Neurology Flashcards
Diabetic CN III Neuropathy
- Ischemic deficit (not compression) affecting somatic fibers only.
- CN III has both somatic and parasympathetic fibers which have different blood supplies.
Somatic fibers: innervates superior, inferior, and medial rectus, inferior oblique, and levator palpebrae
Parasympathetic fibers: innervates the sphincter of the iris and ciliary muscle —–> fixed, dilated pupil and loss of accomodation
Symptoms: “down and out” gaze, ptosis (unopposed action of the lateral rectus and superior oblique)
- Intact pupillary response and accommodation.
- Compression neuropathy affects all functions.
Todd’s palsy
preceded by a focal motor seizure
Carotid artery thrombosis
- Most commonly affects the middle cerebral a. —> contralateral hemiparesis and hemianesthesia
Lacunar stroke - pure motor hemiparesis
- Lacunar infarction in the posterior limb of the internal capsule
Presentation: unilateral motor deficit (face, arm, and to a lesser extent leg), mild dysarthria
- No loss of sensory, visual, or higher cortical function
Lacunar stroke - pure sensory stroke
- Stroke in the ventroposterolateral nucleus of the thalamus.
Presentation: unilateral numbness, parasthesias and hemisensory deficit involving the face, arm, trunk, and leg
Lacunar stroke - ataxic hemiparesis
Lacunar infarction of the anterior limb of the internal capsule
Presentation: weakness that is more prominent in the lower extremity, along with ipsilateral arm and leg incoordination
Lacunar stroke - dysarthria-clumsy hand syndrome
Lacunar stroke at the basis of the pons
Presentation: hand weakness, mild motor aphasia
- NO sensory abnormalities
Migraine treatment and prophylaxis
Antiemetics: prochlorperazine, chlorpromazine, and metaclopramide
- NSAIDS or triptans if started early into the headache.
Prophylaxis: amitriptyline, propranolol
Basal ganglia hemorrhage
Symptoms: contralateral hemiparesis and hemisensory loss, homonomyous hemianopsia, gaze palsy
Cerebellar hemorrhage
- Usually NO hemiparesis
- Facial weakness, ataxia, nystagmus, occipital headache, and neck stiffness
Thalamic hemorrhage
Symptoms: contralateral hemiparesis and hemisensory loss, non-reactive miotic pupils, upgaze palsy, eyes deviate towards hemiparesis
Cerebral lobe hemorrhage
Symptoms: Contralateral hemiparesis (frontal lobe), contralateral hemisensory loss (parietal lobe), homonymous hemianopsia (occipital lobe), eyes deviate away from hemiparesis, high incidence of seizures
Pons hemorrhage
Symptoms: deep coma and total paralysis within minutes, pinpoint reactive pupils
Essential tremor
- Bilateral action tremor of the hands, usually without leg involvment
- Possible isolated head tremor without dystonia(involuntary repetitive movement)
- Relieved with alcohol
- no other neurologic defecits.
Treatment: propranolol; second-line: primidone and topiramate
Parkinson’s disease tremor
- resting tremor (4-6Hz) that decreases with voluntary movement
- Usually involves legs and hands
- facial involvement less common
- May progress to involve other areas of the body
Tetrad: resting tremor, rigidity, postural instability, and bradykinesia
- Tremor worsens when performing mental tasks and disappears with movement
- Due to progressive loss of dopaminergic neurons in the basal ganglia.
Cerebellar tremor
- Usually associated with ataxia, dysmetria (lack of accuracy during voluntary movement), or gait disorder
- Tremor increases steadily as hand reaches its target
-Can see postural, action, or intention tremors
Physiologic
- Low amplitude (10-12Hz), not visible under normal conditions
- acute onset with sympathetic activity (drugs, hyperthyroidism, caffeine)
- Usually worse with movement and can involve the face and extremities
Brain death
- Absent cortical and brainstem function
- Spinal cord may still be functioning, so DTR can still be present.
