A 10-year-old male with history of hydrops fetalis, developmental delay, learning impairment, and long-standing focal epilepsy, which had been solely subclinical since the age of two, presented with complaints of recurrent and painful 'tickles' in his right lower back.
The patient's epilepsy is thought to be due to hydrops fetalis. He was born at 35 weeks by emergent cesarean section due to fetal heart decelerations and maternal hypotension. He required resuscitation twice after birth, having APGARS of 1 at 1 min, 2 at 5 min, 1 at 10 min, 1 at 15 min, 5 at 20 min, 6 at 25 min, and 7 at 30 min. Head ultrasound shortly after birth was normal. The electroencephalogram (EEG) at that time was abnormal because of reduced reactivity and discontinuity : but showed no epileptiform abnormalities. Initially, his development was only slightly delayed. However, his cognitive skills were noted to be more significantly delayed when he started school and have continued to lag. He was diagnosed with learning disability.
He underwent formal neuropsychological evaluation at the age of eight. On the Wechsler Intelligence Scale for Children:Fourth Edition (WISC-IV), he performed in the extremely low range (full-scale IQ = 57) when compared with other students his age, placing him at the 0.2 percentile. His abilities were very variable, ranging from extremely low in perceptual reasoning and working memory to borderline in verbal comprehension and processing speed. He is currently in the 5th grade, attends a special school without tests, and has an Individualized Education Program (IEP). When examined in-clinic, he could recall numbers 1-20 and had a concept of 'greater/less than'. His articulation was good, though he spoke in simple phrases. He was able to communicate his basic needs, though he had a low frustration tolerance. He was unable to perform simple calculations, and his verbal skills were at kindergarten levels. Additionally, he displayed a very childish and immature demeanor. His most recent IEP reported similar findings. Overall, this profile is supportive of the fact that his ability to communicate his symptoms was limited.
Seizures were first diagnosed at age two. Clinically, seizures consisted of bilateral arm elevation and extension with brief shaking for 3-5 s. At initial presentation, he had 15-20 seizures per day. Valproic acid significantly reduced his seizures and nearly abolished all clinical seizures within 2 months. Over the next few years, he continued to have frequent subclinical seizures on EEG, but no further clinical events occurred. The subclinical seizures consisted of buildup of 50- to 200-muV, rhythmic 4-Hz sharp waves over the mesial frontal region (maximal at Fz and Cz and also seen at Fp1, Fp2, F3, and F4), which would last for 35-50 s. These occurred solely in sleep, with no clinical correlation. Several medication trials were attempted to better control these events, and at the time of presentation, he was on a combination of valproic acid, levetiracetam, and lacosamide. He has been on a variant of these three medications (though at varying doses) since the age of six. Further workup included a high resolution brain MRI, which showed no abnormalities. Psychiatrically, at the age of eight, he endorsed suicidal ideation. Given these psychiatric concerns in addition to excessive drowsiness in the setting of an improved EEG, his levetiracetam dose was reduced. Vitamin B6 was additionally started, and he began psychotherapy. This resulted in significant improvement in his mood and behavior.
He initially presented with his parents to his pediatrician and pediatric neurologist with complaints of odd new episodes. The episodes started after returning from a three-week vacation. During the episodes, the patient was noted to be restless, hyperactive, and upset. He would scratch his back and, at times, scream as well as run around the house. He complained that his back tickled and itched uncomfortably. These events were initially considered psychogenic based on the following: lack of complete stereotypy, no clinical seizures since the age of 2, history of behavioral problems, and the stresses associated with his parents enforcing limits on him. Additionally, given the presence of frontal epileptiform activity on prior EEGs, a sensory seizure did not appear to fit with the clinical symptoms.
Over time, his spells occurred with increasing frequency, happening multiple times per day including in association with arousal from sleep. He was seen in-clinic, and a decision was made to admit him for continuous video-EEG monitoring.
His cEEG showed frequent subclinical mesial frontal seizures, as he had exhibited previously. During the tickling events, there was initially so much movement it was not possible to tell whether these were behavioral or ictal in nature. Behavioral management to help him relax was provided. Additionally, he was started on clonidine at night to aid with sleep and to medically treat the outbursts. This resulted in improvement in the level of distress of the patient and the family, as the behaviors interfered less with the patient's daily functioning, though the frequency of the tickling attacks remained unchanged.
After these modifications reduced the emotional upset and hyperactivity associated with the tickling attacks, the stereotyped nature of these events became more apparent and the ictal EEG features more discernable. The patient reliably complained of itching of the right middorsal region, which suggested a more posterior focus than was apparent on EEG. To better define the potential epileptogenic and symptomatogenic zones, double-density electrodes were placed over the bilateral frontal and parietal head regions. He had numerous events per day and, at times, numerous events per hour, resulting in an EEG that showed 130- to 180-muV spikes and sharp waves over the left frontocentral region (FC3 > F3, Fz, FCz) which built up into rhythmic, sharply contoured slowing (1-4 Hz, 200-300 muV) over the left frontocentral region (Fp1, F3, C3). At times, this activity would propagate to the right frontocentral region (Fp2, F4, C4) as well. Clinically, the patient cried out "it tickles" consistently. Occasionally, right shoulder jerking or rubbing/scratching of the right side of his back were associated with his cries. These runs of epileptiform discharges were only seen during the tickling attacks, thus confirming them as true ictal phenomena.