aka trigeminovascular syndrome
Paroxysmal headache (HA) associated with multiple signs and symptoms that can overlap with sinus symptoms
Throbbing pulsatile headache in the frontotemporal or orbital area
Can arise before or during the HA
Usually visual but can be sensory or motor
Pain builds over 1 to 2 hours and progresses posteriorly
Headache lasts 4 to 72 hours
Nausea—80%, vomiting—50%, anorexia and food intolerance and light headedness
Tachycardia or bradycardia
Hypertension or hypotension
Hemisensory changes or hemiparesis
Adie’s-like pupil (light-near dissociation)
Two of the following characteristics
Moderate to severe pain
Aggravated or caused by physical activity
During the HA patient must have one of the following
Nausea and/or vomiting
Photophobia and phonophobia
Diagnostic imaging is not necessary in patients with stable history of migraine headaches and a normal neurologic examination.
CT is not recommended for headache evaluation when MRI is available, unless in an emergency situation.
Persistent over-the-counter (OTC) pain medication for the treatment of unrelenting headache is not advised as this symptom may indicate underlying brain pathology.
First-line treatment of migraine should not include opioid or butalbital-containing medications.
Avoidance of carrying heavy purses or bags over one shoulder
Avoidance of certain foods
Maintaining hydration status
These medications aim to reverse or at least stop the progression of a headache.
Most effective when given within 15 minutes of symptom onset when the pain is mild.
Triptans: selective serotonin receptor agonists
Ergot alkaloids: ergotamine, dihydroergotamine
Analgesics and nonsteroidal anti-inflammatory drugs (NSAIDs)
Acetaminophen, aspirin, and caffeine (ie, Excedrin)
Butalbital, aspirin, and caffeine (Fiorinal)
Isometheptine, dichloralphenazone, acetaminophen (Midrin, Duradrin, and others)
More than two headaches per month
Duration of headache is more than 24 hours
Significant disability for equal to or more than 3 days
Abortive therapy fails or is overused
Abortive medications used more than twice a week
Ca2+ channel blockers
Botulinum toxin: up to nine treatment cycles for progressive improvement in symptoms
Most common type of recurring headache thought to be related to muscular factors and psychogenic forces (stressful event).
Throbbing quality with onset more gradual than migraines; usually tension headaches are more constant and less severe.
Headaches can last up to 7 days.
Not associated with nausea or vomiting, photophobia, and/or phonophobia.
Two of the following must be present:
Tightening in frontal-occipital locations
Mild to moderate intensity
Not aggravated by physical activity
Imaging is only required if headache pattern changes and is not a common primary headache disorder, such as migraine, cluster or tension headache
Massage, relaxation techniques
Lifestyle modification: regular exercise, balanced meals, adequate sleep
Trigger points injection/occipital nerve block
Tricyclic antidepressants, muscle relaxers
Group of headaches (known as histamine headaches) with multiple characteristics
Severe unilateral pain that is orbital, supraorbital, or temporal.
Each headache lasts 15 to 180 minutes and can occur eight times a day or every other day.
The HA may occur 1 to 8 times a day for as long as 4 months.
HA are often nocturnal, during sleep or early morning hours.
Associated with one or more of the following ipsilateral signs:
Two main forms of cluster headaches
Episodic: at least two cluster headache phase that last 7 days to 1 year are separated by a cluster-free interval of 1 month of longer.
Chronic: cluster occurs more than once a year without remission or the cluster-free interval is less than 1 month.
8 L/min for 10 minutes or 100% by mask
Stimulation of 5-HT1 (serotonin) receptors produce vasoconstrictive effect and may abort attack
Intranasal administration effective
Stimulation of alpha receptors to cause vasoconstriction
Most effective when given early in a cluster headache attack
Intranasal administration is effective
Calcium channel blockers
Verapamil considered most effective
Can be combined with ergotamine and lithium
Topiramate and divalproex found to be most effective for prophylaxis
Mechanism of action is unclear
Percutaneous radiofrequency ablation of the gasserian ganglion
50% success rate
20% have fair-to-good results
30% failure rate
Gamma knife radiosurgery
Increased risk for disturbance in facial sensation
Occipital nerve block
Abortive procedure for cluster headache
Deep brain stimulation (DBS)
Electrodes placed in ipsilateral posterior inferior hypothalamus.
Candidates for DBS are those patients who have chronic cluster headaches refractory to pharmacologic therapy.