- HR becomes invariant due to loss of vagal control.
Status epilepticus
- single seizure lasting longer than 30 mins
- Brains that have seized for >5 minutes are at risk of permanent injury due to excitatory cytotoxicity —-> cortical laminar necrosis
Neurofibromatosis type 2
- mutation in tumor suppressor gene on chromosome 22
- Subcutaneous neurofibromas, cafe-au-lait spots, deafness due to acoustic neuromas
severe variant - Wishart - framshift or nonsense mutations
milder variant - Gardner - missense or splice-site mutation
Primary sclerosing cholangitis
Clinical features: fatigue, pruritis
- 90% of pts have underlying IBS, mainly ulcerative colitis
Labs: Aminotransferases
Ascited Fluid Characteristics
Neutrophils: 250 peritonitis (secondary or spontaneous)
Total protein: >or= 2.5 (high-protein ascites) - CHF, constrictive pericarditis, peritoneal carcinomatosis, TB, Budd-Chiari syndrome, fungal
or=1.1 (indicates portal hypertension) - cardiac ascites, cirrhosis, Budd-Chiari syndrome
Blood supply of the brain
Common carotids —> internal carotids —> opthalmic artery and then anterior and middle cerebral arteries
Subclavian arteries —> vertebral a. —> posterior inferior cerebellar a., then anterior spinal a., then basilar a. —> anterior inferior cerebellar a., then pontine a. —> superior cerebellar a., then posterior cerebral a. which connect to circle of willis vis posterior communicating a.
Middle cerebral artery supply
lateral surface of the frontal, parietal, and upper temporal lobes, genu and posterior limb of internal capsule, majority of the basal ganglia, proximal parts of the visual radiations as they emerge from the lateral geniculate nucleus of the thalamus and course in Meyer’s loop
Anterior cerebral artery supply
medial surface of the frontal and parietal lobes (motor and sensory areas for pelvis and lower limbs), superior inch of the frontal and parietal lobes, anterior 4/5 of corpus callosum, anterior limb of internal capsule
Posterior cerebral artery supply
inferior temporal lobe and occipital pole
Anterior cerebral artery occlusion deficits
spastic paresis and anesthesia of contralateral lower limb
Note: if bilateral, urinary incontinence can occur
- if anterior corpus callosum is involved, a transcortical apraxia of the left limbs may occur (disconnection of left hemisphere’s (language dominant) from motor cortex of right hemisphere)
Apraxia
Inability to perform particular purposive actions
Middle cerebral artery occlusion deficits
- spastic paresis of the contralateral lower face and upper limb and anesthesia of the contralateral face and upper limb
- Aphasia can occur when left MCA is affected and left-sided neglect can occur when right MCA is affected
- Contralateral superior quadrantanopsia w/ occlusion of the branches supplying Meyer’s loop.
Vertebral a. course
through transverse processes of upper 6 cervical vertebrae; enters posterior fossa after passing through foramen magnum
Anterior spinal a. supply
ventrolateral 2/3 of cervical spinal cord and ventromedial part of medulla
PICA supply
cerebellum and dorsolateral medulla
Labyrinthine a. supply
branch of the basilar a. that follows the course of CN VIII; supplies the inner ear
AICA supply
part of pons, anterior and inferior cerebellum
Superior cerebellar a. supply
rostral pons and superior cerebellum
Pontine branches supply
pons via paramedian and circumferential branches
PCA supply
midbrain
Midbrain CNs
III and IV
Upper pons CNs
V
Lower pons CNs
VI, VII, VIII
Upper medulla CNs
IX, X, XII
Medial medullary syndrome
Occlusion of the vertebral a. or anterior spinal a.
