Family Medicine: Neurology

Ischemic Stroke

Acute focal neurologic deficit resulting from diminished blood flow to an area of the brain due to thrombosis, embolism, or systemic hypoperfusion

-Presentation: contralateral paralysis, can wrinkle forehead (vs Bell’s palsy)

-Dx: non-contrast head CT to rule out hemorrhage

-Tx: IV thrombotics (tPA or Tenecteplase) for eligible patients within 3-4.5 hours of symptom onset, mechanical thrombectomy for large vessel occlusion, lower BP if > 220/120, start ASA + clopidogrel x 21 days, statin therapy, BP control

Carotid

What artery is implicated in this ischemic stroke?

-Amaurosis fugax

MCA

What artery is implicated in this ischemic stroke?

-Aphasia (dominant hemisphere), neglect, hemiparesis, gaze preference, homonymous hemianopsia

ACA

What artery is implicated in this ischemic stroke?

-Leg paresis, hemiplegia, urinary incontinence, abulia

PCA

What artery is implicated in this ischemic stroke?

-Homonymous hemianopsia, visual agnosia, alexia without agraphia

Basilar

What artery is implicated in this ischemic stroke?

-Coma, cranial nerve palsies, apnea, drop attack, vertigo

Lacunar Infarct

What type of ischemic stroke is being described?

-Pure motor stroke, pure sensory stroke, dysarthria, ataxic hemiparesis

Hemorrhagic Stroke

~13% of strokes, further divided into intracerebral hemorrhage and subarachnoid hemorrhage

-Causes: HTN, cerebral amyloid angiopathy, anticoagulation, AVM, trauma, brain tumor, drugs

-Presentation: sudden severe headache, vomiting, and decreased LOC alongside focal neurologic deficits. Markedly elevated BP, bradycardia, irregular respirations, papilledema, fixed pupils

-Dx: non-contrast CT of the head

-Tx: stop bleeding, lower BP, treat ICP

TIA

Transient episode of neurologic dysfunction due to focal brain, retinal, or spinal cord ischemia without acute infarction on MRI. The blockage does not last long enough to cause permanent damage

-Presentation: mirror stroke symptoms but last minutes to < 1 hour with complete resolution of symptoms within 24 hours

-Dx: CT without contrast, MRI with diffusion-weighted imaging is more sensitive for excluding small infarcts, carotid doppler US to evaluate for stenosis, EKG to rule out AFIB, ABCD score

-Tx: admit to hospital, ASA 325 immediately + clopidogrel x 21 days, statin, carotid endarterectomy if > 70% stenosis

Carotid Endarterectomy

What is indicated when a patient has symptomatic internal/common carotid artery stenosis > 70%?

-Associated with amaurosis fugax in TIAs and strokes

Bell’s Palsy

Cranial nerve VII palsy, can be due to HSV or URIs

-Presentation: acute onset of unilateral facial weakness where both the upper and lower parts of the face are affected, unable to wrinkle forehead, decreased taste, hyperacusis

-Dx: clinical, consider Lyme disease

-Tx: oral prednisone within 72 hours of onset, antivirals if herpes zoster is suspected

CN III

What cranial nerve is affected?

-”Down and out” eye, ptosis, mydriasis

-If painful and involving the pupil, think PCA aneurysm

-If painless and pupil sparing, think ischemic/microvascular

CN IV

What cranial nerve is affected?

-Vertical diplopia, worse when looking down (trouble going down the stairs), head tilt away from the affected side. Often post traumatic

CN VI

What cranial nerve is affected?

-Inability to abduct the affected eye, leading to horizontal diplopia worse at distance

-Often the first sign of elevated ICP

CN V

What cranial nerve is affected?

-Paroxysmal lancinating facial pain

Alzheimer Disease

Neurodegenerative disease characterized by beta-amyloid plaque and neurofibrillary tangle formation, leading to impaired neuronal signaling and neuronal apoptosis

-Most common form of dementia and a leading cause of death in older adults

-Presentation: starts with short-term memory loss and progresses to loss of motor skills, long-term memory, disorientation, and death (5-10 years)

-Dx: clinical, definitive is by brain autopsy. Can diagnose by intellectual decline in 2+ areas of cognition documented by the MMSE. CT/MRI can show diffuse cortical atrophy with enlargement of the ventricles

-Tx: cholinesterase inhibitors (rivastigmine, donepezil) and memantine, avoid anticholinergics

Rivastigmine + Memantine

What is the dual therapy for Alzheimer’s?

