Seizures and Epilepsy

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Table 3-4. Distinguishing Characteristics of the Progressive Myoclonic Epilepsies Clinical Features Chorea Dentorubral-pallidoluysian atrophy Juvenile neuroaxonal dystrophy Juvenile Gaucher disease Deafness Biotin-responsive encephalopathy MERRF Sialidosis type II Focal Occipital Spikes MERRF Unverricht–Lundborg disease Little or No Dementia Biotin-responsive encephalopathy Noninfantile Gaucher disease Myoclonus—nal failure Sialidosis type I Unverricht–Lundborg disease Severe Dementia GM2 gangliosidosis Juvenile neuroaxonal dystrophy Lafora disease Late infantile NCL Severe Myoclonus Lafora’s disease MERRF Sialidosis Genetics Autosomal Dominant Dentatorubral-pallidoluysian atrophy Kuf disease Geography Canada Myoclonus—renal failure Finland Santavori disease Unverricht–Lundborg disease Japan Dentatorubral pallidoluysian atrophy Sialidosis type II Sweden Gaucher disease Maternal Inheritance MERRF 21/100,000 children. 20 The clinical features include a single nocturnal seizure with clonic movement of the mouth and gurgling. Secondary generalization is common. Alteration in consciousness, aura, and postictal confusion 21, 22 are rare. The seizures resolve by age 16 years. CHAPTER 3 Seizures and Epilepsy ■ 33 Table 3-5. Possible Autonomic Seizure Clinical Features Abdominal sensations Apnea Arrhythmia Chest pain Cyanosis Erythema Flushing Genital sensations Hyperventilation Incontinence Miosis Perspiration Vomiting The interictal EEG consists of central and midtemporal high-amplitude spike and wave with a characteristic dipole. The ictal EEG usually consists of a focal central or mid-temporal ictal onset, with the possibility of secondary generalization. 23,24 Temporal lobe epilepsy. Temporal lobe epilepsy accounts for approximately 70% of partial seizures. Many patients have a prior history of febrile seizures or head trauma. A prodrome consisting of lethargy is common. Auras are also common but not universal and include an array of findings such as déjà vu. The ictal findings or semiology include oral or motor automatisms, alteration of consciousness, head and eye deviation, contralateral twitching or tonic–clonic movements, and posturing. Right temporal lobe seizures are often hypermobile. Left temporal lobe seizures often result in behavior arrest. Versive head movements are relatively common, and 90% of patients with versive head movements had a primary epileptogenic zone in the contralateral hemisphere. Ipsiversive movements are less common but occur most commonly in patients with temporal foci. The postictal phase consists of minutes to hours of confusion and somnolence. 24–30 Frontal lobe epilepsy. Frontal lobe epilepsy accounts for approximately 20% of partial seizures. A prodrome is rare. Auras are unusual. The seizures typically consist of combinations of behavior alteration and automatisms of very brief duration. Frontal seizures often have atypical presentations and vary widely depending on the region of the frontal lobe from which the seizures arise (Table 3-6). Postictal confusion is rare. 31–36
34 ■ Section 2: Common Pediatric Neurologic Problems Table 3-6. Frequency of Aura Types by Location Temporal Frontal Occipital Aura Type (%) (%) (%) Auditory 10 0 0 Cephalic 5 15 5 Epigastric 50 15 5 General 10 15 5 Gustatory 10 0 10 None 15 40 5 Olfactory 10 0 10 Psychical 15 5 15 Somatosensory 5 15 0 Visual 10 5 50 Vertiginous 10 2 0 Occipital lobe epilepsy. Occipital lobe epilepsy is rare, accounting for less than 10% of partial seizures. Prodromes are rare with occipital lobe seizures and auras are unusual. As with the frontal lobe seizures, the seizure characteristics are dependent on the area of the occipital lobe involved. When the striate cortex is involved, there are typically elemental visual hallucinations. Involvement of the lateral occipital lobe results in twinkling, pulsing lights. Seizures arising from the temporo-occipital are usually associated with formed visual hallucinations. 37-39 Parietal lobe epilepsy. Parietal lobe seizures are also relatively uncommon. The may be seen as simple partial seizures but they will often propagate. The initial features can include contralateral paresthesias, contralateral pain, idiomotor apraxia, and limb movement sensations. As the seizure progresses and propagates, asymmetric tonic posturing and automatisms may develop. 40-42 Landau–Kleffner syndrome. Landau–Kleffner syndrome is a rare, invariably progressive, idiopathic acquired aphasia related to a focal epileptic disturbance in the area of the brain responsible for verbal processing. 43 The syndrome begins between ages 3 and 10 in a child with normally acquired language abilities. The child then develops a verbal auditory agnosia and infrequent nocturnal partial or secondarily generalized seizures. The syndrome has a pathognomonic EEG pattern consisting of high-voltage multifocal spikes, predominating in the temporal lobes. 44 Treatment is usually with valproic acid and benzodiazepines. 45 Sometimes corticosteroids and IV Ig or even surgery with subpial transection 46 are used in refractory cases. The outcome for overall language and cognitive function depends in part on how early the syndrome is recognized and treated, but over 2/3 of children are left with significant language or behavioral deficits. 47 Rasmussen encephalitis. Rasmussen encephalitis is a syndrome of diffuse lymphocytic infiltration of the brain associated with partial seizures and progressive neurological deterioration with hemiparesis. This disorder typically affects children 1 to 14 years old. The syndrome is associated with perivascular cuffing on pathologic sections, and antibodies to the glutamate subunit GluR3 are commonly identified. The disorder is usually unilateral. Rasmussen encephalitis is very difficult to treat and frequently requires surgical management with hemispherectomy. EVALUATION As with many facets of neurology, the history is the most important diagnostic tool and should include information on each of the items in Table 3-7. The history should be obtained from family and eyewitnesses, if possible. Many patients are unable to provide accurate descriptions of the seizure and the postictal period. MRI of the head with temporal lobe protocol (thin coronal slices through hippocampi) is the preferred imaging modality for most patients. The MRI sequences are much more sensitive to the causes of epilepsy than is CT imaging. CT can, however, be of help in the emergency department setting. EEG is a vital component of the evaluation to categorize the seizure type and assist with planning of the treatment strategy. The need for laboratory testing is highly variable depending on the history. Initial evaluation with fluid balance profile (FBP), Ca++, Aura Types Table 3-7. Psychical Auras Illusion Hallucination Memory Déjà vu, jamais Flashbacks vu, strangeness Sound Advancing, receding, Voices, music louder, softer, clearer Self-image Depersonalization, Autoscopy remoteness Time Stand-still, rushing, slowing Vision Macropsia, micropsia, Objects, faces, near, far, blurred scenes
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Seizures and Epilepsy

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