-- Relaxants work, but are a poor choice in chronic disease (diazepam) because of abuse and addiction potential
What is Pseudotumor Cerebri?
aka Idiopathic Intracranial Hypertension
Usually seen in young obese women (90%)
-- Rare over the age of 45
Watch for other causes including vitamin A overuse, tetracycline and corticosteroid use and oral contraceptives
How do you diagnose Pseudotumor Cerebri?
Hallmark is headache and papilledema
Papilledema is unilateral > bilateral
-- Enlarged blind spot
-- Decreased peripheral vision
-- Ophthalmoplegia (due to CN VI problem) - there is weakness when the person looks to the sides
Otherwise nonfocal neuro exam
Diagnosis is clinical. Headache and papilledema are most common features
CT brain and MRI brain will be normal
-- LP: opening pressure is > 25 cm H2O
-- Can be 250 - 500 cm H2O
Treatment of Pseudotumor Cerebri
- acetazolamide 250 - 500 mg tid
- furosemide 40 - 80 mg/d
- prednisone 40-60 mg/d
- dexamethasone 6-12 mg/d
- Lumboperitoneal shunt
- Optic nerve sheath fenestration
Most cases resolve in 6-12 months
Treatment of temporal arteritis
Prednison 1 mg/kg/day ( usually 40-60 mg/day)
Often needed for 6-24 months
ESR will decrease with treatment
What is Coital (Exertion) Headache?
Affects males >females
Treatment: indomethacin, propranolol, ergotamines given before sexual activity
Evaluation should exclude SDH, aneurysm
What is Trigeminal Neuralgia?
Also called tic douloureaux
90% occur in people over 40 years old
Pain is lancinating
- affects the V2 >V3 distrubution of the trigeminal by touching the face, toothbrushing, chewing, yawning
If < 40 years old, think MS
Treatment for Trigeminal Neuralgia
- Carbamazepine, oxcarbazepine, phenytoin, and gabapentin (also probably pregabalin)
Glycerol injection in the trigeminal cistern
-- Radiofrequency rhizotomy - destroy the pain fibers
-- Microsurgery to reposition a blood vessel that crosses the 5th cranial nerve
What is Normal Pressure Hydrocephalus?
Wobbly, wet, weird
Neuroimmaging (NPH vs. ex vacuuo)
LP - diagnostic and "therapeutic"
Medical: Acetazolamide (decrease CSF production)
Surgical: V-P shunt
What is Pick's Disease?
Onset and progression is similar to Alzheimer's
Probably is about 2% of people with Dx'd Alzheimer's
Radiologically: some have identified frontal/temporal atrophy greater than the rest of the brain
Diagnosis is made at autopsy -- Pick bodies are intranuclear inclusions (argentophilic = silver staining)
What is the Treatment of Alzheimer's and Pick's?
- tacrine - watch LFTs for hepatotoxicity
- donepezil, rivastigmine, galantamine - no hepatotoxicity
Glutamate regulation - memantine
What is Creutzfeldt-Jacob Disease?
Similar to Kuru
Rapidly progressive (acute or subacute)
Associated signs are:
- Myoclonus (startle)
- Cerebellar signs
- EEG shows 1 Hz periodic discharges
- Biopsy shows spongiform changes
What is the difference between Peripheral and Central Vertigo?
Central: Vertical nystagmus, normal calorics, mild dizziness, no nausea, no hearing loss, CN abnormality is present
Peripheral: Horizontal nystagmus, abnormal calorics, severe dizziness, (+) nausea, (+) hearing loss, no CN abnormality
What is Neuronitis?
A type of Vertigo
Sudden prolonged vertigo
-- lasts hours to weeks
-- Associated with nausea/vomiting, dysequilibrium
Nystagmus - horizontal
Calorics are abnormal
Treatment is symptomatic
What is Benign Positional Vertigo?
Vertigo is precipitated by movements of the body or head
Nystagmus is usually horizontal, transitory and fatigable, brought out with Nylan-Barany maneuver
No tinnitus or hearing loss
Treatment is symptomatic
What is Meniere's vertigo?
