Reference | Age at onset (years)/Gender | Trigger | Spontaneous seizure | Intellectual disability | Semiology features | Brain imaging | Inter-ictal EEG | Ictal EEG | Anti-seizure medicine | Outcome |
---|---|---|---|---|---|---|---|---|---|---|
Rathore [2] | 3/M | Micturition | – | – | Forward flexion of the upper trunk and extension of the upper extremities | – | – | Beta burst at the Cz electrode | / | / |
Viswanathan [3] | 4/M | Micturition | + | + | Slow neck flexion, eye deviation to the right and posturing of the upper limbs | MRI: normal; Inter-ictal MEG: equivalent current dipoles clustered in the left midline posterior parietal region | Multiple foci | Fast activity in the midline centroparietal leads that culminated in the bilateral centroparietal region | Lamotrigine and valproate | Complete cessation |
Zivin [4] | 13/M | Micturition | – | – | Stiffening, lack of awareness and automatic activity | – | Right temporal foci | Slow waves and irregular dysrhythmic pattern in all the leads | Primidone | Remission |
Bourgeois [5] | 5/M | Micturition and immersion of feet in hot water | + | + | Sensation of pins and needles in the buttocks, staring, drooling, vocalization and fall | MRI: Chiari Type I malformation | Multiple foci | Onset in Cz | Carbamazepine and valproate | Partially controlled |
Higuchi [6] | 6/F | Micturition and defecation | + | + | Extension of the arms and rhythmic jerking in a conscious state | – | Spike-and-wave activity on central electrode recording | Rhythmic theta waves at the central electrodes | Clobazam and phenytoin | |
Yang [7] | 12/M | Micturition | – | + | Forward flexion of the upper trunk and eye deviation to the right | – | Bilateral multiple foci | Unclear activity | Oxcarbazepine | Remission |
Okumura [8] | 8/F | Micturition | + | _ | Speech arrest, vocalization and extension of the upper extremities with preservation of consciousness | MRI: normal Subtraction ictal SPECT: mesial frontal | Normal | Fast waves without clear focal features followed by frontal dominant rhythmic multiple spikes | Phenytoin | Remission |
Seth [9] | 9/F | Micturition | – | – | Sense of fear, loss of awareness, tonic posturing and automatism | – | Possible left frontal slowing | Unclear activity | Lamotrigine | Remission |
Whitney [10] | 11/F | Micturition | + | + | Head and eye deviation to the right, flexed dystonic posturing of the limbs and eventual generalized tonic-clonic seizures | – | Multiple foci | Rhythmic theta activity in Cz followed by generalized activity | Lacosamide and clobazam | 1.5 months without seizures |
Glass [11] | 10/F | Micturition and prayer | + | + | Staring, deviation of the eyes and head to the left and rhythmic clonic activity of both arms | MRI: normal Ictal SPECT: hyperperfusion in the anterior cingulate gyrus and anterolateral right frontal lobe | / | 20–22 Hz rhythmic epileptiform activity at Cz, and spread to the bilateral frontal regions | Phenobarbital, valproic acid, clonazepam, topiramate, lamotrigine, clobazam, and ketogenic diet | Refractory |
Cvetkovska [12] | 9/M | Micturition and getting startled | + | Borderline | Tonic posturing, and occasional loss of body tone and fall | MRI: Focal cortical dysplasia in the right middle frontal gyrus | Focal rhythmic epileptiform discharges at F4/Fp2/Fz | Fast-spike discharges over the right lateral frontal region followed by generalized discharges | / | / |
Rho YI [13] | 7/M | Micturition | + | + | Hand automatism, secondarily generalized tonic posture and loss of consciousness | – | Generalized burst of spikes and slow complex waves, predominantly on both frontocentral areas | Onset from the left frontotemporal region and spread to the right frontotemporal region | Valproate sodium | Remission |
Casciato [14] | 18/M | Micturition | + | – | Genital pain followed by manipulation with preserved awareness; head deviation to the right and arm/truncal asymmetric tonic posturing, followed by bilateral tonic-clonic seizures | – | Spiking over the left fronto-temporal regions | Onset of rhythmical delta activity evolving into sharp waves over the midline involving the central-parietal and left fronto-temporal channels, with the recruiting rhythm evolving into a bilateral tonic-clonic seizure | Lacosamide | Seizure free for 3 months |
Present case | 8/F | Micturition | + | + | Speech arrest, deviation of the head and eyes to the left, tonic posturing, brief jerking and impaired awareness | MRI: Hydrocephalus | Generalized slowing background | Fast waves without clear focal features | Valproate sodium and levetiracetam | Partially controlled |