Introduction: Deviation of the eyes may occur with seizures involving any cerebral lobe. Most notably, a lesion of the FEF that causes excess neural activity, like a focal seizure, or spreading of ictal discharges to the FEF will drive the eyes contralaterally during the period of the seizure. The head also may turn contralateral to the seizure focus during the ictus. In the post-ictal state, when there may be lingering hypoactivity of the FEF neurons, the eyes may deviate ipsilateral to the side of the lesion because of a relative increase in the input from the unaffected FEF on the opposite side of the brain. Methods: A 14- year old right handed boy with refractory epilepsy was admitted for video EEG monitoring in an attempt to better define his seizures and to evaluate him for possible epilepsy surgery. He had thirteen clinical seizures with different semiology. In one of these, he had disconjugated head and eye movement that is an unusual finding. In this investigation, we noted his brain MRI with and without contrast that reported focal signal abnormality in the right prerolandic region indicative of AV malformation and also abnormal intraaxial infratentorial cystic mass located in the left cerebellar peduncle. Results: In the present case, disconjugated head and eye movement occurred during the seizure, probably; due to brain stem cystic lesion that is mainly located in mesencephalic area that may interrupt the connections between FEF and superior colliculus. Conclusion: The patient studied in the present study experienced Rt. Side (ipsilateral) eye gaze with dis-conjugated left sided (contralateral) head version during his seizure. While long term video-EEG monitoring and imaging studies pointed to epileptogenic zone of right centroparietal region. This is probably due to a cystic lesion located in the mesencephalic region mainly in the left cerebellar peduncle. Manuscript profile

Introduction: Hyperekplexia, or startle disease, is a rare neurological disorder characterized by excessive startle responses to noise or touch stimulation that can cause serious injury from frequent falls. The disease is characterized by a triad of generalized stiffness while the patient is awake, nocturnal myoclonus, and an exaggerated startle reflex. Hereby, an interesting case of hyperekplexia with comorbidities is reported that was misdiagnosed as epilepsy for some years. Methods: Our patient was a 16-year-old right handed boy with frequent falls, without loss of consciousness that caused serious head trauma. He had exaggerated myoclonic jerks with tactile and auditory stimuli. Also, he had nocturnal attacks including a startle myoclonic jerk followed by sudden stiffening of the limbs and trunk with vocalization that lasted about 3 seconds without loss of awareness. There was a positive family history of jerky movements in his mother only when she was sleeping. He had a posttraumatic encephalomalacia in the right frontotemproparietal region due to one of his falls 10 years ago. He was diagnosed with epilepsy and was treated with valproate with no success. After total antiepileptic discontinuation and 10 days of video-EEG monitoring, no interictal epileptifrom finding was recorded. The attacks were not accompanied with ictal activity on EEG. Hyperekplexia may be misdiagnosed with startle epilepsy. The preservation of consciousness and absence of epileptiform discharges on EEG can be helpful for differentiating hyperekplexia from startle epilepsy. Startle epilepsy is often resistant to treatment; however, hyperekplexia usually responds well to clonazepam. Manuscript profile

Introduction: Long QT syndrome is a rare hereditary disorder that could be a potentially fatal condition. One of the symptoms of long QT caused by ventricular arrhythmia is seizure. The diagnosis of this syndrome might be delayed when an initial diagnosis of epilepsy is made. Case presentation: The patient to be studied in this research was a 24-year-old right handed female. She had the spells since she was 14; which were characterized by uncomfortable anxiety, nausea, pallor, and palpitation followed by generalized weakness and occasionally generalized clonic jerks with obvious impairment of consciousness. She was treated with Depakine and Carbamazepine. During the video-EEG monitoring, she had one habitual attack accompanied with ventricular tachycardia and cardiac arrest for which cardiorespiratory resuscitation was immediately started, and fortunately the patient returned to normal condition. Cardiac evaluation was requested and diagnosis of long QT syndrome was confirmed. Implantable cardiac defibrillator was placed for her. Conclusion: Long QT syndrome possesses considerable mortality decreased with proper therapy. Long QT syndrome imitates seizure disorders. Hence, taking electrocardiography is required for individuals with vague causes of seizure and uncommon semiology. Manuscript profile