Neuroplus Flashcards
Status epilepticus
Criteria
≥ 5 min of continuous seizures
OR ≥ 2 seizures with consciousness not being fully regained in the interictal period
Aetiology
Common causes are withdrawal from antiepileptic drugs, metabolic disturbances (e.g., hyponatremia), drug toxicity (e.g., tricyclic antidepressants), structural brain lesions/injury (e.g., tumors, trauma, stroke), and CNS infections.
Treatment
Treatment
Treatment of status epilepticus
Initial assessment and supportive treatment:
Place patient in recovery position to prevent injury.
Quick neurological examination (to determine type and cause of status epilepticus) and general medical evaluation (particularly airway, breathing, and circulation)
Establish secure IV access (two, if possible), collection of blood for routine blood tests (particularly electrolytes and glucose levels), toxicology screen, antiepileptic drug levels, and arterial blood gas (ABG) analysis
Supportive therapy as necessary (e.g., oxygen, glucose, thiamine naloxone )
Monitoring of vital signs: especially oxygen saturation (via pulse oximetry), blood pressure, cardiac action, and breathing
If patient does not regain consciousness after seizures stop or nonconvulsive status epilepticus is suspected → continuous EEG monitoring
If acute brain injury (e.g., intracerebral hemorrhage) is suspected → obtain a cranial CT
If CNS infection is suspected → conduct a lumbar puncture
Pharmacological interruption of seizures: initial treatment
First line: IV lorazepam; second line: IV diazepam or midazolam → if IV access is not possible or drugs are administered by someone who is not a medical professional → select another application form (e.g., rectal diazepam, buccal or intranasal lorazepam/midazolam)
If the patient does not respond within 1 minute → administer additional lorazepam (or a second-line benzodiazepine)
If the patient does not respond within another 10–20 minutes → saturation with fosphenytoin via separate access (alternatively: phenobarbital, levetiracetam, or valproate)
If seizure activity does not stop despite application of a benzodiazepine and a nonbenzodiazepine antiseizure drug → refractory status epilepticus
No later than 45–60 min after onset: continuous administration of anesthetics with intubation and ICU monitoring; e.g., thiopental, propofol, or midazolam
Nonbenzodiazepine therapy (to prevent recurrence): fosphenytoin or valproate
Prognosis
Mortality of ∼ 20% (in adults with first occurrence of GCSE)
Seizure disorders (epilepsy)
A seizure is irregular electrical activity in the brain caused by the hyperexcitability of neurons, especially in cortical areas. Hyperexcitability, in turn, is the result of altered cellular electrochemical properties, which may be caused, for example, by electrolyte imbalances. The etiology varies according to age. Seizures may be provoked by acute conditions (e.g., stroke, traumatic brain injury, alcohol withdrawal) or unprovoked, in which case they are indicative of epilepsy. The lifetime risk for experiencing at least one seizure is approx. 3% in the general population. Individuals who experience a single seizure do not necessarily have epilepsy. Seizures may be classified as generalized or focal. Generalized seizures arise from discharges in both hemispheres, whereas focal seizures begin with discharges in one hemisphere. Depending on the origin of the epileptiform discharge and the type of the seizure, various temporary motor, sensory, autonomic, or psychological symptoms may occur. However, the most frequent clinical presentation involves rhythmic twitching and loss of consciousness (tonic-clonic seizure). After a first seizure occurs, the likely cause must be determined based on medical history (evaluation of provocative factors and seizure type), laboratory tests, and imaging (to detect or rule out structural or metabolic causes). Electroencephalography (EEG) is used to establish the diagnosis. Important antiepileptic drugs are lamotrigine (first-line treatment in focal seizures), valproate (first-line treatment in generalized seizures) and ethosuximide (first-line treatment in typical absence seizures). With appropriate pharmacotherapy, the majority of patients remain seizure‑free in the long term. Epileptic seizures can evolve into ongoing seizure activity (status epilepticus), which is a potentially life‑threatening event and must be addressed as soon as possible (pharmacologic interruption of seizures with a benzodiazepine).
Also see the article on generalized epilepsy in childhood.
Seizures and Epilepsy
Seizure: abnormal, unregulated electrical activity of cortical neurons that results in transient changes in behavior and/or EEG findings
Epilepsy: a chronic neurologic disorder characterized by any of the following:
Two or more unprovoked seizures separated by more than 24 hours
One unprovoked seizure with an underlying predisposition to seizures (recurrence risk over the next 10 years that is similar to the recurrence risk after two unprovoked seizures)
Diagnosis of an epilepsy syndrome
Symptomatic epilepsy: epilepsy due to an identifiable condition (e.g., brain tumor, structural abnormalities of the brain) that causes an increased predisposition to seizures
Cryptogenic epilepsy: epilepsy due to an unknown cause (genetic association suspected)
Nonepileptic seizure: seizures that are provoked by acute conditions (e.g., intoxication, metabolic disturbances). See provoked seizure for details.
Epilepsy syndromes: epileptic disorders defined by a collection of characteristic clinical manifestations and other features (e.g., age of onset, brain lesions)
Epidemiology Prevalence: Infants: ∼ 100/100,000 Adults : ∼ 40/100,000 The elderly : ∼ 140/100,000 Risk of experiencing at least one seizure up to the age of 75 (in the general population): ∼ 3%
Aetiology
Epilepsy
Unprovoked seizure (epileptic seizure): due to a general increase in neuronal hyperexcitability
Genetic: genetic mutations of ion channels or transmitter receptors, chromosomal abnormalities
Cryptogenic (idiopathic)
Structural/metabolic: preexisting, chronic cerebral lesion of CNS abnormality (e.g., hypoxic-ischemic injury, PKU, tuberous sclerosis, congenital cerebral malformations)
Although these seizures are referred to as unprovoked, they may be triggered by certain provocative factors!
Provocative factors for epilepsy
Situational factors that can trigger epileptic seizures in epilepsy patients:
Excessive physical exertion
Sleep deprivation
Strobe light flashing
Loud music
Provoked seizures
Acute symptomatic seizures that are secondary to acute conditions:
Metabolic and electrolyte disturbances: hypoglycemia and hyperglycemia, hyponatremia and hypernatremia, hypocalcemia, uremia, thyroid storm, hyperthermia, water intoxication
Mass: brain tumors and metastases, hippocampal sclerosis
Withdrawal
Alcohol withdrawal (most common trigger in adults)
Noncompliance with anticonvulsant treatment
Medication withdrawal
Intoxication
Acute intoxication: cocaine, ecstasy, carbon monoxide poisoning, metal poisoning
Pharmacotherapy: amitriptyline, penicillin, maprotiline, lithium, lidocaine, antipsychotics, theophylline
Infections: encephalitis, brain abscess, meningitis, septic shock
Ischemia: stroke, perinatal injuries
Trauma: traumatic brain injury
Increased ICP and cerebral edema: eclampsia, hypertensive encephalopathy, cerebrovascular malformation
Fever in infants and children (see febrile seizures)
Withdrawal from ABBA (alcohol, benzodiazepines, barbiturates, antiseizure drugs) can cause seizures!
