Case Files Neurology Flashcards
What is the most common focal epilepsy of childhood and what is its cause?
The most common focal epilepsy of childhood is Benign Rolandic Epilepsy (BRE). It is highly heritable although the mode of inheritance remains unclear.
The typical onset of BRE is at the age of …?
The typical onset of BRE is between 3 and 13 years of age.
What is the typical seizure presentation with BRE like?
The typical seizure presentation with BRE begins with sensorimotor manifestations on one side of the face and mouth (unilateral oral paresthesias and facial clonic or tonic activity).
Speech arrest, which can be seen regardless of whether the seizure involves the dominant or non dominant hemisphere, and hyper salivation are also frequently reported. Most of the seizure activity is at night.
When is a seizure referred to as “generalized”?
When it involves both cerebral hemispheres.
When is a seizure referred to as “focal” or “partial”?
When it begins in one part of one hemisphere.
What distinguishes “complex partial seizures” from “simple partial seizures”?
In complex partial seizures the consciousness is impaired, in simple partial seizures it is not.
When is a seizures said to be “secondarily generalized”?
It is not uncommon for partial seizures to spread and involve a greater cortical region as the seizures progresses. If this abnormal activity spreads to involve the contralateral hemisphere then the seizure is said to be secondarily generalized.
How can partial seizures be categorized according to their underlying cause?
1) Symptomatic:
With anatomical substrate (e.g. tumor, malformation, stroke, injury etc.)
2) Cryptogenic:
Cause remains hidden.
3) Idiopathic:
There is a known or presumed genetic etiology.
BRE belongs to which category of partial seizures?
BRE is a considered a idiopathic partial seizure as it is highly heritable although the mode of inheritance is unclear.
What is “semiology” of a seizure?
Semiology refers to the behavioral manifestation of focal seizures.
Why is BRE labeled “benign”?
BRE is labeled benign for following reasons:
1) Initially all patients will cease having their habitual seizures by 20 years of age
2) Two thirds of patients will have only one or very few seizures
Is it helpful in making the diagnosis of BRE to obtain an EEG? Explain your answer!
Obtaining an EEG is helpful in a diagnosis of BRE because there are characteristic feature that can be seen interictally. If a period of sleep is captured during the recording session, approximately 30% of patients with BRE will have characteristic centrotemporal sharp waves.
How many patients with EEG abnormalities consistent with BRE actually experience seizures?
Only around 10% of patients.
Why do many neurologists withhold anticonvulsive treatment in BRE until a patient has experienced at least there or more seizures?
1) Most patients only experience one or very few seizures
2) All patients eventually outgrow BRE
3) There is no evidence that the typical seizures of BRE are harmful to the developing nervous system
4) Most anticonvulsants have considerable side effects
Which anti convulsive agents are frequently used as first-line agents in BRE?
Oxcarbazepine and Gabapentin
What is the most common focal epilepsy in adults?
The most common focal epilepsy in adults is temporal lobe epilepsy.
A “malignant rolandic epilepsy” has been described in literature. What does this term refer to?
A small subset of patients have been described to have seizures similar to those in BRE but are frequent, difficult to treat and do not remit by 16 years of age. It is likely that this patients have a different syndrome rather than a severe form of BRE.
What is “Benign Epilepsy of Childhood with Occipital Paroxysms”?
A syndrome with seizures with visual symptoms, followed by psychomotor, sensorimotor, or migraine-like phenomena. EEG reveals occipital spikes that go away with eye-opening. It is much less common than BRE.
