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Generalized Tonic-Clonic Seizures(grand mal)
1. Prodrome
- Hours before the seizure, patients may experience vague prodromal symptoms, such as:
- Sleep disturbances
- Lightheadedness
- Mood changes (anxiety, irritability)
- Impaired concentration
2. Ictal Phase
- Loss of consciousness: Patients lose consciousness completely during the seizure.
- Tonic phase: Generalized muscle stiffness and contraction.
- Clonic phase: Rhythmic jerking movements in all limbs.
- Absence of auras or automatisms: These are typically absent unless the seizure is secondary to a focal seizure that generalizes.
3. Postictal Phase
- Confusion and lethargy: Patients often experience confusion and fatigue after the seizure.
- Lactic acidosis:
- Excessive muscle activity leads to hypoxia, anaerobic respiration, and lactate accumulation.
- Manifests as an anion gap metabolic acidosis (serum bicarbonate ↓).
- Usually transient and resolves within 2 hours; no need for IV bicarbonate therapy.
- Other laboratory abnormalities:
- Hyperprolactinemia: Serum prolactin levels rise (at least three times baseline) within 30 minutes post-seizure (serum prolactin ↑), which can help differentiate epileptic seizures from psychogenic seizures, though results are unreliable (as syncope or stress can also elevate prolactin).
- Elevated creatine kinase (CK): A marker of muscle injury (CK ↑).
- Elevated white blood cell count, cortisol, neuron-specific enolase (NSE), and lactate dehydrogenase (LDH): White blood cell count ↑, cortisol ↑, NSE ↑, LDH ↑. These findings are nonspecific and not commonly used for diagnosis.
4. Diagnosis and Management
- Electroencephalogram (EEG): Helps guide decisions on long-term anticonvulsant therapy.
- Monitor electrolytes and arterial blood gases: Until they normalize.
- No specific treatment needed: Lactic acidosis typically resolves on its own without intervention.
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ddx
- Sydenham chorea is a symptom of rheumatic fever, typically occurring 1–8 months after group A streptococcal infection.
- It is the most common form of acquired chorea in childhood.
- It presents with rapid, irregular, and non-stereotyped movements.
- Symptoms are usually generalized, involving the head, neck, and limbs.
- Movements persist while awake and worsen over hours to days.
----------------- myoclonic(no stiffening phase) Generalized Myoclonic Seizures &Juvenile Myoclonic Epilepsy (JME)
Generalized Myoclonic Seizures:
- Brief, sudden, shock-like muscle jerks (myoclonus) affecting the entire body.
- Occur without warning (no prodrome) and cause minimal impairment of consciousness.
- No aura or postictal confusion.
Juvenile Myoclonic Epilepsy (JME):
- Onset in adolescence.
- Myoclonic jerks occur immediately after waking up.
- Often coexists with absence seizures and generalized tonic-clonic seizures.
Juvenile Myoclonic Epilepsy (JME) follows an age-related seizure progression:
1️⃣ Childhood (~around 10 years old): Some patients may initially experience absence seizures.
2️⃣ Early adolescence (~12-18 years old): Morning myoclonic jerks typically emerge, often as the first manifestation of JME.
3️⃣ Mid-to-late adolescence (~14-20 years old): Some patients develop generalized tonic-clonic seizures (GTCs), often triggered by sleep deprivation or early morning awakening.
--------------------- Ddx Convulsive Syncope
- Etiology: Caused by transient cerebral hypoperfusion, often triggered by situational factors (e.g., prolonged standing, dehydration, fear, pain).
- Clinical Features:
- Brief loss of consciousness, often with a prodrome (e.g., dizziness, nausea, tunnel vision).
- Myoclonic movements or seizure-like activity due to reduced midbrain perfusion.
Key Differentiation from Seizures:
- Typically shorter duration and lack of postictal confusion.
- No tongue biting, no prolonged tonic-clonic phase, and rapid recovery.
- EEG in convulsive syncope is normal, whereas seizures show abnormal epileptiform activity.
----------------------- ddx Breath-Holding Spells (BHS) Definition: Reflexive episodes of prolonged expiratory apnea, triggered by emotional distress, tantrums, or minor injuries, leading to cyanosis/pallor, syncope, and/or anoxic seizures.
Epidemiology:
- Occurs in children aged 6 months to 6 years, peaking at 6–18 months.
- Usually resolves by age 4–8 years.
Clinical Features:
- Triggers: Emotional distress, pain, minor trauma.
- Sequence: Crying → Forced exhalation → Breath-holding → Cyanosis (cyanotic BHS) or pallor (pallid BHS) → Brief loss of consciousness (<1 min) → Rapid recovery.
- Pallid BHS: Often triggered by minor trauma and associated with bradycardia before syncope.