Common causes according to age group
-on word.
Classifications
1. Etiological classification (ILAE 2010)
-Genetic: without a discernible structural or metabolic cause
-Structural or metabolic: with a discernible structural or metabolic cause
-Unknown: no tangible cause (the cause is inferred from the clinical presentation and history)
2. Classification according to anatomical origin and clinical features
–>Focal seizures
Focal seizures without dyscognitive features
Focal seizures with dyscognitive features
–>Primary generalized seizures
Classic tonic‑clonic seizures (see grand mal seizure)
Further generalized seizures
Absence (see also generalized epilepsies in childhood)
Myoclonic (see juvenile myoclonic epilepsy)
Clonic seizures
Tonic seizures
Atonic seizures
Clinical features
Ictal phase
Sudden onset
Rapid progression of symptoms
Duration usually 1–3 minutes
Postictal phase
Varying degree of confusion, impaired alertness
Residual neurologic symptoms (e.g., Todd’s paralysis)
Duration usually minutes or hours
Symptoms of, e.g., CNS infection, stroke, intoxication, hypoglycemia, electrolyte disturbances → indicative of acute symptomatic seizure
Focal seizures
Focal seizures are more likely to be caused by focal structural abnormalities. Symptoms depend on the anatomical location of the lesion or disturbance within the brain.
Table on word
Ddx
Confirm a seizure
Patient history (from patient or witnesses): e.g., evidence of an aura, observation of typical symptoms (e.g., twitching), identification of provocative factors (e.g., sleep deprivation or unreliable intake of antiseizure medication )
EEG
–>During the seizure (ictal)
Epileptiform discharges (e.g., spikes, sharp waves, spike waves, and hypsarrhythmia) are usually detected.
If no epileptiform discharges are detected, diagnosis of pseudoseizures should be considered.
–>Between seizures (interictal)
Often normal findings (even after provocation via sleep deprivation, hyperventilation, or visual stimuli)
Possibly showing epileptiform activity (bursts of abnormal discharges featuring spikes and/or sharp waves)
Exclude an underlying condition -ECG: In every patient with loss of consciousness during an ictal event, cardiogenic causes (e.g., cardiac arrhythmias resulting in cerebral hypoxia) should be ruled out. -CT/MRI (with and without contrast): structural lesions (e.g., brain tumors) should be ruled out after a first seizure. -Laboratory tests Blood Glucose Electrolytes Prolactin Antiepileptic drug levels Toxicology screen CBC ESR Rapid plasma reagin Creatine kinase Renal and liver function tests -Lumbar puncture: cerebrospinal fluid analysis (e.g., if CNS infection is suspected)
The first focal seizure or the first focal seizure evolving into a generalized seizure in adults indicates a seizure of structural or metabolic origin and requires further evaluation!
ddx:
Psychological disorders:
Pseudoseizures: not true seizures; paroxysmal events that may clinically resemble epileptic seizures but have a psychiatric origin
Panic attacks
Psychogenic hyperventilation
Syncope:
Vasovagal syncope
Stokes-Adams attack
Carotid sinus syndrome
Cardiac failure
Orthostatic hypotension
Stroke (including transient ischemic attack )
Migraine
Sleep disorders
REM sleep behavior disorder
Narcolepsy
Breath-holding spell (benign condition)
Occurs in children (6 months–6 years )
Strong association with iron deficiency anemia
Clinical features
Episodes of prolonged expiratory apnea
Followed by loss of consciousness (syncope)
Transient paroxysms of cyanosis or pallor
Possibly generalized stiffness and jerky movements of the limbs (anoxic seizure)
Triggers: distress, strong emotions (e.g., anger, frustration) due to tantrums or injury
Diagnosis: based on typical presentation of BHS (no confirmatory test exists)
Further work-up is generally not necessary. Only if atypical presentation of BHS:
EEG to differentiate from epileptic seizures
ECG to rule out cardiac causes of syncope
Laboratory analysis: CBC, serum ferritin (rule out iron deficiency anemia)
Treatment: reassurance
Postictal disorientation is key to differentiating between seizures and syncope. Syncope may be accompanied by twitches; however, patients become completely reoriented after a few seconds!
Treatment
Acute management
Cardiopulmonary resuscitation (ABCs)
Avoid hazards
Monitor vital signs (especially oxygenation via pulse oximetry)
First seizure
Long-term medical therapy is usually not required, unless there are abnormalities seen on EEG or MRI (or the patient is in status epilepticus).
Remove cause or provoking factors (e.g., stop illicit drug abuse, treat underlying disorders).
Recurrent seizures
Medical therapy
Principles of treatment
In epilepsy, the seizure threshold is pathologically lowered → Antiepileptic drugs work by raising this lowered threshold and thus protecting against future seizures.
Criteria for the choice of antiepileptic drugs
Type of epilepsy (as per clinical evaluation or instrumental findings)
Patient age
Comorbidities and contraindications
Indications
Recurrent seizures (2 seizures/6 months) of unknown cause or with a cause that can not be eliminated
After the first seizure → only if neuroimaging or EEG show specific findings (e.g., hippocampal sclerosis, characteristic spike‑wave patterns).
Treatment options table on word
-Combination therapy
If possible, monotherapy should be maintained → increase dosage before initiating combination therapy
If combination therapy is administered, drugs from different classes and/or with different pharmacologic modes of action should be tried.
-Termination of treatment
To be evaluated on a case‑by‑case basis
May be considered if the patient has < 2 seizures/year, an inconspicuous provocation EEG, normal psychological findings, and no hereditary predisposition
Generally possible after 2–5 seizure‑free years with normal EEG results
Medications must be tapered cautiously.
Nonpharmacological therapy
-Indications: pharmacoresistance
-Surgery
–>Prerequisite: epilepsy that does not respond to medications → often epilepsies with structural origin (most commonly temporal lobe epilepsy)
–>Procedures
-Resection (surgical removal of pathological lesions)
–Resection of the anteromedial temporal lobe or of the amygdala and the hippocampus → in patients with temporal lobe epilepsy
–Resection of an entire hemisphere (hemispherectomy) → in patients with severe intractable seizures due to structural cerebral abnormalities confined to one hemisphere
Disconnection (surgical breaking of neuronal circuits)
-Stimulation techniques: vagus nerve stimulation, deep brain stimulation
-Dietary measures: ketogenic diet
It is necessary to distinguish between focal and primary generalized seizures in order to decide on the appropriate therapy!
