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  1. Epileptic syndromes
  2. The classification of the epileptic syndromes considers the predominant seizure type, age of onset, natural history, EEG, response to AEDs, etiology, family history, and prognosis.(21) Some of more common syndromes are West syndrome characterized by infantile spasms, arrested psychomotor development, and hypsarrhythmia; Lennox-Gastaut syndrome which includes tonic, atonic, atypical absence, myoclonic, generalized tonic-clonic, partial seizures with a characteristic EEG, febrile convulsions, and neonatal seizures; and juvenile myoclonic epilepsy characterized by myoclonic jerks, generalized tonic-clonic, and absence seizures. Rolandic epilepsy is a well-defined epileptic syndrome that begins between the ages of 3 and 13. It is characterized by high-voltage centrotemporal spike focus which may shift from side to side. The patients has simple partial seizures with sensory and motor symptoms and there is often secondary generalization.(22) Rolandic epilepsy tends to be benign and may not require AED therapy. Table 6 is the International Classification of important Epileptic Syndromes.
  3. Table 6. Classification of important epileptic syndromes
    1. Location-related
      (focal, partial syndromes)
      Idiopathic
      • Rolandic epilepsy (benign childhood epilepsy with centroentemporal spikes)
      • Occipital epilepsy
    Generalized syndromes
      Idiopathic
      • Childhood absalizaence epilepsy
      • Juvenile absence epilepsy
      Cryptogenic or symptomatic
      • Infantile spasms (West syndrome)
      • Myoclonic astatic epilepsy (Lennox-Gastaut syndrome)
      • Epilepsy with myoclonic absences
      Syndromes undetermined whether focal or generalized
        With both generalized and focal seizures
        • Neonatal seizures
        • Epilepsy with continuous spike-wave during sleep

      Classification

      The International League Against Epilepsy has developed a classification system that combines clinical description with EEG findings. Over 90% of seizure patients may be classified using this system. Using the international classification scheme, seizures may be divided into partial, generalized, or unclassified.

      Table 2. Classification of epileptic seizures

      Traditional classification

      New nomenclature

      Focal motor; Jancksonian seizure
        1. Partial seizures (seizures begin locally)
          1) Simple (without impairement of consciousness)
          • With motor symptoms
          • With special sensory or somatosensory symptoms
          • With autonomic symptoms
          • With psychic symptoms

      Temporal lobe
      or psychomotor seizures

          2)Complex (with impairment of consciousness)
          • Simple partial onset followed by impairment of consciousness - with or without automatisms
          • Impaired consciousness at onset-with or without automatisms
          3) Secondarily generalized
          (partial onset evolving to generalized tonic-clonic seizures)
      Petit mal
        2. Generalized seizures (bilaterally symmetrical and without local onset)
          1) Absence

      Minor motor

          2) Myoclonic

      Limited grand mal

          3) Clonic
          4) Tonic

      Grand mal

          5) Tonic-clonic

      Drop attacks

          6) Atonic
          7) Infantile spasms

      -

        3. Unclassified seizures
        4. Status epilepticus
        (prolonged partial or generalized seizures without recovery between attacks)

      1) Partial epileptic seizures

      Partial seizures begin in one hemisphere of the brain and, unless they become secondarily generalized, result in an asymmetric seizure. Partial seizures may be described as focal or unilateral seizures and may be subdivided into simple and complex. Partial seizure manifest as alterations in motor functions, sensory or somatosensory symptoms, or autonomic symptoms. If there is no impairment of consciousness, the seizures are classified as simple partial. If there is impairment of consciousness, the seizures are described as complex partial. With complex partial seizures, aberration of behavior (automatisms) may occur. Patients may exhibit inappropriate behavior like lip smacking, tearing of clothing and aimless wandering. Often there is postictal period. Complex partial seizures are more likely to progress to a secondarily generalized seizure.

      Table 3. Partial epileptic seizures


      Simple partial seizures (focal)

      Complex partial seizures

      Consciousness Unaffected Impaired
      Age group Any age Any age
      Duration Seconds-minutes Minutes
      Symptoms Depends upon focus localization ;
      no postictal confusion

      Depends upon focus localization ;
      postictal confusion

      Ictal EEG Contralateral epileptiform discharges ;
      in many instances no ictal abnormalities
      (in scalp recording)
      Unilateral or bilateral epileptiform
      discharges, diffuse or focal
      Special features An aura is simple partial seizure !

      -

      Table 4. Secondarily generalized seizures

      Initial symptoms

        Depends
        • seizure type (simple/complex partial)
        • localization of epileptic focus
      Subsequent symptoms
        Generalized tonic-clonic convulsion

      2) Generalized epileptic seizures

      Generalized tonic-clonic seizures are the most common form of epilepsy. It is characterized by the patients losing consciousness and falling at the onset of the seizure. Tonic muscle spasms (rigid posturing of the limbs or torso) occur and may be accompanied by a cry which results from air being moved out of the larynx. Following the tonic phase, there is a period of generalized clonic movements. After the clonic phase, the patients returns to consciousness, but may remain lethargic and confused for varying periods of time.

      ¨ç Absence seizure
      Absence seizure consists of episodes of brief loss of consciousness. Eyelid fluttering, small chewing movements or mild shaking of the hands may be exhibited. The EEG during the seizure has a characteristic 2 to 4 cycles spike and slow wave complex. The patient regain awareness quickly and there is no postictal state. Complex absence seizure may be accompanied by muscle twitching, myoclonic jerking or autonomic manifestations. The patients do not fall as with tonic-clonic seizures.

      ¨è Tonic-clonic seizures
      Tonic-clonic seizures are what many people think as epilepsy. Although they may be preceded by premonitory symptoms known as aura, the majority of patients lose consciousness without warning. The seizure results in a sudden sharp tonic contraction of muscles followed by a period of rigidity. During this period the patient may fall and be injured. During the seizure the patient may cry or moan, lose sphincter control, bite his or her tongue, or develop cyanosis. After the seizure the patient may be unconscious for a variable time, and frequently goes into a deep sleep. Tonic and clonic seizures may occur separately.

      ¨é Myoclonic
      Brief shock-like muscular contractions of the face, trunk, and extremities are known as myoclonic jerks. They may be isolated events or rapidly receptive.

      ¨ê Atonic
      A sudden loss of muscle tone is known as an atonic seizure. This may be described as a head drop, the dropping of a limb, or a slumping to ground.

      3) Unclassified seizures

      Unclassified seizures include all seizures that cannot be classified because of inadequate or incomplete data.

      Table 5. Generalized epileptic seizures


      Absences

      Myoclonic seizures

      Atonic seizures

      Genenalized tonic-clonic seizure

      Age group Children and juveniles Children and juveniles Infants and children Any age
      Duration

      Few-30 seconds

      1-5 seconds Few seconds 1-3 minutes
      Symptoms Sudden loss of consciousness accompanied by staring and blinking; immediate regain of consciousness Brief jerks in arms or legs Sudden loss of muscle tone causing severe head injuries (fall) Initial cry (sometimes); loss of muscle tone; respiratory arrest; cyanosis; tonic convulsions; clonic convulsions; relaxation followed by deep sleep
      Ictal EEG Bilateral regular 3 (2-4) Hz spike-waves Polyspike-waves, spike-waves, or sharp and slow waves Polyspike-waves, flattening, or low-voltage fast activity Often obscured by muscle artifacts
      Special features Long absence with automatism, lip smacking, chewing, fiddling, fumbling Often occur in series - Possible tongue biting and urinary incontinence

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