Differentiation from seizures:
- No postictal confusion, no prolonged tonic-clonic movements.
- Provoked by emotional/physical stimuli, whereas seizures often occur spontaneously.
- ECG is warranted if BHS is frequent, prolonged (>1 min), or if there is a family history of arrhythmias.
Diagnosis & Management:
- Clinical diagnosis; routine testing not required.
- Iron-deficiency anemia screening (CBC, serum ferritin), as iron supplementation may reduce frequency.
- Reassurance is key; no special precautions needed.
- If atypical (e.g., prolonged apnea, frequent spells), consider EEG to rule out epilepsy or ECG for arrhythmia evaluation.
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--------------------- ddx Psychogenic Nonepileptic Seizures (PNES) Definition: PNES are seizure-like episodes of psychiatric origin, which mimic epileptic seizures but lack neurophysiological abnormalities on video EEG or neuroimaging.
Key Features Differentiating PNES from Epileptic Seizures:
- Prolonged seizure duration (typically >2 minutes)
- Closed eyes during the episode (vs. open eyes in true seizures)
- Unimpaired awareness (e.g., partially responsive or purposeful movements)
- No postictal confusion or lethargy
- Lack of typical seizure-related injuries (e.g., tongue biting, incontinence)
Epidemiology & Risk Factors:
- More common in women
- Strongly associated with psychiatric comorbidities, including:
- Depression, anxiety, PTSD
- Personality disorders (e.g., borderline personality disorder)
Diagnosis & Management:
- Diagnosis confirmed with video EEG (shows no epileptiform activity during events).
- Explain the diagnosis supportively to help the patient gain insight.
- Refer for psychotherapy (e.g., cognitive behavioral therapy) to address underlying psychiatric issues.
- Antiepileptic drugs are not effective and should not be used.
------------ Breakthrough Seizure
- Definition: A seizure occurring in a patient with epilepsy after a period of remission while on antiepileptic drugs.
Common Causes:
- Missed doses of medication
- New drug interactions
Triggers:
- Alcohol consumption
- Recreational drug use
- Sleep deprivation
- Illness (e.g., infection)
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Status Epilepticus Management andCortical Laminar Necrosis (CLN) First-line Treatment:
- Parenteral benzodiazepines (e.g., lorazepam, midazolam) are used for early status epilepticus (seizures lasting 5-20 minutes).
- Benzodiazepines increase GABAergic transmission, reducing neuronal excitability and aborting the seizure.
- If IV access is not possible, alternative routes (e.g., intramuscular, rectal, buccal, nasal) should be used.
- If initial treatment is ineffective, benzodiazepines should be repeated every 5-10 minutes.
- Investigate and treat rapidly reversible causes of seizures (e.g., hypoglycemia, hyponatremia, hypocalcemia).
- Second-line Treatment (for persistent status epilepticus lasting 20-40 minutes):
- Intravenous fosphenytoin, levetiracetam, or valproate are used.
Refractory Status Epilepticus (seizures lasting > 40 minutes):
- May require repetition of second-line therapy or induction of coma (e.g., IV propofol, thiopental, midazolam, pentobarbital).
Cortical Laminar Necrosis (CLN):
- Status epilepticus can lead to irreversible CNS tissue damage, including cortical laminar necrosis (CLN), which causes permanent neurological deficits.
- CLN is associated with repeated seizures, hypoxia, and hypoglycemia.
- Symptoms vary depending on the affected cortical area and can include motor dysfunction, vision impairment, and aphasia.
- CLN increases the risk of further seizures and cortical damage.
- Imaging (e.g., diffusion-weighted imaging) shows cortical hyperintensity in CLN.
ddx:Migraine with Aura and AlternativeDiagnosis Migraine with Aura:
- Migraines can be accompanied by an aura, which includes positive and negative focal neurologic symptoms (e.g., visual disturbances, paresthesias, pareses).
- Auras develop gradually (usually >5 minutes), last no longer than 1 hour, and are completely reversible.
- Hemiplegic migraine specifically involves motor weakness as part of the aura.
- Prodromes (e.g., depression, euphoria) may occur 24–48 hours before the headache, but the headache itself is the defining feature of a migraine.
- Aura without headache (silent migraine) is uncommon but can occur in some patients, but most hemiplegic migraine patients have a headache with each attack
Differential Diagnosis:
- Migraines can cause focal neurological symptoms, including arm weakness and speech impairments, but rarely produce multiple simultaneous focal neurological deficits.
- Migraines do not typically involve loss of consciousness or altered mental status, indicating the need to consider alternative diagnoses.
-------------- Conversiondisorder is an unconscious response to psychological stress, either acuteor remote, that manifests as a neurological disorder and is distressing for thepatient. Possiblesymptoms include altered motor function, non-epileptic seizures, and/orweakness
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Todd Paralysis (TP) vs. Stroke/TIA Todd Paralysis (TP)
- Definition: Transient focal weakness following a seizure, typically resolving within 48 hours.