Complications
-Status epilepticus
General complications of seizures and epilepsy
Acute
Hyperthermia, cardiorespiratory deficits, excitatory toxicity → potentially irreversible tissue damage (particularly to the CNS, for example in the form of cortical laminar necrosis), which in turn may also result in further seizures
Postictal transient anion gap metabolic acidosis with increased lactic acid and reduced serum bicarbonate → usually resolves spontaneously within 60–90 minutes after seizure activity stops
Physical trauma (e.g., posterior dislocation of the glenohumeral joint)
Long‑term
Associated psychiatric disorders (e.g., anxiety, depression and risk of suicide, cognitive impairments)
Psychosocial issues (e.g., problems at the workplace).
Prognosis
Risk of seizure recurrence
Overall risk of recurrence
After the first unprovoked seizure (within 2 years): ∼ 40%
After the second unprovoked seizure (within 1 year): ∼ 60%
After a single tonic‑clonic seizure: ∼ 40%
In genetic epilepsy: ∼ 25–30% (within 3 months)
Patients with normal EEG results: ∼ 15% (within 1 year)
Patients with abnormal EEG results: ∼ 40% (within 1 year)
Impact of medical therapy
Immediate initiation of pharmacotherapy after a first unprovoked seizure lowers risk of recurrence in the short term (within 2 years) but does not significantly improve long‑term risk.
Approx. 80% of epilepsy patients who are treated with antiepileptic drugs remain seizure‑free for extended periods (3–5 years).
Tension-type headache Dr Deac PIMP
Tension-type headache (TTH) is a primary headache disorder and the most common type of headache overall. Tension-type headaches are characterized by a dull, nonpulsating, band-like pain that is often bilateral. Autonomic symptoms like photophobia, phonophobia, or nausea are usually not present. Depending on the frequency and duration of episodes, tension-type headaches are classified as episodic or chronic. Infrequent episodic tension-type headaches are treated with NSAIDs, while chronic and frequent episodic forms may benefit from prophylactic amitriptyline. Nonpharmacological treatment options include lifestyle modification (e.g., stress reduction) and cognitive behavioral therapy.
Epidemiology
Most common type of headache
∼ 70% of primary headaches
∼ 50% of the population will have had at least one episode in their lifetime.
Sex: ♀ > ♂
Peak incidence: 30–40 years
Ethnicity: increased prevalence in white populations.
Aetiology
The exact pathophysiology of tension headaches remains unknown.
Exacerbating factors: fatigue, lack of sleep, poor posture, anxiety, stress, depression.
Clinical features
Episodic nature
Headaches last 30 minutes to a couple of days. [8]
Holocranial or bifrontal, band-like headache (mild to moderate intensity)
Dull, pressing, nonpulsating (“vice-like”) quality
Headache does not increase with exertion.
Maximum of one autonomic symptom (nausea, phonophobia, or photophobia)
No vomiting or aura
Palpation of muscles of the head may reveal increased pericranial tenderness.
Investigations
Tension-type headache is primarily a clinical diagnosis based on a history of typical features and normal neurological examination. Severe underlying conditions should be ruled out (see red flags for headache and “Diagnostics” in headache). A headache diary can be helpful to establish the diagnosis and guide management.
Diagnostic criteria for tension-type headaches
At least two of the following:
Dull, pressing, nonpulsating quality
Mild to moderate intensity
Bilateral
No increase in intensity with exertion
Not better explained by any other headache disorder
Categorized into three entities (which guide treatment); all criteria have to be fulfilled for the diagnosis
ddx:
Treatment:
Both pharmacologic and non-pharmacologic strategies can be used for the treatment of tension-type headache. In addition, any underlying conditions (e.g., depression) should be identified and treated.
Pharmacological therapy
Episodic tension-type headache: NSAIDs (e.g., ibuprofen, aspirin) or acetaminophen
Chronic tension-type headache and frequent episodic type: consider prophylactic therapy (e.g., with amitriptyline).
Non-pharmacological therapy: Consider if there is a significant decrease in patient’s quality of life.
Lifestyle and behavioral modification (e.g., exercise, weight reduction)
Psychobehavioral treatments (e.g., cognitive-behavioral therapy, relaxation training)
Avoid prolonged use (> 15 days/month) of NSAIDs for chronic tension headache, as this may cause medication overuse headaches.
migraine
Migraine is a primary headache characterized by recurrent episodes of unilateral, localized pain that are frequently accompanied by nausea, vomiting, and sensitivity to light and sound. In approximately 25% of cases, patients experience an aura preceding the headache, which involves reversible focal neurologic abnormalities, e.g., visual field defects (scotomas) or paresis lasting less than an hour. Migraine is a clinical diagnosis and imaging is generally not indicated. Treatment of attacks consists of general measures (e.g., minimizing light and sound) together with administration of nonsteroidal anti-inflammatory drugs (e.g., aspirin) and antiemetics (e.g., prochlorperazine) if nausea is present. In severe cases, triptans may be added. Prophylactic treatment (e.g., beta blockers) may be indicated if migraines are especially frequent or long lasting, or if abortive therapy fails or is contraindicated.
Epidemiology
Prevalence: ∼18% of females and ∼6% of males
Peak incidence: 30–40 years
Migraine is the second most common type of headache.
Aetiology The exact pathophysiology is unclear. Genetic predisposition Potential triggers Emotional stress Weather changes Certain food and beverages: alcohol, nicotine, citrus fruits, dairy products, food containing tyramine (e.g., chocolate, red wine) Poor sleeping habits Hormonal changes in women: menstruation, hormone intake (oral contraceptive pills).
pathophysiology
The pathophysiology of migraine is not fully understood. Different aspects contribute to the development and severity of migraine, such as
Vascular dysregulation: vasodilation appears to play a role and there is an association between migraine and disorders with generalized vasospasms
Dysregulation of pain sensitization in the trigeminal system
Cortical spreading depression: continuously spreading depolarization of neuronal cells in the cortex,
Clinical features
Migraine is characterized by recurrent attacks and may occur with aura (∼ 25% of cases) or without aura (∼ 75% of cases). A typical migraine attack passes through four stages, and the aura (if present) typically occurs before the headache. However, migraine patterns may differ and not follow the characteristic stages.
1. Prodrome (facultative)
24–48 hours before the headache starts
Excessive yawning
Difficulties with writing or reading
Sudden hunger or lack of appetite
Mood changes
2. Aura
Paroxysmal, focal, neurologic symptoms that precede (or, in some cases, occurring during) the headache.