Localizing sign
- CN XII - ipsilateral paralysis of half the tongue w/ atrophy (tongue deviates towards the lesion)
Affected nerve tracts
- medial lemniscus - contralateral deficit of proprioception and touch, pressure, and vibratory sensation in limbs and body
- pyramid (corticospinal tract) - contralateral spastic hemiparesis of both limbs
Lateral medullary syndrome (Wallenberg)
Occlusion of PICA
Localizing CNs
- vestibular or cochlear parts of CN VIII - may produce nystagmus, vertigo, N/V (w/ vestibular nystagmus, the fast component will be away from the side of the lesion)
*Lesions of the cochlear nucleus or auditory nerve produce an ipsilateral sensorineural hearing loss.
- CN IX - diminished or absent gag reflex
-CN X - dysphagia or hoarseness, palate will droop on affected side and the uvula will deviate away from the side of the lesion
- spinal nucleus and tract of CN V - loss of just pain and temperature on ipsilateral side of half the face (touch and corneal blink reflex will be intact)
Affected nerve tracts
- spinothalamic tracts - pain and temperature deficit in contralateral limbs and body
- descending hypothalamic fibers - ipsilateral Horner syndrome
* If solitary nucleus is affected, taste sensations may be altered
Medial Pontine Syndrome
occlusion of the paramedian branches of the basilar a.
- CN VI - internal strabismus of the ipsilateral eye (diplopia on lateral gaze to the affected side)
- Corticospinal tract - contralateral spastic hemiparesis of both limbs
- Medial lemniscus may be affected - contralateral deficit of proprioception and touch pressure and vibratory sensations in the limbs and body.
- CN VII may be affected if it extends laterally
- Long tract signs are the same as in medial medullary syndrome but the CN signs localize the lesion
Upper motor neuron
Due to spinal cord lesions
Myesthenia gravis
- AutoAbs against acetylcholine receptor on post-synaptic membrane
Signs and symptoms: - extraocular muscle weakness (ptosis and diplopia), bulbar muscle weakness (jaw fatigue with chewing and dysarthria with talking).
- symmetrical proximal weakness of the extremities (upper more than lower), neck (flexors and/or extensors), and bulbar muscles (dysarthria/dysphagia)
- Normal reflexes, muscles bulk/tone, sensory function and autonomic function.
Diagnosis: CPK is normal differentiating it from primary muscle problem
- Associated with the presence of a thymoma —> always order CT of the chest bc thymoma can sometimes be invasive.
- Sometimes associated with thyroid disease —-> ultrasound can be helpful.
Amyotrophic lateral sclerosis
- Affects distal muscles more
- sensory abnormalities and ocular deficits are usually absent
Mononeuropathies
- Affect both motor and sensory function of single nerve
Multiple sclerosis
- Autoimmune CNS demylenating disease
- Upper motor neuron signs
- Oligoclonal IgG bands in the CSF with normal opening pressure, protein, and cell count.
Treatment of acute exacerbations:
- IV steroids in symptoms are severe.
- Mild sensory symptoms do not require steroids.
- Steroids do not prevent future attacks or prevent progression.
Reduce frequency of acute exacerbations:
- interferon, plasmapheresis, cyclophosphamide, IV immunoglobulin, glatiramer acetate
Polyneuropathies
- in diabetes mellitus
- vitamin B12 deficiency
- Guillain-Barre syndrome
- Chronic inflammatory demyelinating polyneuropathy
Signs and symptoms: reduced or absent reflexes, parathesias, motor and sensory symptoms of both upper and lower extremities, but not usually bulbar symptoms
Exertional heat stroke
Risk factors: obesity, certain medications (anticholinergics, antihistamines, phenothiazines, tricyclics)
Clinical manifestations: core temperature >104F AND CNS dysfunction (altered mental status, seizure, confusion, irritability)
- Additional organ or tissue damage (renal/hepatic failure, DIC, ARDS, coagulopathic bleeding)
Other possible signs and symptoms: dry or sweaty skin, hypotension, tachycardia, hyperventilation, diarrhea, cramps, ataxia
Management: rapid cooling: ice water immersion preferred, fluids, electrolyte correction, no role for antipyretic therapy
- Evaporative cooling is preferred in nonexertional heat stroke (elderly patients with comorbidities).