Onset in 20s and 30s
Episodic and intense vertigo (min to h)
Associated with hearing loss and tinnitus
Relapsing and remitting
Caused by increased endolymph
What is the treatment for Meniere's?
Decreased salt intake
Diuretics -- thiazides, acetazolamide
Surgery - endolymph shunt
Report of deja vu by a seizure patient point toward what type of seizure?
What is absence seizure?
3 Hz spike on EEG
What is myoclonic seizure?
sudden, brief, shock-like muscle contractions, which may be focal, multifocal, or generalized in distribution
What are atonic seizures?
Atonic seizures consist of a sudden loss of muscle tone that causes a drop to the ground and carries a high risk of injury
How do 'Simple Partial Seizures' manifest?
may manifest as an “indescribable feeling,” a rising epigastric sensation, an unpleasant olfactory or gustatory sensation, a psychic phenomenon (such as déjà vu), or autonomic disturbances (such as flushing, diaphoresis, or lightheadedness).
A period of postictal confusion lasting minutes to hours is common.
What type of seizure usually manifest "complex automatisms?"
Complex partial seizures
What anti-epileptic medications work for partial seizures?
All of them except ethosuximide
In young women with seizures, what medication should you avoid due to high risk of birth defects?
What are the medications for Generalized T-C Seizures?
"top level valley zones feel lame"
valproic acid (Depakote)
What anti-seizure medications can make Generalized Tonic-Clonic Seizures worse?
"to car garage"
What is Juvenile Myoclonic Epilepsy?
an idiopathic generalized epilepsy syndrome characterized by both myoclonic seizures on awakening and generalized tonic-clonic seizures.
Onset is usually in adolescence
Seizures may be provoked by sleep deprivation, alcohol, or flickering lights.
The EEG demonstrates generalized spike and wave discharges of 4 to 6 Hz.
lamotrigine and valproic acid considered first-line treatments.
the susceptibility to seizure persists over the patient’s lifetime, and 75% to 100% of patients will relapse if medication is withdrawn -- so do not d/c antiseizure meds
What anti-seizure medications can reduce the effectiveness of oral contraceptives?
Inducers such as: p-cop
What antiseizure medication can cause irregular menses?
Remember that it also has a tetratogenic effect
What is a risk with long-term use of phenytoin?
When do neurological injury occur during seizure?
after 60 minutes in status
How do you use Fosphenytoin?
Monitor BP, ECG during infusion
20 mg PE/kg IV -- Max rate is 150 mg/min
If still seizing, give 5-10 mg PE/kg IV
If no response, go to next medication
What is the maximum rate of phenytoin infusion?
max rate is 50 mg/min
What is a risk of phenytoin infusion?
Describe the treatment algorithm for Status Epilepticus management
0-5 Diagnose, serum tests
6-10 D50 + thiamine; lorazepam
10-20 fosphenytoin 20 mg PE/kg
20-30 fosphenytoin 5 - 10 mg PE/kg
30-60 phenobarbital 20 mg/kg; intubation
> 60 Additional phenobarbital or iatrogenic coma
What is Narcolepsy?
It is clinical diagnosis comprising of the quartet:
1. Sleep attacks (irresistible)
3. Sleep paralysis
4. Hypnagogic hallucinations
Evaluation: EEG ma show sleep onset REM
What is the treatment for Narcolepsy?
Monoamine oxgenase (MOA) inhibitors
What is recommended for Cataplexy?
Tricyclic antidepressants (imipramine)
Discuss Ischemic stroke of the ACA
- contralateral monoparesis (foot and leg)
- contralateral grasp
- abulia (abnormal ability to act or make decisions)
- urinary incontinence
What are the clinical features of a stroke of the PCA?
Color anomia (inability to name an object based on the color)
Mild contralateral hemiparesis
Mild contralateral heminanesthesia
What is the clinical feature of a Vertebrobasilar stroke in the Pontine area?
- Conralateral hemiplegia and hemianesthesia
- Ipsilateral ataxia
- Ipsilateral 7th
- Internuclear ophthalmoplegia
Internuclear ophthalmoplegia indicates a lesion where?