Typical aura [4][5]
Visual disturbances; sensory and/or speech symptoms (positive and/or negative )
Scintillating scotoma: an arch-shaped scotoma that starts centrally and shifts peripherally; appears for ∼ 15–30 minutes
Central scotoma
Flashing lights
Distorted color perception
Fortification spectra: star-like, zigzag figures
Impaired sensibility, paresthesia
Aphasia
No motor symptoms
Develops gradually
Completely reversible
Symptoms last ≤ 60 minutes each
Atypical aura
Paresis
Dizziness
Persistent or long-lasting symptoms
3. Headache
Localization
Typically unilateral, but bilateral headache is possible
Especially frontal, frontotemporal, retro-orbital
Duration: usually 4–24 hours; rarely over 72 hours
Course: progression of pulsating, throbbing, or pounding pain
Exacerbated by physical activity
Accompanying symptoms: photophobia, phonophobia, and nausea/vomiting
4. Postdrome (facultative)
Feeling of exhaustion or euphoria
Muscle weakness
Anorexia or food cravings
The typical migraine headache is “POUND”: Pulsatile, One-day duration, Unilateral, Nausea, Disabling intensity.
Diagnostics
Migraine is a clinical diagnosis based on history and physical examination. The most important step is to exclude red flags for headache that suggest a secondary headache (e.g., infection, hemorrhage, intracranial mass) and require more exhaustive investigation (e.g., imaging). Suspect a primary headache when no red flags are identified, and confirm the diagnosis using the diagnostic criteria for migraine.
Migraine is a clinical diagnosis that is based on patient history and physical examination!
Diagnostic criteria table on word
Imaging
Neurological imaging is not routinely indicated for uncomplicated migraine.
Indications
Clinical features suggest a secondary headache (see red flags for headache and high-risk headache).
Migraine with the following characteristics: [10]
Unusual, prolonged, or persisting aura
First episode of brainstem aura, hemiplegic migraine, retinal migraine, aura without headache
Change in baseline migraine clinical features (frequency, severity, aura)
Consider in first migraine
Procedure: MRI is preferred over CT (except in emergency settings if there is suspicion of a vascular hemorrhagic event).
See imaging for headaches
Findings [10]
Typically normal
Non-specific white-matter changes may be seen.
Ddx:
See differential diagnoses of headache.
Paroxysmal hemicrania
Medication overuse headache
Treatment
Abortive therapy
All patients
Limit stimuli (i.e., light, loud noises) and activity.
Start abortive treatment as soon as possible.
Treat nausea/vomiting, if present.
IV fluids
Parenteral antiemetics [12]
Metoclopramide
Prochlorperazine
Consider extrapyramidal syndrome (EPS) prophylaxis with diphenhydramine. [13][14][15]
Mild to moderate headache [12][4]
First-line treatment consists of NSAIDs, acetaminophen, acetylsalicylic acid, or combinations including caffeine.
If tolerating PO, consider one of the following:
Ibuprofen
Aspirin
Acetaminophen
Acetaminophen/aspirin/caffeine
If nausea/vomiting are present, consider one of the following:
Ketorolac
Diclofenac
Second-line: proceed to “Moderate to severe headache” below
Children: ibuprofen and family counseling
Moderate to severe headache [12][4]
Start a migraine-specific agent: triptans (e.g., sumatriptan) or ergotamine (do not combine these agents!)
First-line: oral or parenteral triptans
If tolerating PO, consider one of the following:
Sumatriptan-naproxen
Zolmitriptan
If nausea/vomiting are present or there is a higher analgesic requirement, consider one of the following:
Sumatriptan
Zolmitriptan
Second-line: consider one of the following
A parenteral ergotamine (e.g., dihydroergotamine)
Butorphanol
Consider in patients who cannot receive triptans or ergotamines.
Use with caution due to frequent side effects , risk of rebound, and medication dependency.
Consider recurrence prevention with dexamethasone. [16]
Do not combine triptans and ergotamines (within a 24-hour period) because of potentially dangerous drug interactions (e.g., coronary vasospasm and serotonin syndrome).
table on word
Triptans and ergotamine have severe pharmacological interactions (e.g., coronary spasm, serotonin syndrome) with one another and with other drugs (e.g., SSRI, macrolides). ALWAYS take a detailed history of the patient’s usual and recent medications before selecting a drug.
T
riptans and ergotamines are contraindicated in pregnancy.
A SUMo wrestler TRIPs ANd falls on his head: SUMaTRIPtANs are used for headaches (cluster and migraine).
Prophylactic therapy
Nonpharmacological [19]
Lifestyle modifications
Exercise in moderation
Maintain a healthy diet
Identify and try to avoid potential triggers
Follow a regular sleeping schedule
Other: There is some evidence that the following nonpharmacological interventions have some benefits for patients with migraine
Noninvasive neuromodulation
Behavioral therapy
Relaxation techniques
Biofeedback
Pharmacological [20]
Indications [21][22]
≥ 2 attacks/month that produce disability that lasts ≥ 3 days
Severe disability regardless of frequency (e.g., hemiplegic migraine)
≥ 2 attacks/week regardless of severity
Failure/contraindications/major side effects from acute medications
General prophylaxis
First-line [20]
Anticonvulsants (e.g., topiramate, valproate)
Beta blockers (e.g., propranolol , metoprolol, timolol)
Petasites (butterbur)
Second-line [20]
Tricyclic antidepressant: amitriptyline
NSAIDs: fenoprofen
Other: calcium channel blockers (e.g., flunarizine)
Menstrual-related migraine [20]
First-line: frovatriptan
Second-line:
Naratriptan
Zolmitriptan
Chronic migraine
Botulinum toxin (e.g., OnabotulinumtoxinA ) [23][5]
Monoclonal antibodies: anti-CGRP or anti-CGRP receptor (e.g., erenumab, galcanezumab, fremanezumab)
complications
Status migrainosus [4]
Description: Debilitating migraine attack in a patient with a known migraine diagnosis (with or without aura)
Exceptional in duration (≥ 72 hours) and severity
Often related to medication overuse
Treatment: stepwise therapy with reassessment between drug administration [24]
IV fluids
Antiemetic (e.g., metoclopramide)
NSAID (e.g., ketorolac)
Dihydroergotamine
Dexamethasone
Valproate [25]
Consider inpatient management by a specialist if there is no improvement.
Transient ischaemic attack
Transient ischemic attack (TIA) is a temporary, focal cerebral ischemic event that results in reversible neurological symptoms but is not associated with a visible acute infarct on neuroimaging. Cardiogenic embolism (e.g., from atrial fibrillation) and atherosclerosis (e.g., carotid artery stenosis) are the most commonly identified etiologies. Symptoms depend on the affected territory and may mimic an acute stroke; however, symptoms are transient. Because patients with TIA have an increased stroke risk, early diagnosis and initiation of secondary preventive therapies for subsequent stroke are vital. Management typically includes urgent neuroimaging, antithrombotic therapy (e.g., antiplatelet therapy), and prompt determination of the underlying cause (e.g., using echocardiography and neurovascular studies) to guide targeted preventative measures, such as the management of underlying atrial fibrillation or carotid artery stenosis. See also ischemic stroke and overview of stroke.
TIA dr deac pimp
Definition:
TIA refers to temporary, focal cerebral ischemia that results in reversible neurologic deficits without acute infarction (i.e., imaging findings show no signs of infarction).
