis
Snakelike movements
Assoc with: cerebral palsy, encephalopathy, Huntington disease, Wilson’s disease
Treat underlying disease
Dystonia
Sustain contractions of proximal limb and trunk
Assoc with: neuroleptic drugs, Wilson’s disease, Parkinson’s disease, cerebral palsy, Huntington disease, encephalitis
Tx: levodopa, carbidopa, Botox
Stop neuroleptic
Hemiballismus
flinging movements of extemities
Assoc with stroke of subthalamic nucleus
Tx: haloperidol
Tics
Involuntary movements like blinking, grimacing, grunting, sniffing, throat clearing
Assoc with: tourette’s syndrome, OCD, ADHD
Tx:
fluphenazine
pimozide
tetrabenazine
Complex regional pain syndrome
Features:
Regional pain (distal extemity) not associated with a specific neurological lesion or dermatomal distribution
-deep burning, stinging pain
May include sensory disturbances, heat or cold sensitivity, Motor impairment
May include skin/ muscle atrophy, joint contraction, edema, nail changes, bone resorption
Begins following injury or trauma, surgery, MI or stroke
Dx:
CT and MRI r/o other causes
Bone scintigraphy - bone lesions and demineralization
Tx:
PT/OT
pain: NSAIDs, TCAs (amiltryptyline) and/or gabapentin
Bisphosphonates or calcitonin to prevent bone resorption
Eye innervation
CN II - vision
CN III - EOM - except Superior oblique and Lateral rectus; levator palpebrae muscle (raise eyelid)
-parasympathetic Control of ciliary muscles and pupillary spincter
CN IV - superior oblique m. - depresses the eye when adducted
CN VI - lateral rectus - abducts eye
Light reflexes when optic nerve damage distal to pretectal nucleus
Afferent defect
No construction of either pupil when light shone on side of lesion
Both pupils construct when light shone on normal side
Light reflexes when oculomotor nerve (CNIII) damaged
efferent defect
Affected side will not respond to light shone in either eye
Opposite pupil will constrict one light shown in either eye
Horner syndrome
Ptosis
miosis
anhydrosis
Associated with Pancoast tumor - apex of lung
Argyll robinson pupils
pupils accomondate but don’t react
Assoc with neurosyphilis
Review visual field defects
page 263
Most common causes of blindness by age group
over 55 - macular degeneration
Under 55 - DM
Blacks any age - glaucoma
Astigmatism
Warped or asymmetrical cornea -> blurred vision at all distances
Tx: corrective lenses
myopia
Axial length of eye too long for the refractive power of the cornea and lens -> blurred distance vision (“nearsightedness”)
Tx: corrective lenses or laser surgery
hyperopia
axial length of the eye is too short for the refractive power of the cornea and lens -> blurred near vision (“farsightedness”)
Tx:
Corrective lenses or laser surgery
Amblyopia
Severe reflective error or eye misalignment -> developmental defect in neural pathway -> unilateral vision loss
Tx:
Vision training - patch good eye
MC cause is strabismus
Strabismus
Misalignment of the eyes -> diplopia, may result in amblyopia
Tx:
Vision training - patching
Surgical correction
Cataracts
opacification of lens due to build up of epithelial cells
Risk factors: Advanced age, diabetes, glucocorticoid use, tobacco/ alcohol use
Presentation:
Usually bilateral but not symmetrical
Painless progressive decrease in vision manifested by difficulty driving at night, Reading roadsigns, Reading FinePrint
Possible debilitating glare in bright sunlight or from oncoming headlights (MC with steroid induced)
Nearsightedness - early manifestation
Tx:
Surgical removal of the opacified lens and replacement with synthetic lens
Age-related macular degeneration (ARMD)
Degeneration of the macula -> loss of central vision
Presentation:
Dry (atrophic) ARMD: Cellular debris (drusen - pale yellow dots) accumulate on the retina -> gradual loss of vision
Wet (exudative) ARMD: neovascularization (leaky new vessels) -> hemorrhage -> sudden vision loss, more rapid progression
Dx:
Slit lamp - dry
Fluorescein angiography - wet
Tx:
Smoking cessation
Antioxidant supplements - beta-carotene, vitamin C, lutein, selenium, zinc