Heat exhaustion
> 104 F, but no CNS dysfunction
Multiple system atrophy (Shy-drager syndrome)
Degenerative disease
Characteristics:
- Parkonsonism
- autonomic dysfunction
- widespread neurological signs (cerbellar, pryamidal, LMN)
- Accompanied by bulbar dysfunction and laryngal stridor.
Treatment: anti-Parkinsonism drugs are ineffective; treated w/ intavascular volume expansion w/ fludrocortisone, salt supplementation, alpha adrenergic agonists, and constrictive garments on the lower body
Idiopathic orthostatic hypotension
- Degeneration of postganglionic sympathetic neurons
- Solely autonomic dysfunction.
Familial dysautonomia (Riley-Day syndrome)
Autosomal recessive condition found in Ashkenazi Jews
- autonomic dysfunction with severe orthostatic hypotension.
Chemotherapy-induced peripheral neuropathy
- symmetrical parasthesias of the fingers and toes, early loss of ankle jerk reflexes, loss of pain and temperature sensation, occasional motor neuropathy
- Commonly due to platinum-based meds (cisplatin), taxanes (paclitaxel), and vinca alkaloids (vincristine)
Allodynia
Perception of a normally innocuous stimulus as painful
Benign essential tremor
- action tremor
Alzheimer’s disease
Pathophysiology: selective loss of cholinergic neurons
Early findings:
- anterograde memory loss
- visuospatial deficits
- language difficulties
- cognitive impairment with progressive decline
Late findings:
- neuropsychiatric
- dyspraxia (difficulty performing learned motor tasks)
- lack of insight regarding deficits
- noncognitive neurologic deficits (pyramidal and extra-pyramidal motor, myoclonus, and seizures)
- urinary incontinence
Criteria:
- two or more areas of cognitive deficits
- progressively worsening memory and other cognitive function
- no disturbance of consciousness
- onset after 60
- absence of other systemic or neurologic disorder causing progressive cognitive deficits
Treatment:
- first-line: cholinesterase inhibitors (donepizil, galantamine, rivastigmine).
- Moderate to severe: memantine
Stroke due to endocarditis
- commonly occurs in the MCA
- Antibiotics reduce the risk of septic emboli in patients with endocarditis.
Guillain-Barre syndrome
associated with Campylobacter jejuni, Herpes viruses, Mycoplasma, and H. influenza as well as certain vaccines (influenza)
Cause: antibodies directed against Schwann cells
Clinical features: symmetric ascending muscle weakness with absent or depressed DTRs
- bulbar symptoms (dysarthria and dysphagia)
- Facial nerve palsy
- Albumino-cytologic dissociation (elevated protein and normal cell count)
Diagnosis: nerve conduction studies which show demyelination
Treatment: IVIG or plasmapharesis
Miller-Fischer variant: begins with cranial nerve deficits (uncommon)
Malignant hyperthermia
-affects genetically susceptible patients who have anesthesia with halothane or succinylcholine
Seizures
Circumstances:
- sleep loss
- emotions
- alcohol withdrawal
- flashing light
Clinical clues:
- aura
- can occur with sleeping/sitting position
- head movements
- tongue biting
- rapid, strong pulses
Sequelae:
- delayed return to baseline
- usually sleepy and confused afterward (postictal state)
Syncope (vasovagal)
Circumstances:
- upright position
- emotions
- heat
- crowded places
Clinical cues:
- symptoms of presyncope (lightheadedness)
- unlikely to occur with sleeping/sitting position
- rarely, several clonic jerks can occur with prolonged cerebral hypoperfusion as well as urinary incontinence
- pallor and diaphoresis
- weak, slow pulses
Sequelae:
- immediate spontaneous return
Hypokalemia
Symptoms: - weakness - fatigue - muscle cramps Severe: - flaccid paralysis - hyporeflexia - tetany - rhabdomyolysis - arrhythmias
ECG findings:
- broad flat T waves
- U waves
- ST depression
- premature ventricular beats
Can lead to:
- torsades de pointes
- V fib
- A fib
Deep brain stimulation
Used to treat Parkinson’s disease, dystopia, or essential tremors; blocks faulty nerve signals that cause tremors, rigidity, etc.