Epidemiology: Prevalence: ∼ 2.3% [2][3][4] Incidence [3] Increases with age ♂ > ♀ Risk factors: same as those for acute ischemic stroke (see “Risk factors” in ischemic stroke).
Aetiology:
Classification [5]
Embolic (e.g., due to atrial fibrillation)
Lacunar/small vessel disease (e.g., due to chronic hypertension)
Large vessel disease/low-flow state (e.g., atherosclerosis of MCA, severe carotid artery stenosis)
Blood disorders/hypercoagulable states.
Ddx:
Clinical features
Acute, transient focal neurologic symptoms
Typically, symptoms last < 1 hour (the majority of cases resolve in < 15 minutes).
Symptoms depend on the affected territory (see stroke symptoms by affected vessels and stroke symptoms by affected region) and etiology. [6]
Atypical symptoms may be seen.
Management:
Approach
Perform an initial clinical evaluation , immediate ECG, and point-of-care glucose.
Complete laboratory studies and neuroimaging (within 24 hours of symptom onset). [8][9]
Identify and treat the underlying etiology.
Initiate stroke prevention measures (e.g., antiplatelet agents).
If there is evidence of an acute infarct on imaging, start immediate management for an acute ischemic stroke.
Diagnostics:
TIA is a clinical diagnosis (see “Clinical features” section).
Laboratory studies [10][8]
Immediate: serum glucose
Subsequent (within 24 hours of presentation)
CBC
BMP
Coagulation panel
Serum troponin
Serum lipid panel [10]
Optional
Hypercoagulable workup: Consider after initial diagnostic findings, notably in younger patients with TIA and no vascular risk factors or identifiable cause. [8][1]
Toxicological screen (e.g., urine drug screen, blood alcohol level): Consider if there is clinical suspicion for drug intoxication (e.g., physical signs, history of substance misuse). [11][12]
Neuroimaging [10][8]
Neuroimaging is indicated for all patients with suspected TIA within 24 hours of presentation to rule out acute infarct.
Head CT (without IV contrast)
Supportive findings: typically normal or no acute findings
MRI brain
Indication: DW-MRI is the recommended imaging modality for a suspected TIA. [8]
Supportive findings
Signs of reversible brain ischemia
Old infarcts
Normal study
A brain MRI is preferred for TIA evaluation, but a head CT (without IV contrast) must be performed first and immediately if there is concern for hemorrhage or acute infarction requiring reperfusion therapy.
Cardiac evaluation [8][10]
All patients: ECG to evaluate for atrial fibrillation or acute myocardial infarction.
Patients with suspected embolic source or unknown etiology: Further cardiac evaluation is indicated. [14]
Echocardiography
Cardiac monitoring: for a minimum of 24 hours to detect occult atrial fibrillation.
Treatment:
General principles
There is no specific treatment for the TIA itself.
Therapeutic goals consist of preventing subsequent stroke (i.e., secondary prevention) and treatment of underlying conditions.
For primary prevention measures that decrease the likelihood of a first TIA, see “Prevention” in ischemic stroke.
Antithrombotic therapy for TIA [10][1][21]
Choice of agent should take the following into consideration:
Preventative pharmacotherapy the patient was taking at the time of the TIA event
TIA severity (and risk of subsequent ischemic stroke)
Suspected etiology
Patient comorbidities
Timing: within 24 hours (start as soon as safely possible after establishing ischemic diagnosis and ruling out hemorrhage) [22]
Avoid triple therapy (DAPT plus anticoagulation) because of the increased risk of hemorrhage. [1]
Long-term stroke prevention based on suspected etiology [10][1]
Therapy should be tailored to the suspected underlying etiology.
Embolic: e.g., anticoagulation for atrial fibrillation or valvular heart disease
Lacunar/small vessel disease
Antiplatelet therapy
Treatment of hypertension (general goal < 140/90 mm Hg) [1]
Statin therapy
Large vessel disease/low-flow state
Treatment of carotid artery stenosis (e.g., revascularization), if present (see “Treatment” in carotid artery stenosis)
Treatment of ASCVD risk factors (e.g., statin therapy, anti-hypertensive therapy)
Blood disorders/hypercoagulable states: e.g., antithrombotic therapy for inherited/acquired thrombophilias
Management of other modifiable risk factors [1]
Diabetes screening and treatment
Obesity screening and lifestyle counseling
Physical activity counseling
Consider nutritionist consultation or assessment. [24]
Consider obstructive sleep apnea (OSA) screening. [10]
Smoking cessation and elimination/reduction of alcohol intake
Prognosis:
Increased risk of future ischemic stroke [3]
Within 2 days: ∼ 3–10%
Within 90 days: ∼ 9–17%
subarachnoid haemorrhage
Subarachnoid hemorrhage (SAH) refers to traumatic as well as nontraumatic bleeding into the subarachnoid space. SAH most often results from head trauma. Nontraumatic SAH is responsible for 5–10% of all strokes and is most commonly caused by the rupture of an aneurysm involving the circle of Willis. SAH typically presents with severe headache, nausea, vomiting, and/or acute loss of consciousness. Acute bleeding in the subarachnoid space appears hyperdense on noncontrast CT scan, which is the initial recommended test in diagnosis. CT angiography and lumbar puncture may be necessary for further evaluation if the initial noncontrast head CT is unremarkable. Treatment consists of carefully lowering blood pressure and preventing cerebral vasospasm. Definitive management typically consists of clipping or coiling the bleeding aneurysm to prevent potentially fatal rebleeding. SAH has a high mortality rate as a result of complications such as rebleeding and secondary ischemic strokes due to vasospasm.
Subarachnoid haemorrhage dr deac pimp
Definition
Subarachnoid hemorrhage: bleeding into the subarachnoid space
Intracerebral hemorrhage: bleeding within the brain parenchyma
Intracranial hemorrhage: a broad term used to describe any bleeding within the skull (including intracerebral hemorrhage, subarachnoid hemorrhage, subdural hemorrhage, etc.)
Hemorrhagic stroke: cerebral infarction due to hemorrhage
Intraventricular hemorrhage: bleeding within the ventricles
Epidemiology:
Traumatic
Head trauma is the most common cause of SAH.
40–60% of patients with traumatic brain injury have subarachnoid bleeding. [1]
Nontraumatic
Ruptured cerebral aneurysm is the most common cause of nontraumatic SAH.
Peak incidence: approx. 50 years of age
♀ > ♂ (3:2)
Nontraumatic SAH is responsible for 5–10% of all strokes.
