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发表于 2025-3-8 20:23:42 | 显示全部楼层 |阅读模式
本帖最后由 岛民A 于 2025-3-11 06:53 编辑

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.

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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.

-


---------------------
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)


------------------------

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


----------------

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.
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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

------------------
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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