Stimulation of the pterygopalatine ganglion
Candidate for stimulation are selected patients with chronic cluster headaches.
Distinct, sudden, lancinating pain following the sensory distribution of the trigeminal nerve
Pain is most similar to an “electric shock” that worsens in less than 20 seconds and begins to fade to burning
Pain shoots from the corner of the mouth to the angle of the jaw in 60%.
Pain shoots from the upper lip or teeth to the eyebrow or area around the eye in 30%.
V1 involved in less than 5% of cases.
V2 and V3 distribution affected in 35% of patients.
Triggers: chewing, talking, smiling, drinking hot or cold fluids, touching, shaving, teeth cleaning, encountering cold air on the face
Common causes include vascular compression at the root entry zone or demyelination due to diseases like multiple sclerosis (MS)
No laboratory, radiographic or electrophysiologic studies are routinely needed for patients with characteristic history and normal neurologic examination.
Criteria established by International Headache Society
Paroxysmal attacks of pain that lasts up to 2 minutes and involves a distribution of the trigeminal nerves (V1-V3).
Pain has at least one of the following characteristics: intense, sharp, superficial or stabbing, or pain starts from specific trigger areas or by triggering factors.
Attacks are stereotyped in the individual patient.
No neurologic deficit based on clinical examination.
Pain is not attributed to another disorder.
Multiple treatment options
75% of patients have substantial relief and adequate control with medical therapy.
Carbamazepine best studied and only indicated drug by FDA.
Initial response to carbamazepine is diagnostic.
Side effects: ataxia (15%), dizziness (44%), drowsiness (32%), nausea (29%), vomiting (18%).
Lamotrigine and baclofen are second-line options; gabapentin has been shown effective particularly with MS patients.
Percutaneous glycerol or thermal rhizotomy of the gasserian ganglion
Open cranial procedures (ie, microvascular decompression)
Most durable treatment
Radiation therapy (ie, gamma knife radiosurgery)
Cranial neuropathy syndromes
Episodic unilateral orbital or retro-orbital pain with opthalmoplegia
Involvement of cranial nerves (CN) III to VI
Thought to be due to a nonspecific granulomatous inflammation involving the posterior superior orbital fissure, orbital apex, and cavernous sinus
Diagnosis of exclusion (MRI needed to rule out other pathologies)
Symptoms last for average of 8 weeks if untreated
First division of trigeminal nerve involved causing neuropathic pain or sensory loss; other divisions of trigeminal nerve can also be involved
Sympathetic dysfunction also (miosis, ptosis) without anhidrosis
Neuroimaging necessary to exclude other diagnoses
Syndrome not specific to any underlying pathologic process
Apical petrositis: infection of petrous temporal bone
Due to infection extending from middle ear and mastoid air cells into the pneumatized petrous apex via the perilabyrinthine air tract
Involvement of Dorello canal and possibly the trigeminal nerve
Abducens nerve palsy with possible orbital or retro-orbital pain
Lesion (tumor, infection, fracture, thrombosis) of jugular foramen
CN IX to XI effected: ipsilateral trapezius and sternocleidomastoid weakness, dysphonia, dysphagia, loss of taste over posterior one-third of tongue, depressed pharyngeal sensation
Unilateral palsy of CN IX to XII
Lack of sympathetic dysfunction
Rule of thumb: acute neurologic deterioration with ipsilateral CN deficit(s) and contralateral sensory or motor dysfunction localizes the stroke to the brain stem (midbrain, pons, medulla)
Stroke of the thalamus involving the (PCA)
Contralateral sensory loss, contralateral dysesthesia (thalamic pain), ballistic or choreoathetoid movements, transient hemiparesis
Stroke of the caudal midbrain involving the posterior cerebral artery (PCA)
Ipsilateral gaze weakness (oculomotor nerve palsy), contralateral weakness
Stroke of the tegmentum (midbrain floor) involving the PCA