Wet: injections of anti-VEGF drugs (ranibizumab) into the vitreous humor
Retinal detachment
Presentation:
Painless and vision loss
Flashing lights and floaters -> curtain pulled over the eye
Exam: wrinkled, pale area of detached retina “billowy cloud”
Tx:
Laser photocoagulation
Cryotherapy
Surgical reattachment
Central retinal artery occlusion (CRAO)
Risk factors: Atherosclerosis Hypertension Diabetes Atrial fibrillation Valvular heart disease
Presentation:
Acute, painless vision loss - more profound than retinal detachment
Exam: pale retina with Cherry red spot
Tx: Thrombolytics Acetazolamide or mannitol to reduce IOP Anterior chamber paracentesis - removal of aqueous humor Hyperbaric oxygen
Central retinal vein occlusion
Presentation:
Gradual, painless vision loss
Exam: retinal edema, flame hemorrhages, venous dilation, cotton wool spots
Tx:
VEGF inhibitors
Laser photocoagulation
Cherry red spot ddx
central retinal artery occlusion
Niemann Pick dz
Taysachs
Other lysosomal storage diseases
B/L bells palsy ddx
Guillian Barre Sn
Lyme Dz
Acute angle closure glaucoma
lens pushes anteriorly against the iris -> blocking flow of aqueous humor through the pupil -> increased intraocular pressure
Presentation:
sudden onset, u/l eye pain
+/- frontal headache, N/V
Blurred vision and color halos encircle light sources
Affected eye: red and tearing, hazy cornea, pupil fixed and mid-dilated
Eye feels rock hard
Dx:
tonometry shows elevated IOP
Gonioscopy - measure iridocorneal angle
Do not dilate pupil
Tx:
Immediate referral to ophtho -> laser peripheral iridotomy
Initial meds: Pressure lowering eye regime: -1 drop 0.5% timolol -1 drop 1% apraclonidine -1 drop 2% pilocarpine Acetazolamide 250 mg two tabs at once
If refractory -> IV mannitol once dx confirmed
Open-angle glaucoma
Impermeability of the trabecular meshwork -> reduce resorption of aqueous humor -> gradual increase in IOP
Features: Develops insidiously Need new glasses every few months Usually bilateral Gradual loss of peripheral vision -> permanent blindness
Dx:
Tonometry shows a elevated IOP (air puff)
Cup to disc ration >50%
Tx:
Prostaglandins - latanoprost - increased outflow aqueous humor
B-blockers - timolol - inhibit aqueous humor production
alpha 2 - adrenergic agonist - apraclonidine, brinomidine - reduce aqueous humor production and increase outflow
Cholinergic agonists - pilocarpine - cause miosis and open trabecular meshwork -> increased outflow of aqueous humor
Topical acetazolamide- reduce aqueous humor production
Surgery: laser trabeculoplasty
Orbital vs periorbital cellulitis
Orbital cellulitis involves contents of the orbit - fat, EOMs
Both orbital and periorbital cellulitis - eye redness, swelling, pain
Orbital cellulitis presentation: Proptosis Pain with eye movement Ophthalmoplegioa - weak EOM -> diplopia rare vision impairment
Complications orbital cellulitis -> permanent vision loss, 1-2% mortality
Chalazion
Inflammation of an internal Meibomian sebaceous glands - eyelid swelling
Tx: self-limited
Can be treated with surgical excision and/or intralesional steroid injection
hordeolum
Infection of the external sebaceous glands of Zeiss or Mol
Tender, red swelling at the lid margin
tx:
Warm compress 3-4 Times a day for 10-15 min
If unresolved in 48 hours - abx ointment q3 hr
+/- I&D
Anterior blepharitis
Infection of the eyelids and lashes
Secondary to seborrhea
Red, swollen lid margins + dandruff on lashes
Tx:
Wash lid margins daily with shampoo
Remove scales daily with cotton ball
Abx ointment to lid margins
Viral conjunctivitis
MC - adenovirus
Watery discharge -eyelid may be sealed in AM
Highly contagious
+/- fever, URI, LAD, pharyngitis, diarrhea
Tx: supportive
Bacterial conjunctivitis
S. aureus
S. pneumo
N. gonorrhoeae
C. trachomatis
purulent, copious discharge, 24 hrs/day
Tx: erythromycin ointment or eye drops
Allergic conjunctivits
Pollen, pet dander
Bilateral watery discharge
eyelid may be sealed in AM
pruritis, other allergy sxs
