Table 1.
Electroclinical features and diagnosis of 18 patients with EAS.
| No./Gender | Family history* | Age at SZ onset (months) | Age at last follow-up (months) | SZ types at onset (18) | New SZ types (months from SZ onset) | Behavioral disorder | ADHD | Cognitive impairment (Intel. Level/Lan. deficits/Learn. diff) | Motor dysfunction | EEG | Diagnosis | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Side and pattern | Max. SWI (months from SZ onset) | |||||||||||
| 1F | No | 42 | 144 | GTCS | AA/AS (48) | Dull/irritability/attention deficit | NG | 3/language delayed, slurred speech/learning difficulties, sensory integration disorder | Drooling, fine motor clumsiness, drooping of the upper limbs | Diffuse | ≥85% (53) | CSWS |
| 2M | Yes | 77 | 156 | GTCS | Absences/AS/eyes blink attack (40) | Irritability | NG | 3/dysarthria/learning difficulties | Hypotonic, drooling, dysphagia | Multifocal | ≥85% (41) | CSWS |
| 3M | No | 51 | 156 | GTCS | AS (20) | NG | NG | 3/auditory agnosia, expressive aphasia/learning difficulties | NG | Focal (L rolandic) | ≥85% (24) | LKS + ESES |
| 4M | Yes | 107 | 151 | GTCS | None | NG | NG | 0/N/learning difficulties | NG | Diffuse | ≥85% (1) | ABPE (I) |
| 5F | No | 80 | 122 | FS/FBTCS/GTS/eyes blink attack | None | Attention deficit/slow reactiveness | NG | 3/N/learning difficulties | Drooling, drooping of the upper limbs | Hemispheric (L) | ≥85% (22) | CSWS |
| 6F | No | 67 | 117 | GTCS | Absences (29) | Dull/irritability/attention deficit | NG | 3/N/learning difficulties | Nocturnal urinary incontinence | Diffuse | ≥85% (29) | CSWS |
| 7F | No | 72 | 108 | GTCS/SE | AA/AS (24) | Attention deficit | NG | 2/language regression/learning difficulties | Drooling | Focal (L rolandic) | 50–84% (24) | ABPE (mix) |
| 8F | No | 35 | 72 | FMS/FSIA | AA (23) | Slow reactiveness | NG | 2/N/NG | NG | Diffuse | 50–84% (23) | ABPE (I) |
| 9M | No | 28 | 67 | FBTCS | None | Social interaction difficulty | NG | 0/auditory agnosia, language regression/NG | Hypotonic, unwilling to walk | Focal (L rolandic) | <50% | LKS |
| 10M | No | – | 80 | None | None | Slow reactiveness | NG | 2/language regression, expressive aphasia/learning difficulties | Night terrors, poor sleep | Diffuse | ≥85% | ABPE (II) |
| 11M | Yes | 27 | 97 | FBTCS | None | Attention deficit | + | 1/dysarthria/spatial-visual disorder | Tongue dyspraxia, drooling | Diffuse | 50–84% (33) | ADHD+ABPE (II) |
| 12F | No | 30 | 110 | GTCS | SE/FSIA (1) | NG | NG | 3/language delayed, slurred speech/learning difficulties | NG | Diffuse | ≥85% (3) | CSWS |
| 13M | No | 29 | 60 | GTS/PMS/absences/AS | None | Social interaction difficulty, irritability with pinching people more often | NG | 3/auditory agnosia, expressive aphasia/learning difficulties | Fine/gross motor clumsiness, negative myoclonus with sudden fall | Multifocal | <50% | LKS |
| 14F | No | 51 | 138 | FSIA | None | Slow reactiveness | NG | 2/auditory agnosia, expressive aphasia/learning difficulties | NG | Multifocal | 50–84% (6) | LKS + ESES |
| 15F | No | 32 | 85 | FS/GTS | SE/PMS/NMS/AS (9) | Slow reactiveness | NG | 1/auditory agnosia, language regression/learning difficulties | Drooping of the upper limbs | Hemispheric (R) | ≥85% (13) | LKS + ESES |
| 16M | Yes | 100 | 168 | GTCS | None | Attention deficit/hyperactive | + | 1/N/learning difficulties | Night terrors, poor sleep | Diffuse | <50% | ABPE (I) + ADHD |
| 17M | No | – | 138 | None | None | NG | NG | 0/speech dyspraxia, slurred speech/N | Tongue dyspraxia | Focal (R rolandic) | <50% | ABPE (II) |
| 18M | No | 32 | 72 | FBTCS/PMS/AS | None | Social interaction difficulty | NG | 3 auditory agnosia, language regression/learning difficulties | NG | Multifocal | <50% | LKS |
F, female; M, male; N, normal; SZ, seizure; FMS, focal motor seizures; FS, focal seizures; FSIA, focal seizures with impaired awareness; FBTCS, focal to bilateral tonic-clonic seizures; SE, status epilepticus; GTCS, generalized tonic-clonic seizures; GTS, generalized tonic seizures; AA, atypical absences; PMS, positive myoclonic seizures; NMS, negative myoclonic seizures; AS, atonic seizures; DD/ID, developmental delay/intellectual disability (normal IQ or no DD/ID = 0 points, borderline DD/ID = 1 point), mild DD/ID = 2 points, moderate DD/ID = 3 points, severe DD/ID = 4 points); SWI, spike-wave index; R, right; L, left; LKS, Landau-Kleffner syndrome; CSWS, epileptic encephalopathy with continuous spike-and-wave during sleep; ABPE, atypical benign partial epilepsy; ADHD, attention-deficit hyperactivity disorder; ESES, electrical status epilepticus during slow-wave sleep; CZP, clonazepam; VPA, sodium valproate; LEV, levetiracetam; LTG, lamotrigine; OXC, oxcarbazepine; NZP, nitrazepam; Intel. Level, intellectual level; Lan. deficits, language deficits; Learn. diff, learning difficulties.
Epilepsy/febrile seizures.