Oculomotor nerve palsy, contralateral hemichorea, hemiathetosis (red nucleus damage)
Stroke of the pons involving the paramedian branches of the basilar artery
Ipsilateral lateral gaze weakness, contralateral weakness
Stroke of inferior medial pons involving the basilar artery
Contralateral hemiparesis, ipsilateral facial palsy (CN VII), lateral gaze palsy (CN VI), reduced touch and position sense (medial lemniscus)
Millard Gubler syndrome
Stroke of the basis pontis (caudal pons) involving the basilar artery
Contralateral weakness, ipsilateral lateral gaze weakness, ipsilateral facial weakness
Palatopharyngeal paralysis of Avellis
Stroke of cephalad portion of nucleus ambiguous
Paralysis of the palatal, pharyngeal muscles with sparing of laryngeal muscles
Somatotopic organization of motor nucleus places laryngeal muscles caudal
Dejerine’s anterior bulbar syndrome
Stroke of paramedian medulla involving the basilar artery
Sudden onset of reduced touch and position sense (dorsal column/medial lemniscus) of the contralateral side of body with contralateral hemiplegia, preservation of pain and temperature, with ipsilateral tongue weakness (CN XII) and upbeat nystagmus, without facial droop
Stroke of lateral medulla involving the vertebral artery
Ipsilateral loss of facial sensation, ataxia, nystagmus/vertigo/nausea (vestibular nucleus involvement), dysphagia (nucleus ambiguous), Horner syndrome, contralateral hemisensory loss (spinothalamic tract)
Infantile spasms (West syndrome)
Sudden flexor or extensor spasms of head, trunk, or limbs
Associated with tuberous sclerosis (neurocutaneous syndrome)
On EEG, see hypsarrhythmia
Juvenile myoclonic epilepsy
Myoclonic jerks that arise usually in the morning
Patients have normal intelligence
On EEG, see 4- to 6-Hz spike waves and polyspike discharges
Benign focal epilepsy of childhood
Generalized seizures at night, focal seizures during the day
On EEG, see di- or triphasic sharp waves
Frequent, blank staring spells
On EEG see generalized, symmetric 3-Hz spike-wave discharge
Involuntary laughter with alternating crying or sobbing spells; with precocious puberty when associated with hypothalamic hamartoma
25% associated with hypothalamic hamartoma
21% of patients with hypothalamic hamartoma have gelastic seizures
Hypothalamic hamartoma associated with other midline deformities (ie, callosal agenesis)
Temporal lobe seizures
Most common in adults
Involves mesial temporal structures
Associated with auras (hallucinations, sensory illusions, viscerosensory symptoms such as sensation over the abdomen or chest wall) and automatisms (stereotyped repetitive movement of the mouth, tongue, lips or jaw → lip smacking)
Anterior temporal lobe resection is 80% effective
On EEG, see focal temporal slowing or epileptiform sharp waves or spikes in the anterior temporal lobe region
Inflammatory disorders of the nervous system
Multiple sclerosis (MS)
Acquired relapsing demyelinating disease that is disseminated throughout the central nervous system in time and space
Female predilection with peak presentation at 35 years of age
Optic neuritis or trigeminal neuralgia are common symptoms
Multiple patterns of presentation
Relapsing-remitting: most common; occasional symptoms that resolve on their own
Secondary progressive: begin as relapsing-remitting but then become progressively worse
Primary progressive: symptoms do not relapse and become progressive
Progressive with relapses: symptoms that resolve but when they return are worse than the prior symptom
Benign MS: patient with symptoms but remain functionally active for over 15 years
Dawson’s fingers: hyperintense lesions seen on MRI FLAIR sequence that are perpendicular to the ventricles
Open ring sing: enhancement is often incomplete on contrasted MRI studies
Lesions are often hyperintense on T2 MRI
IgG and albumin values from the serum and CSF should be measured in order to obtain the IgG index (a value greater than 0.7 is elevated).
Oligoclonal bands are often seen in CSF.