Tx: topical or systemic antihistamines
HSV keratitis
HSV infection of the cornea - branching pattern
-> corneal ulceration
Treatment for a corneal abrasion
thorough eye exam with removal of foreign body by irrigation
Topical abx QID x 3-5 days or until sxs free for 24 hours
-erythromycin, sulfacetamide, ciprofloxacin, ofloxacin
OTC lubricant
Patch less than 24 hrs
Pain: systemic opioids or ophthalmic NSAIDs - diclofenac, ketorolac
NEVER topical anesthetic or steroids
24 hr f/u for: contact lens abrasion, abrasion >= 3 mm, or abrasion with diminished vision
Anterior Uveitis
Causes:
Assoc with HLA-B27 seronegative sponyloarthropathies
juvenile idiopathic arthritis
sarcoidoisis
presentation:
Pain and redness of iris, may extend to conjunctiva
+/- photophobia
Tx systemic inflammatory dz with topical or systemic glucocorticoids
Posterior uveitis
Causes: HSV CMV Toxoplasma gondii Bartonella spp. Treponema pallidum
Presentation: paniless, mild vision abnormalities
treat infection with topical antibiotics
Vitamin A deficiency
Night blindness or complete blindness
Xerophthalmia
Bitot spots - areas of abnormal squamous cell proliferation and keratinization of the conjunctiva (white spot on top of vessels)
Acute otitis media
6-18 mo
S. pneumo, H flu, Moraxella catarrhalis, RSV, rhinovirus, adenovirus, influenza
Dx: bulging TM, purulent effusion
Tx:
Abx for under 2 yo
Observation over 2 yo with mild sxs
First line: high dose amox (90 mg/kg)
Alternatives: azithromycin, cefdinir
recurrent - tubes - more than 3 in 6 mo
Complications:
Hearing loss
Bullous myringitis - painful bulla on TM
Acute mastoiditis - erythema, swelling and pain behind external ear - confirm with CT
MC bacterial causes of otitis externa
Pseudomonas
S. epidermidis
S. aureas
fungal
Otitis externa
Ear pain worse with manipulation Pruritis Hearing loss Drainage/discharge External canal maybe swollen or erythematous
Dx: clinical
Tx:
Clean ear canal - water irrigation
Topical Abx: ofloxacin, ciprofloxacin, polymyxin B/neomycin
Topical glucocorticoids
Benign paroxysmal positional vertigo
Brief lesson one minute episodes of room spinning provoked by head movement, nystagmus
Caused by otoliths in inner ear
Dx: Dix-Hallpike maneuver
Tx: Epley maneuver
R/o CNS pathology, schwannoma
Vestibular neuritis
+ hearing loss: Labrynthritis
Episodes last days - weeks
Viral or post viral inflammatory disorder of vestibular portion of CN VIII
Exam:
Horizontal nystagmus that can be suppressed with visual fixation
Fast phase away from the affected side
Head thrust: rapid head turn patient is unable to maintain Visual fixation
Tx:
Corticosteroid taper
antiemetics, antihistamines, anticholinergics
Meniere disease
Excessive fluid in inner ear
Vertigo lasting at least 20 minutes
Low frequency hearing loss
tinnitis
Tx:
Limit salt, caffeine, nicotine, alcohol
Diuretics - HCTZ
Destructive therapy - gentamicin injected into ear
last resport - labrynthrectomy or vestibulectomy
Weber test
Normal -> midline (both ears)
Conductive hearing loss -> lateralizes to the side of the affected ear
Sensorineural hearing loss -> lateralizes to side opposite affected ear
Rinne test
Normal -> air conduction > bone conduction
Conductive hearing loss -> bone conduction > air conduction
Cholesteatoma
Overgrowth of desquamated keratin debris in middle ear -> erode ossicles
Causes:
Negative middle ear pressure - Eustachian tube dysfunction
Direct growth of epithelium through a TM perforation
Assoc with chornic middle ear infection
Grayish-white “pearly” lesion behind or involving TM
Conductive hearing loss
vertigo
Tx:
Surgical removal - tympanomastoidectomy and reconstruction of ossicular chain
Ramsay Hunt Syndrome
herpes zoster oticus
Reactivation of VZV in ear - latent in geniculate ganglion
S/S: Ipsilateral facial paralysis Ear pain Vesicles in auditory canal/auricle Abnormal taste perception Tinnitis vertigo Abnormal lacrimation
Tx: narcotic analgesia
Oral steroids
Antivirals - Val/acyclovir, famciclovir