Dementia

Significant or moderate impairment of cognition or memory that represents a marked deterioration from a previous level of function, associated with increasing age

-Dx: MMSE/Mini-Cog, labs to rule out reversible causes (B12 deficiency, hypothyroidism, normal pressure hydrocephalus)

-Tx: tx reversible causes if possible

Vascular Dementia

Second most common type of dementia, which is associated with arteriosclerotic small-vessel disease

-Correlates with cerebrovascular events

-Stepwise deterioration with periods of clinical plateau, which may cause a sudden decline after each event

-Tx: aggressive vascular risk factor control

Frontotemporal Dementia

Form of dementia where personality changes precede memory changes

Lewy Body Dementia

Form of dementia characterized by Parkinsonian motor symptoms, visual hallucinations, rapid eye movement sleep behavior disorder

-Severe neuroleptic sensitivity, avoid antipsychotics

Delirium

Acute cognitive dysfunction secondary to an underlying medical condition and is usually reversible

-Presentation: acute, rapid deterioration in mental status with a fluctuating level of awareness and disorientation. Inattention and visual hallucinations are most common symptoms. Seen after surgeries and in alcohol withdrawal

-Dx: MMSE, labs to rule out reversible causes

-Tx: address underlying cause, nonpharmacologic reorientation, low-dose haloperidol for severe agitation

Multiple Sclerosis

Immune-mediated, chronic inflammatory demyelinating disease of the CNS characterized by lesions disseminated in time and space

-Peak onset in women aged 20-40, MC is relapsing-remitting

-Presentation: episodes of focal neurologic dysfunction lasting > 24 hours such as optic neuritis, internuclear ophthalmoplegia, paresthesias, electric sensation down spine with neck flexion, hyperreflexia, Babinski sign, cerebellar signs, urgency, transient worsening of symptoms with heat, fatigue

-Dx: MRI of the brain and spinal cord with and without contrast, LP shows oligoclonal bands

-Tx: high dose IV methylprednisolone, disease modifying therapy to reduce relapse rate

Optic Neuritis

Unilateral painful vision loss, central scotoma, decreased color vision, afferent pupillary defect

-Classic first presentation of MS

Parkinson Disease

Loss of dopamine-containing neurons in the substantia nigra and locus coeruleus, which can as a result of an acetylcholine and dopamine imbalance + show Lewy bodies on pathology

-Presentation: resting tremor, cogwheel rigidity, and bradykinesia. Can also present with masked facies, loss of postural reflexes, shuffling, stooped posture, anosmia, REM sleep behavior disorder, constipation, depression

-Dx: clinical, Levodopa response, MRI, gold standard is autopsy

-Tx: symptomatic care, levodopa/carbidopa if > 65 or bromocriptine if < 65 years to delay levodopa dyskinesia

Essential tremor

Bilateral postural/action tremor, which worsens with voluntary movement, of the hands, forearms, and/or head without a resting component

-Shaking occurs with simple tasks like tying shoelaces, handwriting, shaving, holding cups, or holding the hands against gravity. Improves with alcohol

-Autosomal dominant

Propranolol

What is the first line treatment for an essential tremor?

Tourette Syndrome

Multiple motor tics + > 1 vocal tic, onset before age 18 with a duration > 1 year

-Strongly comorbid with OCD and ADHD

-Tx: behavioral therapy, clonidine or aripiprazole for refractory cases

Fetal Alcohol Syndrome

Condition affecting children of mothers who consumed alcohol during pregnancy

-Presentation: Must have all three

• Characteristic facial features → smooth philtrum, thin vermilion border of the upper lip, short palpebral fissures

• Pre- and/or postnatal growth restriction

• Central nervous system abnormalities → microcephaly, intellectual disability, learning disabilities, hyperactive and impulsive behaviors, executive dysfunction

-Can also have congenital heart defects like VSD and ASD

-Dx: clinical diagnosis

-Tx: prevention is the only cure

Antalgic Gait

Shortened stance phase on the painful side

-Associated with osteoarthritis, hip/knee injury

Parkinsonian Gait

Stooped posture, narrow base, shuffling steps, decreased arm swing, festination, and freezing

Hemiparetic Gait

Arm flexed, leg extended/circumducted. History of prior stroke

Apraxic Gait

Feet appear glued to the floor, wide base, small steps. Associated with normal pressure hydrocephalus

Ataxic Gait

Wide based, lurching, unsteady, worse with eyes closed

-Associated with alcohol intoxication, cerebellar stroke, multiple sclerosis, B12 deficiency

Shunt

What is the treatment of choice for normal pressure hydrocephalus?

Wet, wobbly, wacky

What is the triad of normal pressure hydrocephalus?