MLF (medial longitudinal fasciculus)
What are the features of a cerebellar infarct?
Nausea and vomiting
Edema can cause brainstem compression.
Always admit and observe.
Surgical decompression if edema is severe
What are the clinical features of Lateral Medullary Syndrome?
Injury located in the lateral medulla
Ipsilateral Horner's (ptosis, miosis, anhydrosis)
Ipsilateral facial weakness
Contralateral decreased pain and temperature
What are clinical features of Lacunar type Ischemic stroke?
Pure motor or sensory symptoms
Clumsy hand-dysarthria syndrome
Interventions in a pt with cerebral edema?
Steroids are ineffective
Osmotic agents (mannitol)
What is neuroleptic malignant syndrome?
results from dopamine D2 receptor antagonism
Muscular rigidity and altered mental status occur early, followed by hyperthermia, hypertension, tremors, fever, dysphagia, diaphoresis, myoclonus, and autonomic dysfunction
can result from administration of neuroleptic agents, such as haloperidol
Acute and abrupt withdrawal of antiparkinsonian medications may also precipitate the syndrome and may be the cause for this patient’s symptoms
What is the serotonin syndrome?
caused by use of selective serotonin reuptake inhibitors.
Distinguishing features include shivering, hyperreflexia, myoclonus, and ataxia.
The diagnosis of serotonin syndrome is less likely in the absence of evidence that the patient was taking a selective serotonin reuptake inhibitor drug.
What is malignant hyperthermia?
an inherited skeletal muscle disorder characterized by a hypermetabolic state precipitated by exposure to volatile inhalational anesthetics (halothane, isoflurane, enflurane, desflurane, sevoflurane) and the depolarizing muscle relaxants succinylcholine and decamethonium.
Findings include sustained muscle contractions with skeletal muscle rigidity and masseter muscle spasm, tachycardia, hypercarbia, hypertension, hyperthermia, tachypnea, and cardiac arrhythmias
What is the treatment for neuroleptic malignant syndrome?
If the syndrome occurs when antiparkinsonian drugs are discontinued, the drugs should be restarted immediately.
If neuroleptic drug therapy is required in a patient who developed the syndrome, the drug or class of drugs should be changed or the offending drug should be started at a lower dose.
drug therapy with dantrolene and/or bromocriptine has been reported to decrease mortality and duration of symptoms. Therapy is started with intravenous dantrolene sodium and oral bromocriptine. When symptoms begin to resolve, dantrolene is discontinued and bromocriptine is continued for 10 days or up to 3 weeks if parenteral neuroleptic agents were used. Benzodiazepines can also be used.
Common locations of hemorrhagic stroke
Amyloid, bleeding diatheses
What is the clinical presentation with hemorrhagix stroke in the Putamen?
Clinically similar to ischemic MCA infarct
Presentation of hemorrhagic stroke presentation in the thalamus
Sensory symptoms > motor
Difficulty with up gaze
Skew deviation is common
Presentation of hemorrhagic stroke in the Pons
Pinpoint pupils, reactive
Presentation of hemorrhagic stroke in the cerbellum
Similar to cerebellar ischemic infarct
Clinical features of subarachnoid hemorrhage
"Worst headache (thunderclap)"
May transiently lose consciousness
For SAH evaluation, describe evaluation process
CT+ in 85%
LP+ in 95%
Angiogram is gold standard
So if have strong clinical suspicion and CT and LP are (-), do angiogram
MRA also used but cannot detect small aneurysm, so angiogram still the standard
What medication is best for treating focal neurologic defecits that occur a few days after SAH?
This is likely vasospasm
What radiologic study is best for evaluating brain mass?
MRI >> CT
What malignancies commonly metastasize to the brain?
From most to least common:
What are the clinical features of Meningeal Carcinomatosis? Prognosis?
Multiple cranial neuropathies
Focal neurologic deficits -- 33% are associated with parenchymal mets
Prognosis is poor, survival is < 6 months
What neoplasms commonly metastasize to the meninges?
Small cell lung ca
How is Multiple Sclerosis diagnosed?