Lamotrigine
Lamictal
Used to treat bipolar I disorder and epilepsy (partial-onset seizures, generalized seizures of Lennox-Gastaut syndrome, and primary generalized tonic-clonic seizures)
Risk: serious skin rash - greater risk in pediatric pts
Metabolic inhibitor of OCT2
Mechanism: triazine derivative which inhibits the release of glutamate and inhibits voltage-sensitive Na channels, stabilizing neuronal membranes
- weak inhibitor of 5-HT3 receptor
- inhibits dihydrofolate reductase
Lennox-Gastaut syndrome
Severe seizures in childhood
- presentation usually before 8 yrs (typically 3-5)
- may evolve from other epilepsy syndromes
Defining criteria:
- multiple seizure types, particularly tonic and atypical absence seizures, but also atomic and myoclonic seizures. Periods of nonconvulsive status epilepticus occur in most cases at some stage.
- slow (less than 2.5 Hertz) spike wave pattern on the interictal EEG that is generalized and usually has highest amplitude in the frontal region
- mental retardation with or without other neurologic abnormalities.
Treatment:
- no drug is highly effective
- kerogenic diet and VNS
Levetiracetam
Keppra
Use: myoclonic seizures (juvenile myoclonic epilepsy), partial-onset seizures, and primary generalized tonic-clonic seizures
Off-label: status epilepticus, in setting of subarachnoid hemorrhage to prevent seizures
Side-effects: high BP, behavioral problems,
Mechanism: unknown
Donepizil
acetyl-cholinesterase inhibitor used to treat Alzheimer’s
Pseudodementia
- Key factor in differentiating between this and Alzheimers disease is the awareness of problems.
Relapsing-remitting MS treatment
Interferon-beta slows the progression
Progressive MS treatment
Methotrexate, cyclosporine, mitoxantrone
Trigeminal neuralgia
Treatment of choice: carbamazepine (second-line treatment: gabapentin and lamotrigine)
- In cases resistent to medical therapy, treat with surgery to relieve pressure.
Carotidynia
- Inflammation of the carotids and the vagus
Diagnosis: clinical, but sometimes requiring MRI
Burning mouth syndrome
- caused by a virus
Symptoms and signs: reddened mucosa with significant pain
- aggravated by dryness
Treatment: supportive care
Huntington’s chorea CT findings
- atrophy of the caudate nucleus seen as enlargement of the lateral ventricles.
Atrophy of the lenticular nucleus
- seen in Wilson’s disease
Pick’s disease
Ct findings: atrophy of the fronto-temporal cortex
Agnosia
- inability to recognize sensations and hence identify things.
Creutzfeldt-Jakob disease
Probable diagnosis:
- Rapidly progressive dementia
- 2 out of 4 clinical features:
- myoclonus
- akinetic mutism
- cerebellar or visual disturbance
- pyramidal/extrapyramidal dysfunction (hypokinesia)
- Periodic sharp wave complexes on EEG (bi or tri-phasic)
&/OR
- Positive 14-3-3 CSF assay
Definitive diagnosis includes above features in combination with
- brain biopsy findings (gold standard)
OR
- demonstrated PRNP gene mutations
- Lack of inflammatory response
Other symptoms: insomnia, apathy, behavioral changes, and impaired vision
Normal pressure hydrocephalus
Additional symptoms: slow, broad-based, shuffling gait
- Fecal incontinence may be seen in severe disease.