-Urinary incontinence, gait apraxia, dementia

Cluster Headache

Unilateral, excruciating, sharp, searing, or piercing pain with ipsilateral autonomic features like lacrimation, nasal congestion, conjunctival injection, ptosis, miosis, and eyelid edema

-Seen most often in males, patients are restless

-Tx: 100% oxygen at 12-15 L/min x 15 minutes via non-rebreathing mask + sumatriptan

Verapamil

What is the first line prophylactic treatment for cluster headaches?

Migraine

A headache of varying intensity, often unilateral, pulsating, with nausea and sensitivity to light and sound

-Seen most often in teenage to middle age females

-Presentation: pulsating, lasts 4-72 hours, unilateral, disabling with photophobia and phonophobia. Without aura is MC. Triggered by stress, sleep deprivation, menstruation, red wine, aged cheese, MSG, nitrates, and missed meals

-Dx: clinical

-Tx: sumatriptan, antiemetics, beta-blockers/antiepileptics as preventatives

Tension Headache

Bilateral, mild-to-moderate, dull pain that is not worsened by routine activity

-Tx: NSAIDs, acetaminophen, ASA + caffeine

Increased ICP

Sustained rise in pressure inside the rigid skull above the normal ~ 7-15 mmHg in adults. Sustained elevation induces cerebral perfusion and can cause herniation and death

-Causes: TBI, hemorrhage, tumor, hydrocephalus, meningitis, encephalopathy, IIH

-Presentation: headache that is worse in the morning, N/V, papilledema on fundoscopy, visual changes, AMS, herniation symptoms

-Dx: non-contrast CT of the head, LP for opening pressure, MRI, fundoscopy

-Tx: address underlying cause, elevate head of bed to 30 degrees, mannitol or hypertonic, sedation/analgesia, dexamethasone

> 60

In a patient with elevated ICP, you need to maintain a cerebral perfusion pressure (MAP - ICP) of what?

Idiopathic Intracranial Hypertension

Headache, transient visual obscurations, papilledema, pulsatile tinnitus in a young, obese, female

-Dx: normal imaging, elevated opening pressure on LP with normal CSF composition

-Tx: weight loss + Acetazolamide

Glioblastoma

Most common primary malignant brain tumor in adults

-”Butterfly” lesion crossing the corpus callosum on MRI

-Has a poor prognosis

Meningioma

Most common benign adult brain tumor

-Dural based, extra-axial mass with a “dural tail” and homogenous enhancement

-More common in women

-Only surgical if symptomatic

Pituitary Adenoma

Pituitary tumor associated with bitemporal hemianopsia, hormone hypersecretion, acromegaly, Cushing disease

Acoustic Neuroma

Cranial nerve VIII tumor at the cerebellopontine angle, leading to unilateral sensorineural hearing loss + tinnitus + imbalance

Pilocytic Astrocytoma

Most common pediatric brain tumor

-Cystic cerebellar lesion with a mural nodule

Medulloblastoma

Highly malignant cerebellar tumor of childhood

-”Drop metastases” via CSF

Ependymoma

Pediatric brain tumor found in the fourth ventricle

Brain Tumor

Benign or malignant masses of the brain

-Presentation: progressive headache that is worse in the morning or with Valsalva, new-onset focal seizures, personality changes, N/V, papilledema, signs of increased ICP, constitutional symptoms

-Dx: MRI of the brain with/without contrast, biopsy

-Tx: surgical resection, radiation or chemo

Polyneuropathy

Loss of nerve function in multiple areas

-MCC in the US is diabetes mellitus (think feet here) but can also be seen with alcohol use disorder, B12 deficiency, and chemo

-Presentation: stocking-glove burning/tingling/numbness, length-dependent, decreased vibration/proprioception, loss of ankle reflexes. Leads to foot ulcers, Charcot joint, autonomic dysfunction

Carpal Tunnel

Numbness/tingling in the thumb, index, middle, and radial half of the ring finger due to compression of the median nerve

-Dx: positive Tinel/Phalen

-Tx: night splints, NSAIDs, steroid injection, surgical release if refractory

Cubital Tunnel

Numbness of the 4th/5th digits, weak grip, claw-hand deformity due to ulnar neuropathy of the elbow

Peroneal Nerve Palsy

Lateral knee compression leading to foot drop, sensory loss over the dorsum of the foot

Guillain-Barre Syndrome

Acute, ascending symmetric weakness + areflexia 1-3 weeks after an infection (Campylobacter, CMV, EPV, Zika, COVID)

-CSF shows albuminocytologic dissociation

-Tx: IVIG or plasmapharesis

Carbamazepine

What is the treatment of choice for trigeminal neuralgia?

Trigeminal Neuralgia

Brief, severe, lancinating, “electric shock-like” facial pain in the trigeminal distribution that is triggered by light touch, chewing, brushing teeth, or cold air

Postherpetic Neuralgia

Persistent burning pain > 90 days after a herpes zoster rash in the same dermatome.