It is a clinical diagnosis, and tests are used to confirm it.
- History of two (or more) separate "attacks"
- Clinical evidence of two lesions (or more)
- MRI (characteristic lesions)
- LP (*oligoclonal banding)
- Evoked potentials
*oligoclona bands are proteins found in the CNS but not in blood. This indicates something going on in the CNS. Not specific to MS
What is your differential diagnosis with MS?
ADEM (acute disseminated encephalomyelitis)
Collagen vascular disease
Treatment for acute exacerbation of MS?
Treatment for chronic MS?
Synthetic protein polymer called copolymoer I
What do you use to treat spasticity caused by MS?
baclofen or tizanidine
What 3 symptoms would you expect with MS?
Expect this three because MS is an upper motor neuron disease
Differentiate between Pyramidal system vs. Extrapyramidal system
Pyramidal system: corticospinal tracts
Extrapyramidal system: basal ganglia and sustantia nigra influences motor control
How do you treat movement disorders due to "underactivity" of dopamine?
Where is the lesion in a pt with Bitemporal Hemianopsia?
optic chiasm lesion
Where is the lesion on Homonymous Hemianopsia?
lesion of the optic tract
loss of vision on contralateral to the injury
What is coricobasal degeneration?
a rare, sporadic, degenerative condition.
Clinical manifestations include gait impairment, dystonia, myoclonus, tremor, and slurred speech.
Neurologic examination shows asymmetric rigidity, bradykinesia, and dystonia in most patients.
The presence of alien limb phenomenon, by which an extremity moves independent of conscious voluntary control, in conjunction with these signs suggests corticobasal degeneration.
Tremor is less frequently seen than in patients with Parkinson disease.
Diagnosis is based on clinical features, and therapy is symptomatic, similar to that for progressive supranuclear palsy.
What are first line agents for more serious Essential Tremor?
Patients with mild tremor may not require treatment. For more serious tremors, propranolol and primidone are first-line agents
What are the recommended treatments for acute dystonic reaction? How long would you continue treatment?
Oral anticholinergic medications are continued for 48 to 72 hours to prevent relapse.
How do you evaluate a suspected spinal dural a-v fistula?
Dural arteriovenous fistula should be suspected in any patient with a chronic or subacute progressive myelopathy with episodes of more rapid, stepwise clinical deterioration.
If epilepsy is not controlled with the first antiepileptic drug, what is the next intervention?
When patients are unsuccessfully treated with a first antiepileptic drug, the recommendation is to try a second antiepileptic drug as monotherapy. It is generally recommended to gradually transition between drugs, titrating the new drug to therapeutic range before tapering off the initial agent.
How do you treat malignant brain edema in a patient with ischemic stroke? Who is at risk for brain edema? What reduces morbidity and mortality in a patient with malignant brain edema? What is the role of intra-arterial thrombolysis? What is the role of endovascular mechanical clot-retrieval devices?
Young patients with major infarctions affecting the cerebral hemisphere or cerebellum have a heightened risk of brain edema and increased intracranial pressure.
Decompressive hemicraniectomy for malignant stroke reduces morbidity and mortality.
Intra-arterial thrombolysis is an option for the treatment of selected patients who have had a major stroke within the past 6 hours due to occlusion of a major intracranial artery.
The endovascular mechanical clot-retrieval device has been used to extract thrombi from occluded intracranial arteries. However, guidelines recommend that such a device must be used within 8 hours of a stroke.
What are potential adverse effects of dopamine agonist medications?
Patients who are initiated and maintained on dopamine agonist medications to control Parkinson disease should be warned about the potential for developing abnormal, compulsive behaviors, such as excessive gambling, excessive shopping, and hypersexuality
How do you manage a single unprovoked seizure?
Unless special circumstances exist, drug therapy is generally not started in patients with a single unprovoked seizeure.
The risk of recurrence is greatest in patients with status epilepticus on presentation, with an identifiable underlying neurologic cause, or with abnormal results on an electroencephalogram (EEG)
If a second seizure occurs in the future, the recurrence risk is greater than 60%, and antiepileptic medical therapy should be recommended at that time.