Amyotrophic lateral sclerosis
Disease of the upper and lower motor neurons
Signs of anterior horn cell disease without sensory neuropathy
Associated with Parkinson-like dementia complex
Most patients die within 5 years of symptom onset
Progressive multifocal leukoencephalopathy
Due to activation of JC virus (papovavirus) in immunocompromised patients
Reactivated virus attacks oligodendrocytes of subcortical white matter
Usually effects the parietal-occipital lobes
Seen in 4% of AIDS patients and patients with leukemia and lymphoma
MRI findings: hyperintense on T2 and FLAIR sequences, nonenhancing
Diagnosis confirmed by PCR analysis of JC virus RNA in CSF
Neurologic symptoms that arise after tick bite from Borrelia burgdorferi
Most common symptom is painful sensory radiculitis
Other symptoms are: Bell palsy, limb paresis, oculomotor weakness, arthralgias, cardiomyopathy
Treatment: penicillin or ceftriaxone
Most common in the winter months
Glucose decreased, protein elevated, CSF leukocytosis, and elevated opening pressure
Secondary to infection elsewhere, usually lungs
Mortality rate higher than in bacterial meningitis
Early treatment prevents poor outcome
Involves skull base (basal meningitis)
Treatment: isoniazid, rifampin, pyrazinamide, ethambutol
Treatment given for 18 to 24 months
INH depletes B6, therefore its use mandates replacement
CSF profile: slight increase in intracranial pressure (ICP), pleocytosis with lymphocytic predominance, increased protein, decreased glucose
CSF glucose should be normally 60% of serum glucose, usually 45 mg/dL or greater
CSF profile: lymphocytic pleocytosis, mild elevation in protein, normal glucose.
Peaks in the summer and fall seasons.
RNA viruses: enteroviruses, arboviruses, rhabdoviruses, arenaviruses.
DNA viruses: HSV-1, HSV-2, VZV, CMV, HHV-6.
Enteroviruses are the most common cause of viral meningitis.
Syndrome due to lesion of dominant parietal lobe (inferior parietal lobule)
Acalculia, agraphia without alexia, right and left confusion, finger agnosia (inability to distinguish fingers of the hand)
Anosognosia (unawareness of the contralateral side of the body) involves the nondominant parietal lobe
Central pontine myelinolysis
Due to aggressive correction of chronic hyponatremia
Symptoms: seizures, dysarthria, pseudobulbar palsy, dysphagia, hyperreflexia, quadriplegia, coma
Can also occur without symptoms
Diagnosis: confirm with MRI; CSF studies can show elevated myelin basic protein
In order to avoid this condition, correct sodium by 12 mmol/L in 24 hours
Thiamine (B1) deficiency
Opthalmoplegia, nystagmus, anisocoria, gait ataxia, confusion, coma, death
Confabulation, psychosis, amnesia
Mammillary bodies effected
With treatment symptoms of Wernicke involving the eyes improve; confusion improves to a varying degree; Korsakoff amnesia is left in about 80% of patients
Subacute combined degeneration of the spinal cord
Degeneration of posterior and lateral columns of the spinal cord
Decreased pressure, vibration, and touch sense
Weakness of the extremities, especially legs
Trinucleotide repeat (CAG) localized on chromosome 4.
Autosomal dominant disease with complete penetrance.
More common in males with symptoms beginning at age 35 to 40.
Abnormal gene products cause damage to inhibitory pathway between motor cortex and subcortical structures causing hyperkinesia and choreoathetosis.
Disease progresses to death within 20 years of symptom onset.
Trinucleotide repeat (GAA) located on chromosome 9.
Frataxin gene not expressed in this disease; frataxin is an iron storage protein that is not expressed.
Symptoms related to degeneration of spinal cord tracts, medulla, and deep nuclei → ataxia, dysarthria, areflexia.
Symptoms begin between 10 and 15 years and disorder is deadly by middle age (40-50).
A type of dementia associated with language dysfunction, intellectual loss, and memory loss.
Can be a rare cause of new onset auditory hallucinations.
Diagnosis based on history, physical examination, and brain imaging so as to exclude reversible causes of dementia.
Several genes associated with AD → the E4 allele of the apolipoprotein E (chromosome 19) is associated with 25% to 40% of all cases.
Degeneration of the nucleus basalis of Meynert causes decreased cholinergic input to frontal cortex.
Treatment: Tacrine (central acting acetylcholinesterase inhibitor) and donepezil (reversible acetylcholinesterase inhibitor) may slow cognitive decline.
Brain stem auditory evoked response (BAER)
Can be measured from several important components of the auditory pathway.
Seven peaks in BAER:
Wave 1: auditory nerve
Wave 2: cochlear nucleus
Wave 3: superior olive
Wave 4: lateral lemniscus
Wave 5: inferior colliculus
Wave 6: medial geniculate body
Wave 7: auditory radiations/cortex
BAER shows prolonged latency with structural lesion of the brain stem or auditory nerve.
BAER is abnormal in 90% of patients with acoustic schwannoma and usually consists of delayed conduction from the auditory nerve to the caudal pons (waves 1-3).
BAER is abnormal in 33% of MS patients and usually involves increased latency between waves 3 and 5.