- Memory loss w/o focal neurologic deficits
Diagnosis: Enlarged ventricles on CT or MRI along with normal opening pressure on LP
Treatment: sequential CSF removal (serial large volume LPs)
- Ventriculoperitoneal shunt may be placed if the patient responds to LP.
Herpes encephalitis
Cause: most commonly HSV-1
Symptoms and signs: acute onset (
Dominant optic atrophy
Kjer’s optic atrophy
Synopsis: affects the optic nerves, causing reduced visual acuity and blindness beginning in childhood
Clinical presentation:
2 forms:
1. Isolated bilateral optic neuropathy (non-syndromic)
2. Extra-ocular signs (syndromic)
Pathophysiology: optic nerve fiber loss due to mitochondrial dysfunction
Treatment: none
Orthostatic tremor
- Postural tremor thought to be a variant of essential tremor.
- Occurs in the legs upon standing and is relieved by sitting down
- High frequency (14-18 Hz) without other clinical symptoms.
Large fiber peripheral neuropathy
- Can present with action or postural tremors due to impaired proprioceptive input.
Metastatic brain cancer
Lung > breast > unknown primary > melanoma > colon
Primarily solitary brain mets: breast, colon, renal cell carcinoma
Multiple brain mets: lung cancer, malignant melanoma
Rare brain mets: prostate cancer, esophageal, oropharyngeal, hepatocellular carcinoma, non-melanoma skin cancers
Intracerebral hemorrhage
Characteristics: acute focal neurologic deficits that gradually worsen over time —> elevated intracranial pressure
Risk factor: hypertension
Central cord syndrome
Cause: hyperextension injuries frequently in elderly patients with pre-existing degenerative changes in the C-spine
Mechanism: selective damage to the central portion of the anterior spinal cord, specifically the central corticospinal tracts and the decussating fibers of the lateral spinothalamic tract
Signs and symptoms: weakness more pronounced in the upper extremities
- May have loss of pain and temperature sensation in the arms due to damage to the spinothalamic tract
Anterior (ventral) cord syndrome
- Bilateral spastic motor paresis distal to the lesion
Cause: occlusion of the anterior spinal artery or burst fracture of the vertebra
Brown-Sequard syndrome
- Hemisection of the cord (most often due to a penetrating injury).
Characteristics:
- Ipsilateral: weakness, spasticity, loss of vibration sense and proprioception
- Contralateral: loss of pain and temperature sensation.
Posterior (dorsal) cord syndrome
- Bilateral loss of vibratory and proprioceptive sensation , often with weakness, parasthesias, and urinary incontinence or retention.
Causes: most commonly MS and vertebral artery dissection
Primidone side effects
- Is converted to phenylethylmalonamide and phenobarbital
- Can participate acute intermittent porphyria: abdominal pain, neurologic and psychiatric abnormalities.
diagnosis: test urine for porphobilinogen
Parkinson’s disease
Treatment: amantadine (dopamine agonist)
Hypopyon
Inflammatory cells in the anterior chamber of the eye
Anterior spinal artery occlusion
- Bilateral signs commonly seen at mid-thoracic levels with sparing of the dorsal columns.
- Affects the corticospinal tracts and spinothalamic tracts —> spastic paresis and loss of pain and temperature.