Patients with a partial seizure who are age 65 years or older or who have a family history of epilepsy may also be in a higher-risk category.
Menstrually related migraine How do you treat it? When should you avoid use of oral contraceptive therapy?
There is evidence that supports the use of mefenamic acid for perimenstrual prophylaxis, with treatment starting 2 days prior to the onset of flow or 1 day prior to the expected onset of the headache and continuing for the duration of menstruation.
The use of combined oral contraceptive therapy (estrogen plus progestin) is contraindicated in patients with a history of migraine with aura.
Women with migraine with aura are at a two-fold increased risk of ischemic stroke, ischemic myocardial infarction, and venous thromboembolism.
The risk of stroke is increased further, up to eight-fold, in women with migraine with aura who use combined oral contraceptive pills.
Small-fiber peripheral neuropathy What is it? How do you evaluate for it?
It is a distal sensory loss with normal reflexes and muscle strength. Pt usually c/o burning distal extremity.
The most common identifiable cause of small-fiber peripheral neuropathy is diabetes or impaired glucose tolerance.
Workup will start with a glucose tolerance test which is more sensitive and can detect patients with diabetes and a normal fasting plasma glucose level.
Approximately 30% of patients with normal fasting plasma glucose levels will have an oral glucose tolerance test diagnostic for diabetes.
A result of 140 mg/dL to 199 mg/dL (7.8 mmol/L to 11.0 mmol/L) from this test establishes a diagnosis of impaired glucose tolerance, and a result of 200 mg/dL (11.1 mg/dL) or greater is diagnostic of diabetes
What is Charcot-Marie-Tooth disease?
A hereditary neuropathy with pt showing high arches, hammertoes, peripheral neuropathy.
Patients with this disorder typically do not have neuropathic pain, and many patients also do not have sensory symptoms, despite having sensory loss on examination
Cerebral venous sinus thrombosis What is the usual clinical presentation? What are the major risk factors?
May present with signs and symptoms of intracranial hypertension, such as headache, papilledema, and visual problems; focal neurologic findings or seizures; and mental status changes, stupor, and coma.
Major risk factors for cerebral venous sinus thrombosis in adults include conditions that predispose to spontaneous thromboses, such as inherited or acquired thrombophilia, pregnancy, oral contraceptive use, malignancy, sepsis, and head trauma.
Magnetic resonance venography can readily detect obstruction of the venous sinuses by a thrombus and the damage to the brain caused by the resultant increased pressure and so is the most appropriate next diagnostic test.
How do you differentiate pseudorelapses from true relapses in multiple sclerosis?
Pseudorelapses in MS are the worsening of neurologic symptoms because of another cause, such as a systemic infection requiring antibiotic treatment or supportive care, and should be differentiated from true relapses, which may require costicosteroid treatment.
Pseudorelapses are usually caused by systemic infections or medications.
Pseudorelapses need to be differentiated from true MS relapses, which are not associated with systemic symptoms, such as fever
How do you evaluate the risk of seizure recurrence after a generalized tonic-clonic seizure?
The presence of Todd paralysis predicts a greater risk of future seizures in this patient
Many historical or examination findings predict a higher risk of recurrence, including presentation in status epilepticus, age greater than 65 years, known underlying neurologic disorder(s) or structural lesion(s), and Todd paralysis
Partial-onset seizures are also more likely to recur, perhaps because of the increased likelihood of there being an underlying causative structural lesion.
Todd paralysis is a transient unilateral or focal weakness often seen after partial seizures, with or without secondary generalization. Todd paralysis or other focal abnormalities on the neurologic examination predict a greater risk of a future seizure.
Patients with an electroencephalogram or head imaging scan showing a potentially epileptogenic lesion are also in the higher-risk category.
When do you initiate anti-seizure meds?