Aetiology:
Traumatic SAH: traumatic brain injury [5]
Nontraumatic (spontaneous) SAH
-Causes
1.Ruptured intracranial aneurysms
–>Most commonly occur in the circle of Willis [6]
–>Berry aneurysms account for approx. 80% of cases of nontraumatic SAH. [7]
2.Ruptured arteriovenous malformations (AVM)
3. Others: cortical thrombosis, angioma, neoplasm, infection
-Triggers: most cases unknown, may be triggered by an acute rise in blood pressure (e.g., caffeine consumption, fits of anger, physical exertion) [7]
-Risk factors [7][8][9]
Smoking
Hypertension
High alcohol consumption
Positive family history
Methamphetamine and cocaine use
Large aneurysms [9]
Aneurysm location
Clinical features -Thunderclap headache Sudden, severely painful headache (often described by patients as the worst headache they have ever experienced) Holocephalic Radiates to the neck and back May present with opisthotonus
-Meningeal signs : Neck stiffness Photophobia Nausea and vomiting Kernig sign Brudzinski sign -Nonspecific signs Impaired consciousness (somnolent to comatose) Fever Sweating, hemodynamic instability -Signs due to mass effect Cranial nerve disorders Altered mental status (e.g., delirium) Focal neurologic deficits: See stroke symptoms by affected vessel and stroke symptoms by affected region. Seizures -Prodromal symptoms due to sentinel leak (a "warning leak") 30–50% of patients with SAH report prodromal symptoms days-to-weeks prior to SAH. [10] Sudden, severe headache Transient diplopia Likely due to low-grade leak of blood into the subarachnoid space → thrombus formation → fibrinolysis → hemorrhage
Diagnostic
Initial evaluation
Immediate noncontrast head CT
Best initial test
Sensitivity is almost 100% within the first 6 hours of hemorrhage [10]
Findings: shows blood in subarachnoid space (hyperdense)
Lumbar puncture (LP)
Best test if head CT is negative but suspicion for SAH remains high
Findings
↑↑ RBC count: red discoloration
↑ Protein (gamma globulin)
↑ Or normal opening pressure
Xanthochromia: the yellowish discoloration of CSF is due to the presence of xanthematin, a yellow pigment derived from hematin that is released when RBCs break down
↑ WBCs
Normal glucose.
Subsequent evaluation
Angiography: if CT and lumbar puncture are negative but clinical suspicion for SAH is still high and/or to identify the source of ongoing bleeding (prior to intervention).
Digital subtraction angiography (DSA)
CT angiography (CTA)
Additional testing to consider
Chest x-ray: to exclude pulmonary complications (e.g., pulmonary edema)
Serum troponin in all patients: predicts neurological complications and outcome
Coagulation parameters: to evaluate for coagulopathy
ECG: to exclude myocardial ischemia (e.g., ↓ left ventricular function).
Treatment
Medical therapy [13]
Reverse anticoagulation
Blood pressure management
Target SBP < 160 mm Hg is reasonable to prevent rebleeding [13]
Recommended agents: beta blockers, calcium channel blockers
Prevent vasospasm in all patients: administer calcium channel blocker (drug of choice: oral nimodipine)
Maintain euvolemia
Avoid/treat hyponatremia
Maintain normoglycemia
If patient has elevated ICP:
Consider intubation with hyperventilation
Head elevation (30°)
IV mannitol
See ICP management
Seizure prophylaxis [13]
Consider seizure prophylaxis in the immediate posthemorrhagic period
Consider long-term anticonvulsants in patients with a high risk for seizures (e.g., with a history of prior seizures).
Surgical therapy [13]
Should be performed as early as possible to prevent rebleeding
Definitive treatment options for aneurysmal SAH
Surgical clipping
Following a craniotomy, the neck of an aneurysm is surgically occluded with the help of metal clips.
Treatment of choice but more invasive than coiling
Endovascular coiling
Platinum coils are placed into the aneurysm to induce thrombotic occlusion of the aneurysm.
Less invasive than clipping but higher risk of recurrent bleeding
Consider for poor surgical candidates
The decision on which procedure to perform should be made on an individualized basis.
If the patient has hydrocephalus: ventricular drain, serial LPs, or permanent ventriculoperitoneal shunt may become necessary.
Use of nitrates should be avoided, since they may raise ICP!
Complications
Vasospasm
Occurs in approx. 30% of patients with SAH [13]
Transcranial doppler ultrasound study can help identify vasospasm.
Pathophysiology
Impaired CSF reabsorption from the arachnoid villi → nonobstructive (communicating) hydrocephalus → ↑ intracranial pressure → ↓ cerebral perfusion pressure → ischemia
Release of clotting factors and vasoactive substances → diffuse vasospasm of cerebral vessels → ischemia
Can lead to ischemic stroke
Most common in patients with nontraumatic SAH due to a ruptured aneurysm
Usually occurs between 3–10 days after SAH
Recurrent bleeding
Occurs in 4–14% of patients with SAH in the first 24 hours [13]
Risk of rebleeding is highest in the first 2–12 hours after SAH
The cumulative risk of recurrent bleeding within the first six months is about 50%.
Hydrocephalus
Occurs in 20–30% of patients with SAH [15]
Acute obstructive hydrocephalus
Usually occurs within minutes to hours after SAH
Can lead to coma and death
Chronic communicating hydrocephalus
Usually occurs weeks or months after SAH
SAH impairs CSF resorption from the arachnoid villi.
Other complications [13]
Elevated ICP: hypertension, bradycardia, and irregular breathing (see Cushing triad)
Seizures
SIADH
Hyponatremia due to volume depletion and/or cerebral salt wasting (rare)
Cardiac dysfunction (e.g., arrhythmias, acute MI)
Terson syndrome (20% of cases): preretinal hemorrhage due to SAH
Prognosis
Approx. 30% mortality rate in the U.S. within the first 30 days [13]
Survivors: increased rates of neurologic impairment (e.g., cognitive, mood changes, functional, epilepsy) and increased risk of recurrent SAH
intracerebral haemorrhage
Intracerebral hemorrhage (ICH) refers to bleeding within the brain parenchyma. The term should not be confused with “intracranial hemorrhage,” which encompasses any type of bleeding within the skull, i.e., extradural, subdural, subarachnoid, and intracerebral. The most significant risk factor for spontaneous ICH is arterial hypertension. Symptoms are often nonspecific (e.g., headache), but, depending on the affected vessel and cerebral region, focal neurologic deficits (e.g., hemiparesis) may occur. Compared to ischemic stroke, patients with ICH typically present with more severe headache and symptoms usually progress more rapidly. A noncontrast head CT, the most important diagnostic procedure, shows a hyperdense lesion in acute ICH and a hypodense lesion in hyperacute ICH. Treatment involves management of the underlying and accompanying conditions (e.g., controlling hypertension, reversing coagulopathy) and, in severe cases, neurosurgical intervention. Approximately half of patients with ICH die within 30 days. See also overview of stroke, ischemic stroke, and subarachnoid hemorrhage for more information.