-Tx: gabapentin

Generalized Seizure

Seizures that occur when there is widespread seizure activity in the left and right hemispheres of the brain, starting in the midbrain or brainstem and spreading to both cortices

-Dx: witness history, labs, EEG, MRI brain

-Tx: valproate, levetiracetam, lamotrigine

Absence Seizures

Brief impairment of consciousness with an abrupt beginning and ending

-The patient has no recollection and witnesses often miss them

-EEG shows 3-Hz spike-and-wave activity

-Tx: ethosuximide

Tonic-Clonic Seizure

Bilaterally symmetric seizures without focal onset

-Presentation: sudden loss of consciousness with a fall to the ground. Stiff and rigid phase (10-60 seconds) followed by generalized convulsions and limb jerking. Associated with a postictal phase

-Tx: valproate, lamotrigine, levetiracetam

Atonic Seizure

Sudden loss of muscle tone that can mimic syncope

Myoclonic Seizure

Brief muscle jerks, which often occur in the morning

-Tx: valproate or levetiracetam

Febrile Seizure

Convulsion associated with fever > 38 C, age 6 months to 5 years, no CNS infection associated

-Simple = generalized, < 15 min, once in 24 hours

-Complex: focal features, > 15 min, or recurrent in 24 hours

Infantile Spasms

Clusters of brief tonic spasms in infants

-EEG shows hypsarrhythmia

-Tx: ACTH

PNES

Not due to epilepsy but resemble seizures clinically

-Dx: video EEG monitoring

-Tx: therapy

Status Epilepticus

A single seizure lasting > 5 minutes or two or more seizures within 5 minutes without return to baseline between them, which can present in convulsive and nonconvulsive forms alike

-Tx: IV lorazepam, intubation

-Dx: witness history, EEG, MRI brain

Valproate

What antiepileptic medication should be avoided in pregnancy due to its teratogenic nature?

Subarachnoid Hemorrhage

Bleeding into the subarachnoid space between the arachnoid and pia mater, which is typically caused by the rupture of a saccular aneurysm

-Causes: traumatic SAH, AVM, vasculitis, cocaine, HTN, smoking, heavy alcohol use

-Presentation: sudden, severe “thunderclap” headache, nausea, vomiting, photophobia, neck stiffness, focal deficits

-Dx: non-contrast CT of the head, LP shows xanthochromia

-Tx: secure aneurysm, BP control, Nimodipine to prevent vasospasm, pain control, antiemetics, stool softeners, seizure prophylaxis

Syncope

Transient loss of consciousness and postural tone secondary to an acute decrease in cerebral blood flow, characterized by rapid recovery of consciousness without resuscitation

-Causes: vasovagal, orthostatic hypotension, cardiac, cerebrovascular disease

-Dx: H&P, EKG, orthostatic vital signs, labs

-Tx: treat the underlying cause

Vasovagal Syncope

MCC of syncope

-Classic prodrome of warmth, nausea, diaphoresis, tunnel vision, then brief LOC after standing, pain, fear, micturition, or coughing

Orthostatic Hypotension

Defect in vasomotor reflexes, which is common in elderly, diabetics, and patients on diuretics, vasodilators, alpha blockers

Cardiac Syncope

Syncope due to arrhythmias, structural disease

-Red Flag Sx: exertional syncope, syncope without prodrome, palpitations, family history of sudden cardiac death, abnormal EKG

TBI

Traumatic brain injury on the spectrum from concussion to intracranial hemorrhage, which is listed as the leading cause of death and disability in patients < 45.

-Dx: ABCs + cervical spine immobilization, GCS, non-contrast head CT

-Tx: depends on severity, ranging from observation to sedation/intubation

Concussion

Brief alteration of mental status + loss of consciousness

-Presentation: headache, dizziness, nausea, photophobia, difficulty concentrating, irritability, sleep disturbance

-Risk of second impact syndrome

-Tx: brief physical and cognitive rest, then gradual return. Tylenol for headache, avoid NSAIDs/ASA

Epidural Hematoma

Arterial bleed above the dura (middle meningeal)

-CT: biconvex/lens-shaped hyperdensity that does not cross suture lines

-Presentation: classic “lucid interval” followed by rapid deterioration

-Tx: surgery

Subdural Hematoma

Venous (bridging veins) bleed below the dura mater

-CT: crescent-shaped hyperdensity that can cross suture lines but not the midline

-Common in elderly, alcoholics, and anticoagulated patients

Skull Fracture

Linear, depressed, or basilar

-Basilar → raccoon eyes, Battle sign, hemotympanum, cerebrospinal fluid rhinorrhea