The ff. are indicators of greater risk of recurrence:
Family history of epilepsy
Age greater than 65 years
Abnormal finding on neurologic exam
Abnormal EEG or head MRI
Patients who have had two or more seizures, unless the seizures were widely spaced in time (years apart0 or were nondisabling, simple sensory events
Stroke thrombolysis What is the window for intravenour rtPA When is intra-arterial administratio of rtPA indicated, and what is the time frame allowed? How about for stroke patients on warfarin, what is the INR threshold allowed, or not allowed? What do you do with aspirin for patients who are eligible for thrombolysis?
The window for intravenour rtPA is within 3 hours from the onset of symtoms.
Intra-arterial administration of rtPA is indicated for selected patients with acute stroke who have major intracranial artery occlusion within 6 hours of symptom onset and who are not otherwise candidates for intravenous administration of rtPA.
An INR less than or equal to 1.7 is required for rtPA administration in a patient on anticoagulation.
Aspirin is indicated for acute ischemic stroke in patients who are not eligible for rtPA. For patients with acute stroke who are eligible for thrombolysis, aspirin should be withheld in the ER and for 24 hours after rtPA administration.
How do you treat metastatic epidural spinal cord compression?
The best treatment is intravenous dexamethasone followed by decompressive surgery and radiation therapy.
How do you evaluate for suspected Lambert-Eaton myasthenic syndrome?
Measurement of voltage-gated P/Q-type calcium channel antibody level
How do you treat memory loss in a pt with dementia?
First-line pharmacotherapy is an acetylcholinesterase inhibitor of which there are currently three: donepezil, rivastigmine, and galantamine. D-R-G
Memantine is also used to treat Alzheimer dementia, but only in patients with moderate to severe impairment.
In patients with severe dementia, memantine can be used alone or added to an acetylcholinesterase inhibitor.
Give three examples of acetylcholinesterase inhibitors. What is their indication?
donepezil, rivastigmine, and galantamine
Indicated tor mild to moderate dementia (MMSE score21-25)
What is a cluster HA? Describe its classic presentation. What are the cardinal symptomsof a cluster HA?
A cluster headache is an excruciating unilateral headache of extreme intensity, with a typical duration of 15 minutes to 3 hours.
Its pain is lancinating or boring in quality and is located behind the eye (periorbital) or in the temple, sometimes radiating to the neck or shoulder.
The cardinal symptoms of a cluster headache attack are ptosis (drooping eyelid), conjunctival injection (redness of the conjunctiva), lacrimation (tearing), rhinorrhea (a runny nose), and, less commonly, facial blushing, swelling, or sweating. These features are known as the autonomic symptoms. The neck is often stiff or tender in the aftermath of a headache, with jaw or tooth pain sometimes present.
How do you treat fatigue relate to MS?
Amantadine has been shown in several small placebo-controlled studies to reduce MS-related fatigue.
How do you treat hypertension in a patient with ischemic stroke who has received recombinant tissue plasminogen activator, intravenously?
In a patient with ischemic stroke treated with recombinant tissue plasminogen activator (rtPA), systolic blood pressure should be kept below 180 mm Hg and diastolic below 105 mm Hg for 24 hours after rtPA treatment; intravenous labetalol or nicardipine can best achieve this goal.
What is it characterized by? What is the treatment?
It is a potentially life-threatening neurologic emergency characterized by muscle weakness severe enough to necessitate intubation, typically requires plasma exchange therapy.
Myasthenic crisis is a potentially life-threatening
neurologic emergency characterized by weakness that is severe enough to necessitate intubation. Electromyographic studies, including repetitive stimulation of motor nerves, are indicated to establish a diagnosis of myasthenia gravis.
What causes it? Epidemiology? Signs and symptoms? Diagnostic evaluation? What is the role of Pyridostigmine?
Myasthenia gravis is caused by an immune-mediated attack on postsynaptic neuromuscular junctions. There are two recognized forms: ocular and generalized. The ocular disorder is less common and occurs in approximately 10% of patients. Some patients who initially have only ocular symptoms ultimately develop the generalized disorder. Generalized myasthenia gravis can affect either sex at any age but typically begins in the third and fourth decades in women and after age 50 in men.