Miller-Fischer test
- Assessment of gait and cognitive function both before and after a large-volume lumbar puncture to assess for normal pressure hydrocephalus
Meniere’s disease
classic triad: episodes of dizziness lasting 30 minutes to several hours, unilateral tinnitus, and fluctuating hearing loss
-Thought to be due to an increase in the volume of endolymph —> permanent damage of the vestibular and cochlear appartuses
Treatment: symptomatic with meclizine and scopalimine
Botulism
- Descending motor weakness
Allergic conjunctivitis
Treatment: frequent attacks - antihistamine and mast cell stabilizing properties (ketotifen - H1 antagonist , mast cell stabilizer and eosinophil inhibitor)
Tenting at the junction of the optic nerve and the globe
Expanding of the retrobulbar space causing the globe to stretch the optic nerve
Vitamin A deficiency
night blindness, xerosis and anemia
Retinitis pigmentosa
progressive peripheral vision loss and night vision loss leading to central vision loss
Physical exam: mid-peripheral retinal hyperpigmentation and optic nerve waxy pallor
Pseudotumor cerebri
Diagnosis:
1) MRI to r/o other intracranial processes
2) lumbar puncture to establish diagnosis
3) acetazolamide to treat
Acute angle-closure glaucoma
Treatment: cholinergics, epinephrine, alpha-adrenergics,
emergency treatment: carbonic anhydrase inhibitors, mannitol, and topical beta-blockers
Viral conjunctivitis
Symptoms: clear discharge, preauricular adenopathy
- No treatment necessary, usually resolves in 5-7 days
Progressive multifocal leukoencephalopathy
Symptoms: diplopia, gait ataxia, altered mental status
Diagnosis: CT scan of the brain showing diffuse lesions of the white matter
Treatment: initiate HAART therapy
Central retinal artery occlusion
- Sudden, painless unilateral loss of vision
Opthalmoscopy: pale retina and cherry red spot at the fovea
Epidural abscess
- Most commonly due to S. aureus, but also gram negative rods, Streptococci, and coagulase negative Staph.
Periventricular leukomalacia
Leads to cerebral palsy, specifically spastic diplegia (more commonly of the lower extremities)
Athetoid cerebral palsy
due to damage of the basal ganglia
Causes: kernicterus, asphyxia, or genetic/metabolic diseases
Symptoms: hypotonia with later rigidity and dystonic movements
Cotard’s syndrome
Mental disorder in which patients complain of having lost their possessions, status, strength, and even inner organs
Dementia pugilistica
post-traumatic dementia occurring after blunt-force trauma to the head
Symptoms: decreased attention, slowed information processing, increased distractibility, and problems with memory. Behavioral changes include impulsivity, depression, aggression, and personality changes
beta-2 transferrin test
test to confirm leakage of CSF
- specific for CSF fluid, but can also be found in vitreous humor and the serum of newborns.
Signs of CSF leak: orthostatic headache and tinnitus, neck pain or stiffness, nausea and vomiting, anorexia, vertigo, dizziness, and change in vision
- Defects in anterior cranial fossa —> leakage through the nose; defects in middle cranial fossa —> leakage through the ears
Complications: meningitis (most commonly with Strep pneumonae - most common cause of meningitis in adults)
Reflex sympathetic dystrophy
- Pain and swelling of one extremity (commonly a hand), associated with skin atrophy.
- Thought to be due to vasomotor instability.
Sebaceous cell carcinoma
- Often mistaken for recurrent chalazion
- Diagnosed via biopsy
Polycythemia vera
Treatment: phlebotomy along with hydroxyurea
- Add in aspirin unless contraindicated.
- Interferon A is used in patients who have refractory pruritis or don’t respond to other treatment.
Anagrelide
- Used to manage patients with thrombocytosis who are refractory to other forms of treatment.
Risks: heart failure and orthostatic hypotension in elderly with CV disease
Chlorambucil
- Used to treat CLL and non-hodgkin’s lymphoma
Cluster headache
- May be triggered by alcohol
- Thought to be involve the hypothalamic nuclei that regulate circadian rhythm.
- Pain and vasomotor symptoms probably mediated by seritogenic pathways
Noncommunicating syringomyelia
- Associated with trauma
Syringobulbia
fluid-filled cavity in the medulla
Symptoms: dysphagia, nystagmus, phyrangeal and palatal weakness, asymmetric weakness and atrophy of the tongue, and loss of pain and temperature in the trigeminal nerve distribution.