Intracerebral haemorrhage dr deac pimp
Definition
Intracranial hemorrhage: a broad term used to describe any bleeding within the skull (including intracerebral hemorrhage, subarachnoid hemorrhage, subdural hemorrhage, and epidural hemorrhage) due to traumatic brain injury or nontraumatic causes (e.g., hemorrhagic stroke, ruptured aneurysm, hypertensive vasculopathy)
Hemorrhagic stroke
Rupture of a blood vessel within the brain or the cerebrospinal fluid
Subtypes
Intracerebral hemorrhage (intraparenchymal hemorrhage): bleeding within the brain parenchyma
Subarachnoid hemorrhage: bleeding into the subarachnoid space
Intraventricular hemorrhage: bleeding within the ventricles
Epidemiology
ICH is responsible for approx. 10% of all strokes. [1][2]
Most commonly affects the deep structures of the brain [3]
Intraventricular extension occurs in approx. 30% of patients with ICH
Aetiology
Nontraumatic (spontaneous)
Hypertension: most common cause of spontaneous ICH
Cerebral amyloid angiopathy: most common cause of spontaneous ICH in individuals > 60 years of age
Arteriovenous malformations: most common cause of spontaneous intracerebral hemorrhage in children
Vasculitis (e.g., giant cell arteritis)
Neoplasms (e.g., meningioma)
Ischemic stroke (due to reperfusion injury)
CNS infections (e.g., HSV encephalitis)
Septic emboli
Coagulopathy (e.g., hemophilia, anticoagulant use)
Stimulant use (e.g., cocaine and amphetamines; possibly also caffeine)
Traumatic: see traumatic brain injury
Pathophysiology
Nontraumatic mechanisms of hemorrhage
Chronic arterial hypertension → lipohyalinosis of lenticulostriate vessels, which supply the basal ganglia → formation and rupture of Charcot-Bouchard microaneurysms → lacunar strokes (ischemia) of the basal ganglia
Putamen most commonly affected
Other locations: thalamus (second most common) and infratentorial parts of the brain (e.g., pons, cerebellum)
Cerebral amyloid angiopathy: deposition of β-amyloid peptides in vessel walls → focal damage with formation of microaneurysms → rupture → recurrent lobar intracerebral hemorrhage
Structural abnormalities (e.g., vascular malformations) → exposure of parts of the abnormal vascular segment to excessive strain → rupture
Venous outflow obstruction and stimulant use (e.g., cocaine) → acute arterial hypertension
Coagulopathies: impaired hemostasis → vascular microtrauma
Inflammatory tissue necrosis → damage to vessels
Traumatic: blunt or penetrating injury → damage to vessels
Clinical features:
Headache
Absent in small hemorrhages
Most common in cerebellar and lobar hemorrhages [9]
Focal neurologic signs and symptoms may occur, depending on the location and size of the hemorrhage (see stroke symptoms by affected vessel and stroke symptoms by affected region in stroke)
Putaminal hemorrhage: contralateral hemiparesis or hemiplegia with less severe contralateral hemisensory loss; eyes deviate toward the side of the hematoma
Thalamic hemorrhage: contralateral hemiparesis, contralateral hemisensory loss, decreased consciousness, wrong way eyes
Course
Symptoms typically progress gradually over minutes to a few hours
Focal deficits worsen with expansion of the hematoma
Late: symptoms of increased intracranial pressure
Nausea and vomiting
Confusion and loss of consciousness
Bradycardia
Fixed pupils
Diagnostics Initial evaluation Immediate noncontrast head CT Best initial test Expected findings Hyperacute: hypodense lesion Acute: hyperdense lesion with hypodense perifocal edema Chronic: hemorrhage may appear as a hypodense lesion Midline shift and/or mass effect may suggest impending herniation Diffusion-weighted MRI More sensitive than head CT Expected findings Hyperacute T1: hypointense T2: hyperintense Acute: hypointense
Subsequent evaluation Laboratory studies CBC Coagulation parameters (PTT and INR) Blood glucose level Troponin Toxicology screen Angiography (e.g., CTA and/or MRA): to identify the source of the bleeding if the patient does not have any risk factors EEG
Treatment
Medical therapy [11]
Reverse anticoagulation
Blood pressure management [11]
Systolic BP > 220 mm Hg → rapidly lower to 140–160 mm Hg
Systolic BP 150-220 mm Hg → rapidly lower to 140 mm Hg
Recommended agents: IV labetalol, nicardipine, enalapril, and/or hydralazine
Maintain euvolemia
Avoid/treat hyponatremia
Maintain normoglycemia
If there are signs of elevated ICP (e.g., Cushing triad)
Consider intubation with hyperventilation
Head elevation (30°)
IV mannitol
Removal of CSF (e.g., via lumbar puncture)
See ICP management
Antiepileptic drugs: for seizures
Surgical therapy [11]
Craniotomy and clot evacuation
Indications
Signs of brain herniation (e.g., Cushing triad)
Brainstem compression
Obstructive hydrocephalus
Cerebellar hemorrhage with progressive neurological deterioration
Cerebellar hemorrhage extension > 3 cm [11]
Patients with hemorrhage in the basal ganglia or the internal capsule should generally not undergo surgical clot removal. [10]
If hydrocephalus is present: ventricular drain, serial LPs, or permanent ventriculoperitoneal shunt may be indicated
Patients with signs of brain herniation should be operated on immediately!
Complications
Elevated intracranial pressure and brain herniation
Intraventricular hemorrhage → hydrocephalus
Recurrent hemorrhage
Vasospasm and cerebral ischemia
Dysphagia: can lead to aspiration of food and pneumonia
Seizures
Hydrocephalus
SIADH
Deep vein thrombosis
See complications of stroke
Prognosis
Approximately 50% of all patients with ICH die within 30 days. [22]
multiple sclerosis
Multiple sclerosis (MS) is a chronic, degenerative disease of the CNS that is caused by an immune-mediated inflammatory process. This process results in the demyelination of white matter in the brain and spinal cord. MS has a higher prevalence among women and people in temperate regions such as Europe and North America. Impaired vision (due to retrobulbar neuritis) is usually the first manifestation of the disease. Other neurological deficits also appear as the disease progresses. The most common clinical course is characterized by exacerbations (relapses) followed by periods of complete/incomplete remission. MRI, which is the investigation of choice, reveals demyelinated sclerotic plaques in white matter. Differential diagnosis of MS includes other chronic demyelinating diseases and neurological infections (e.g., borreliosis, neurosyphilis). Acute exacerbations of MS are treated with high-dose glucocorticoids. Between relapses, patients may be treated with disease-modifying drugs (e.g., β-interferon, glatiramer acetate). No definitive therapy is available for MS.
Multiple sclerosis DR DEAC PIMP
Epidemiology
Sex: ♀ > ♂ (2:1)
Age of onset: 20–40 years of age
Ethnicity: ↑ prevalence among the white population
Prevalence is greater among people in temperate zones.
MS is more common in women!