Signs and symptoms in patients with ocular myasthenia gravis include fatigable blurring of vision, binocular diplopia, and ptosis. Patients with generalized myasthenia gravis typically report limb weakness, diplopia, slurred speech, dysphagia, and dyspnea. Findings on neurologic examination include ptosis, impaired ocular motility, and limb weakness that increases with repeated testing (fatigable weakness). Deep tendon reflexes and the sensory examination are normal.
Diagnostic evaluation includes screening assays for acetylcholine antibodies and, if negative, testing for muscle-specific kinase antibodies. erum thyroid-stimulating hormone levels should also be measured because of the association of myasthenia gravis with autoimmune thyroid disorders. EMG studies, including repetitive nerve stimulation studies, help to exclude other conditions and confirm the diagnosis. Single-fiber EMG is the most sensitive diagnostic test. Once the diagnosis is confirmed, a CT scan of the chest should be performed to exclude a thymoma, which is present in up to 15% of patients.
Pyridostigmine, an acetylcholinesterase inhibitor, is usually given as first-line therapy
Those with significant bulbar dysfunction, respiratory impairment, or severe limb weakness may benefit from plasma exchange
What is corticobasal degeneration?
Corticobasal degeneration is also a rare, sporadic, neurodegenerative disorder that can manifest as gait impairment, slurred speech, dystonia, or myoclonus. Parkinsonian signs are evident on examination and are characteristically asymmetric. Alien limb phenomena can occur, whereby an extremity will move independently of voluntary, conscious control
How do you diagnose frontotemporal lobar degeneration on the basis of progressive nonfluent aphasia?
In frontotemporal lobar degeneration, which encompasses the syndromes of progressive nonfluent aphasia, semantic dementia, and frontotemporal dementia, symptom onset is insidious and progression gradual over the course of several years.
Progressive nonfluent aphasia, semantic dementia, and frontotemporal dementia comprise the three main syndromes of frontotemporal lobar degeneration.
Symptom onset is insidious and progression is gradual over the course of several years.
How do you treat paroxysmal hemicrania?
Indomethacin is the treatment of choice for paroxysmal hemicrania.
What is paroxysmal hemicrania?
one of the trigeminal autonomic cephalalgias, which are characterized by pain referred to the first division of the trigeminal nerve and by accompanying cranial autonomic symptoms, including lacrimation and rhinorrhea. An attack of paroxysmal hemicrania has an intermediate duration (mean, 15 minutes) and an intermediate episodic frequency (mean, 11 per day). Treatment with indomethacin can immediately and completely resolve the headache. Usually, the response occurs within the first 48 hours after treatment is initiated.
How do you evaluate trigeminal neuralgia?
Because trigeminal neuralgia usually presents after age 40 years, its diagnosis in a younger patient should prompt an evaluation for secondary causes, such as multiple sclerosis, posterior fossa tumors, and vascular or aneurysmal compression of the trigeminal nerve.
Anyone with trigeminal neuralgia symptoms should have an MRI of the brain.
Up to 10% of patients with trigeminal neuralgia may harbor an intracranial lesion; therefore, an MRI should be obtained for every patient with trigeminal neuralgia, even those who respond to medication and whose examination findings are normal.
What is the drug of first choice for trigeminal neuralgia? What is the next intervention if no response to medical therapy?
All medications should be started at a low dosage and titrated slowly until the desired effect is achieved or dose-limiting side effects occur. Monotherapy is a treatment goal, but in some patients, combination therapy with two drugs used in smaller dosages may be more effective and better tolerated. The drug of first choice is carbamazepine or oxcarbazepine, but caution must be exercised in the use of these drugs in patients of Asian origin. Oxcarbazepine may be preferred because carbamazepine may cause hematologic and hepatic toxicity, although both drugs may cause symptomatic hyponatremia. Serum sodium concentration should be monitored during treatment with either of these medications.
Approximately 30% of patients with trigeminal neuralgia do not respond to medical therapy and may require a surgical or ablative procedure. Microvascular decompression is the definitive procedure for idiopathic trigeminal neuralgia, but it is often reserved for intractable cases because of the need for a craniotomy. MRI of the head with high-resolution images of the posterior fossa obtained to evaluate for vascular compression of the trigeminal nerve is recommended before surgery.