Non-proliferative diabetic retinopathy
small-vessel disease in the eye
- Occlusion, dilation and increased permeability of the small vessels in the eye —> microaneurysms
- Serous fluids and lipoproteins leak —> hard exudates
Small vessel ischemia —> microinfarctions of the nerve fiber layer —> cotton wool spots
Macular edema treatment
lasar photocoagulation
Proliferative diabetic retinopathy
- Prolonged ischemia and neovascularization
Treatment: lasar photocoagulation to prevent blindness
Cataracts
- Followed closely until functional visual impairment results
- Surgery removes the cataract, leaving the posterior lens capsule. if the capsule opacifies, it can be removed with laser (ultrasonic fragmentation of the lens nucleus).
Bethanechol
Bladder-selective cholinergic agent that helps increase bladder contractility
Oxybutinin
Anticholinergic used to treat urine loss
Age-related macular exudative degeneration
Progression: age-related maculopathy (‘drussen’) —> atrophic (dry) or exudative (wet) degeneration —> no therapy has been proven to treat dry form, but lasar photocoagulation of the subretinal neovascular membranes may delay wet form
CMV retinitis
Symptoms: scotoma, visual field deficits, floaters or flashing lights
Management: perform a fundoscopic exam to r/o retinal detachment —> treat with IV foscarnet or gancyclovir
Acute dystonia
- Includes torticollis
- Can be due to drugs used to treat Parkinson’s disease (dopamine receptor blockade)
Treatment: dopamine agonst (carbidopa/levadopa), anticholinergic/antihistamine (diphenydramine, benztropine, trihexphenidyl), muscle relaxant or benzos
- Focal dystonias can be trated with botox
Toxic labrynthitis
Vertigo caused by alcohol or medications (commonly aminoglycosides)
Pronator drift
Sign of upper motor neuron problem - supination is weaker than pronation
Mononeuritis multiplex
Painful, asymmetric, asynchronous sensory and motor peripheral neuropathy involving damage to > or = to 2 nerves
-Seen in patients with systemic vasculitides
Cryoglobulinemia
- Commonly seen in hepatitis B and C
Deposition of antibody-antigen complexes in small and medium sized arteries
Symptoms: palpable purpura, arthralgias, peripheral neuropathy, and hypocomplementemia
corticosteroid-induced myopathy
-Lower extremity weakness and proximal muscle atrophy
Diagnosis: normal muscle enzymes and EMG results
Treatment: taper the steroids and wait for improvement (3-4 weeks)
Type I spinal motor atrophy (Werdnig-Hoffman disease)
Degenerative disease of motor neurons that begins in fetal life and progresses during infancy
- Progressive denervation of muscles with following atrophy ; no DTRs with fasciculations of the tongue
- Death occurs in early infancy to 2 years old
Diagnosis: muscle biopsy showing perineural denervation, or genetic tests
Sumatriptan
serotonin agonist that is used to treat migraines
Hypoxic-ischemic encephalopathy
- most common cause of neonatal seizures
Treatment: benzos
Tuberous sclerosis
Symptoms: hypopigmented ash-leaf lesions (more visible under UV light), infantile spasms and seizures —> mental retardation
Cause: seizures are thought to be caused by excess CRH which causes excess excitability
Diagnosis: EEG showing hypsarrhythmia (high voltage, asynchronous chaotic slow wave activity)
Treatment: ACTH which suppresses CRH
Wernicke’s encephalopathy
Cause: thiamine deficiency
Classic triad:
- encephalopathy w/ disorientation and indifference
- oculomotor dysfunction (nystagmus, lateral rectus plasy and conjugate gaze palsies)
- Gait ataxia due to combined polyneuropathy, cerebellar involvement, and vestibular paresis
Treatment: give magnesium, thiamine and glucose
- magnesium is needed for thiamine transport into neurons
- give thiamine before glucose because glucose will use up pyruvate dehydrogenase for which thiamine is a cofactor, making the problem worse
Delerium tremens
Treatment: benzodiazipines and haloperidol (for agitation)
Carotid artery dissection signs
Horner’s syndrome: miosis, anhydrosis, and ptosis