Aetiology: Unclear Genetic predisposition Environmental triggers UV radiation, insufficient vitamin D consumption, cigarette smoking Pathogens: EBV, HHV 6.
Pathophysiology
Immune-mediated damage
Exact cause remains unknown
Characterized by inflammation, demyelination, and axonal degeneration
Most commonly accepted theory: Activation of autoreactive T-lymphocytes → inflammatory processes → focal demyelination with partial preservation of axons (acute plaques) → loss of axons and atrophy of oligodendrocytes (chronic plaques) → gliosis → inadequate remyelination
There is evidence for Th1 immune response involving myelin basic proteins
Most common sites of demyelination in MS
Periventricular areas
Brainstem
Cerebellum
Spinal cord
Clinical features
Optic neuritis (most often the earliest manifestation): impaired vision and color blindness
Internuclear ophthalmoplegia (INO) as a result of a lesion in the medial longitudinal fasciculus (MLF)
Ipsilateral medial rectus weakness but an intact convergence reflex
Disconjugate, lateral gaze nystagmus in the contralateral eye
Demyelination of spinal cord tracts
Lhermitte’s sign: a shooting electric sensation that travels down the spine when the patient flexes his/her neck
Absent abdominal reflex
Pyramidal tract lesion: upper motor neuron weakness characterized by spasticity, hyperreflexia, and a positive Babinski’s sign (see upper vs lower motor neuron lesions)
Involvement of the dorsal spinal column : loss of vibration and fine touch sensations, numbness, paresthesias, sensory ataxia
Cerebellar involvement → Charcot’s neurological triad
Scanning speech
Nystagmus
Intention tremors
Cranial nerve palsies
Autonomic dysfunction: bowel and bladder disorders, impaired sexual activity
Change in mental state: memory deficits, impaired concentration, and/or depression
Uhthoff’s phenomenon: a reversible exacerbation of neurological symptoms following physical exertion, a warm bath, or fever
In 60% of cases of optic neuritis, fundoscopy is normal. Neither the patient nor doctor are able to see anything!
Uhthoff’s phenomenon triggered by a viral infection can be confused with an exacerbation of MS!
Diagnosis
Instrument-based diagnostics
Plain MRI (brain and spine): investigation of choice
Multiple sclerotic plaques (most commonly seen in periventricular white matter) with finger-like radial extensions (Dawson’s fingers)
Contrast MRI (with gadolinium): enhancement of active lesion during and up to 6 weeks after the exacerbation
Electrophysiological studies: slowed nerve conduction → increased latency of visually evoked potentials (VEP)
For more detailed information, see McDonald’s criteria.
Lumbar puncture for CSF examination
Only indicated if MRI is inconclusive
Findings
Lymphocytic pleocytosis
Oligoclonal bands (↑ production of IgG subfractions)
The appearance of oligoclonal bands in the early stages of the disease indicates a poor prognosis!
DDX:
Autoimmune diseases
Other causes of inflammatory demyelination
Neuromyelitis optica (Devic’s disease)
Acute disseminated encephalomyelitis (ADEM, acute demyelinating encephalomyelitis)
Vasculitis resulting from connective tissue disorders (e.g., SLE, polyarteritis nodosa, granulomatosis with polyangiitis, Behcet’s disease)
Infections
Neuroborreliosis: diagnosed by CSF examination (intrathecal Borrelia-specific antibodies, lymphocytosis)
Neurosyphilis: diagnosed by a TPHA screening test
Progressive multifocal leukoencephalopathy (PML): perform an HIV test
HIV-encephalopathy: perform an HIV test.
Treatment
Summary of step-wise therapy for multiple sclerosis
The goal is to begin treatment as early as possible to treat the primary exacerbation, prevent further exacerbations, and slow down the disease process.
Therapeutic strategies include
Escalation therapy: Patients who do not respond to first-line therapy with disease-modifying drugs (DMDs), are switched to second-line DMDs.
Induction therapy: Patients with severe disease activity at onset, first receive strong immunosuppressant drugs , followed by long-term maintenance therapy with DMDs.
Treatment of acute exacerbations
First line: high-dose glucocorticoid therapy for 3–5 days (methylprednisolone 500–1000 mg/d IV or PO)
If symptoms decrease: End glucocorticoid therapy by slowly tapering the dose.
Prophylaxis against side effects of corticosteroids: proton pump inhibitors to prevent gastritis and LMW heparin for thromboprophylaxis (see → side effects of glucocorticoids)
Second line: plasmapheresis.
Supportive therapy
Spasticity: dantrolene, baclofen ,physiotherapy
Painful paresthesias: carbamazepine, amitriptyline
Urinary retention: intermittent catheterization and parasympathomimetic drugs
Urinary incontinence: parasympatholytic agents (e.g., oxybutynin)
Erectile dysfunction: sildenafil
Depression: antidepressants
Special patient groups
Effect of pregnancy on MS:
Decreased relapse rate of MS during pregnancy
Increased relapse rate in the postpartum period
The long-term clinical course of MS remains unchanged.
Effect of MS on pregnancy: ↑ rate of caesarean sections and ↓ birth weights when compared to mothers without MS.
Bipolar disorder summary
Bipolar disorder is a psychiatric illness characterized by episodes of mania (or hypomania) and major depression, interspersed with periods of normal mood and functioning. Men and women are equally affected, and there is a strong genetic component to the disease. During manic episodes, patients may experience elevated mood, talkativeness, racing thoughts, and psychosis, and often endanger themselves or others. Depressive episodes are characterized by sadness, anhedonia, and hopelessness. Although episodes of mania or depression can occur anytime, they are especially triggered by environmental factors (eg, lack of sleep, psychosocial stress). Manic episodes are treated acutely with lithium, antipsychotics, and benzodiazepines. Lithium is also commonly used for long-term treatment, as is valproic acid. More on word document
major depressive disorder
Major depressive disorder (MDD) is an episodic mood disorder primarily characterized by depressed mood and anhedonia lasting for at least 2 weeks. Women have a higher risk of developing MDD than men. The peak age of onset is the 3rd decade. The etiology is multifactorial, including both biological and psychological factors. Reduced levels of neurotransmitters (serotonin, noradrenaline, dopamine) are believed to be the pathophysiological basis in most cases. Other symptoms of MDD include sleep disturbance, loss of appetite, and thoughts of suicide. There are various subtypes of MDD characterized by additional symptoms or occurrence in specific conditions, such as atypical depression (additionally characterized by, e.g., weight gain and increased appetite), psychotic depression (with additional psychotic features such as hallucinations and delusions), and peripartum depression (which occurs during or shortly after pregnancy). In elderly patients, MDD can also manifest with memory loss and other symptoms seen in dementia, referred to as pseudodementia. Treatment is multifaceted and often requires pharmacotherapy, psychotherapy, and lifestyle changes. First-line treatment mainly consists of SSRIs (e.g., citalopram) and SNRIs (e.g., venlafaxine).