MS patients are advised to engage in physical activity. What physiologic phenomenon should you tell your patients who are starting their exercise routine?
Temporary re-emergence or worsening of neurologic symptoms with exercise (Uhthoff phenomenon) is common, and patients should be told that this physiologic phenomenon does not worsen nerve injury or trigger exacerbations.
What is the preventive therapy for neuromyelitis optica?
Preventive therapy for neuromyelitis optica involves immunosuppression with agents such as azathioprine or rituximab.
Define the term "secondary progressive course" in MS.
It is defined as the development of gradual neurologic worsening without remission. Typically, this manifests as a gradually worsening gait disorder. Documentation of objective decline for more than 1 year is required to confidently declare the onset of secondary progressive MS.
Define the term "gradually progressive disorder" of MS
Fifteen percent of patients with MS experience a gradually progressive disorder, rather than an attack, at disease onset.
Primary progressive MS develops at a later age (fifth to sixth decade) and follows an unremitting course that is remarkably similar to secondary progressive disease in pace and clinical phenotype.
What is Neuromyelitis optica?
Long considered an MS variant, NMO is now recognized as a distinct demyelintaing disease with a predilection for the optic nerves and spinal cord.
An MRI of the spinal cord typically showe extensive lesions in patients with NMO.
Can be distinguished from MS by the NMO-IgG autoantibody test.
How do you evaluate copper deficency myeloneuroathy?
B12 and copper deficiencies are associated with malabsorption syndromes related to gastric bypass surgical procedures and can cause anemia and syndrome of progressive myeloneuropathy.
What is the fist line treatment for Parkinson disease in patients younger than 65?
Dopamine agonist medications are used as first-line treatment of Parkinson disease in patients younger than 65 years, whereas levodopa is used in patients age 65 years or older.
Dopamine agonist medications, either pramipexole or ropinirole, are indicated for the initial treatment of the parkinsonian symptoms in this young patient with apparent Parkinson disease.
How do you treat eilepsy in a pt with liver disease?
For patients who should avoid hepatically metabolized antiepileptic drugs, either because of drug interaction or underlying liver disease, levetiracetam, gabapentin, and pregabalin can be used.
What is the treatment for primary progressive multiple sclerosis?
Fampridine is the only drug currently approved by the Food and Drug Administration for treatment of primary progressive MS and has been shown to improve walking in patients with MS. Fampridine is contraindicated in patients with a history of seizures or with moderate to severe renal impairment; its most commonly reported side effects include back pain, dizziness, insomnia, fatigue, and nausea.
When do you use plasma exchange in the treatment of MS?
Plasma exchange is often used in patients with a severe, fulminant attack of MS that does not respond adequately to corticosteroid treatment, is inappropriate for this patient's primary progressive MS.
How do you treat metastatic epidural spinal cord compression?
Epidural spinal cord compression usually requires immediate therapy with intravenous corticosteroids followed by decompressive surgery and radiation therapy.
Describe the presentation of cauda equina syndrome
Patients with cauda equina syndrome typically have bilateral leg pain, weakness, and areflexia. Bowel and bladder impairment can occur, and saddle anesthesia is expected in affected patients.
What is diabetic lumbosacral radiculoplexos neuropathy?
This neuropathy is a subacute, progressive disorder that causes asymmetric leg pain, sensory loss, and weakness. Weight loss of4.5 kg (10 lb) or more occurs in most affected patients. Many patients with this disorder are unaware that they have diabetes mellitus before the development of diabetic lumbosacral radiculoplexus neuropathy, and in most patients, glycemic control is not severely compromised. This disorder usually begins with unilateral leg pain followed by weakness and sensory loss, which spread to involve the contralateral leg nearly all the time. Weakness is often greatest proximally initially, but over time, diffuse weakness involving proximal and distal muscles ensues.
Patients typically present with weight loss, unilateral leg pain (often severe), numbness, proximal weakness, and atrophy. Signs and symptoms develop over weeks, may progress over several months, and may later involve the contralateral leg or even the arms.