Makindo Medical Notes

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Introduction

• Benign and self-limiting seizure disorder in children with focal seizures with autonomic features.
• With a good prognosis and spontaneous remission in most cases, treatment is usually not required
• Reassurance is key to managing the condition.
• Occurs in early and mid-childhood, with a peak incidence around the age of 5 years.
• It is one of the most common childhood epilepsies, causes 6% of all epileptic cases.

Clinical Features

• Age of onset: Typically seen in children between 1 and 8 years, with the peak age being around 5 years.
• Seizure characteristics: The seizures often occur at night and can involve autonomic symptoms such as vomiting, eye deviation, and impaired consciousness prior to the onset of convulsions.
• Seizure duration: Many seizures last for around 30 minutes, although some may last for several hours. Despite the prolonged duration, there is no permanent brain damage or neurological deficit.
• Autonomic symptoms: Vomiting and eye deviation are common, and these symptoms are often associated with impaired consciousness just before the convulsions begin.

EEG Findings

• Shifting and/or multiple foci: Often with a predominance of the occipital lobe.
• Occipital predominance: This is a characteristic feature of the syndrome, though not always present in all cases.

Diagnosis

• Diagnosis is primarily clinical and supported by EEG findings. The distinctive features of the seizures and the occurrence of autonomic symptoms, such as vomiting and eye deviation, are key elements in identifying the syndrome.
• A detailed medical history and neurological examination should be conducted to rule out other causes of seizures.

Prognosis

• The prognosis is generally excellent, as most children experience remission of seizures within 2 years of onset.
• There is no permanent brain damage or long-term neurological impairment associated with the disorder.

Treatment

• Given the high rate of remission, antiepileptic medication is often not necessary.
• The primary approach to management is reassurance, as the seizures tend to resolve spontaneously over time.
• Where seizures are prolonged or frequent, medications may be considered to control the symptoms until remission occurs.