- Cause: Likely due to exhaustion of the primary motor cortex.
- Duration: Lasts from a few minutes to 36 hours.
- Common Triggers: Most often occurs after focal seizures, with or without secondary generalization.
- Symptoms: Postictal paresis or paralysis, potentially affecting one limb or half of the body.
Stroke/TIA Key Differences:
- Persistent symptoms in stroke/TIA vs. transient weakness in TP.
- Symptoms localize to a single vascular territory in stroke/TIA, whereas TP follows a postictal pattern.
- Muscle pain is common in TP, but not typical in stroke/TIA.
Diagnosis & Imaging
- Clinical diagnosis is key but may require imaging to rule out stroke.
Diffusion-weighted MRI:
- Detects ischemia within 30 minutes (useful for stroke differentiation).
- Identifies hyperacute hemorrhage if present.
--------------- Absence Seizures(petite mal) Clinical Features:
- Brief episodes of unresponsiveness with interrupted activity and anterograde amnesia.
- Postural tone and staring are maintained throughout the episode.
- May include subtle automatisms (e.g., lip-smacking, eye fluttering, head nodding).
- No prodromal symptoms, postictal lethargy, or confusion.
Duration & Frequency:
- Typically last < 20 seconds but can occur hundreds of times per day.
- Atypical absence seizures may last > 30 seconds, have a more gradual onset, and allow for some responsiveness.
EEG Findings:
- 3 Hz spike-and-slow-wave complexes are characteristic of childhood absence epilepsy.
Key Differentiation:
- Unlike other seizures, absence seizures do not have a postictal phase.
--------------- Atonic Seizures(drop seizure) Clinical Features:
- Sudden, transient loss of muscle tone (lasting < 2 seconds).
- May result in head nodding or drop attacks.
- No aura, postictal confusion, or lethargy.
----------------- Focal Seizures with Impaired Awareness(Formerly Complex Partial Seizures) Cause: Abnormal electrical activity in a specific lobe of the brain, often due to structural abnormalities (e.g., encephalitis, developmental disorders, trauma, stroke).
Phases:
- Aura: May include gustatory (e.g., muddy taste, temporal lobe), visual (occipital lobe), sensory (parietal lobe), or motor (frontal lobe) disturbances.
- Ictal Phase: Impaired awareness, staring, automatisms (e.g., facial grimacing, hand gestures), sometimes focal motor activity.
- Postictal Phase: Confusion, lethargy, amnesia, may vary based on lobe involvement.
Lobe-Specific Features:
- Temporal lobe: Gustatory aura, déjà vu, auditory hallucinations.
- Frontal lobe: Hyperkinetic movements, bicycling motions, rapid onset and offset.
- Parietal lobe: Sensory disturbances (e.g., tingling, numbness).
- Occipital lobe: Visual hallucinations, blindness, flashing lights.
Diagnosis:
- EEG: Focal epileptiform discharges (location depends on seizure origin).
- MRI: To identify structural abnormalities.
Treatment:
- First-line: Lamotrigine, levetiracetam.
- Refractory cases: Surgical intervention (especially for temporal lobe epilepsy).
Prognosis: Variable, but temporal lobe epilepsy tends to have poorer seizure control, with only ~40% achieving remission on medication alone.
--------------------- Simple Febrile Seizures Definition & Characteristics
- Occur in children 6 months to 5 years old
- Generalized tonic-clonic seizures, lasting <15 minutes
- Do not recur within 24 hours
- No focal neurological deficits and rapid recovery
Diagnosis & Management
- No specific workup needed if fever cause is known and neurological exam is normal
- Antipyretic therapy (NSAIDs like ibuprofen or acetaminophen) helps symptom relief and fever reduction
- Dehydrated children with reduced oral intake may require hospital admission for IV fluids
Hospital Admission Criteria
- Recurrent seizures
- Neurological abnormalities
- Failure to return to baseline after seizure
EEG & Further Evaluation
- EEG is not required unless the seizure is complex or neurological exam is abnormal
Risk Factors
- High fever, viral infections (e.g., HHV-6, influenza)
- Family history of febrile seizures
- Recent immunization (e.g., DTaP, MMR)
Prognosis & Parental Reassurance
- Most children recover quickly and can be discharged
- Low risk of recurrence and long-term neurological complications
Emergency Treatment
- Lorazepam is used to abort prolonged (≥5 min) or repetitive febrile seizures
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Epilepsy
mutiple,unprovoked
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Aura
An aura in seizures occurs when the seizure activity affects a sufficient portion of the brain to produce symptoms but not enough to impair consciousness. It is often considered a focal aware seizure and may serve as a warning sign for a larger seizure.
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