• • Febrile Seizures
  • Benign Rolandic Epilepsy
  • Juvenile Myoclonic Epilepsy
  • Infantile Spasms
  • Lennox-Gastaut Syndrome
  • Childhood Absence Epilepsy
  • Benign Occipital Epilepsy
  • Mitochondrial Disorders
  • Landau-Kleffner Syndrome
  • Rasmussen Syndrome

Hypothalamic Hamartoma & Epilepsy

What is Lennox-Gastaut Syndrome?

Lennox-Gastaut syndrome is a severe form of epilepsy. Seizures usually begin before 4 years of age. Seizure types, which vary among patients, include tonic (stiffening of the body, upward deviation of the eyes, dilation of the pupils, and altered respiratory patterns), atonic (brief loss of muscle tone and consciousness, causing abrupt falls), atypical absence (staring spells), and myoclonic (sudden muscle jerks). There may be periods of frequent seizures mixed with brief, relatively seizure-free periods. Most children with Lennox-Gastaut syndrome experience some degree of impaired intellectual functioning or information processing, along with developmental delays, and behavioral disturbances. Lennox-Gastaut syndrome can be caused by brain malformations, perinatal asphyxia, severe head injury, central nervous system infection and inherited degenerative or metabolic conditions. In 30-35 percent of cases, no cause can be found.

Is there any treatment?

What is the prognosis?

What is Dravet Syndrome?

Dravet syndrome, also known as Severe Myoclonic Epilepsy of Infancy (SMEI), is a rare and catastrophic form of intractable epilepsy that begins in infancy. Initial seizures are most often prolonged events and in the second year of life, other seizure types begin to emerge. Development remains on track initially, with plateaus and a progressive decline typically beginning in the second year of life. Individuals with Dravet syndrome face a higher incidence of SUDEP (sudden unexplained death in epilepsy) and have associated conditions, which also need to be properly treated and managed. These conditions include:

• • behavioral and developmental delays
  • movement and balance issues
  • orthopedic conditions
  • delayed language and speech issues
  • growth and nutrition issues
  • sleeping difficulties
  • chronic infections
  • sensory integration disorders
  • disruptions of the autonomic nervous system (which regulates things such as body temperature and sweating)

– http://www.dravetfoundation.org/dravet-syndrome/what-is-dravet-syndrome

What is the Treatment?

Unless a cure or better treatments for Dravet syndrome and related epilepsies are found, individuals with these disorders face a diminished quality of life. Current treatment options are extremely limited and the prognosis for these children is poor. The constant care and supervision of an individual with such highly specialized needs are emotionally and financially draining on the family members who care for these individuals.

What is the Prognosis?

Children with Dravet syndrome do not outgrow this condition and it affects every aspect of their daily lives.

What is the SCN1A Gene?

The SCN1A Gene encodes a protein that is vital for the creation of the vertebrate sodium channel. The vertebrate sodium channel is a voltage-gated ion channel essential for the generation and propagation of action potentials, mainly in nerve and muscle.

Mutations in the SCNA1 gene cause inherited febrile seizures and GEFS+, type 2.

Is there any treatment?

Symptoms of SCN1A gene mutations are usually treated with Anti-Epilepsy Drugs, (AEDs). Antiepileptic drugs (AEDs) include benzodiazepines (diazepam and clonazepam), topiramate, and valproic acid. Phenobarbital is effective but poorly tolerated because of its effects on cognition.

Prevention of secondary complications: Use of protective helmets by individuals with atonic seizures or myoclonic-astatic epilepsy.

Surveillance: Serial neuropsychological evaluation for neurologic, cognitive, and behavioral deterioration; EEG monitoring for new or different seizure types.

Agents/circumstances to avoid: AEDs: carbamazepine, lamotrigine, and vigabatrin, which can induce or increase myoclonic seizures; phenytoin, which can induce choreoathetosis. Activities in which a sudden loss of consciousness could lead to injury or death (e.g., bathing, swimming, driving, or working/playing at heights).

Other: The AEDs clobazam and stiripentol, used in the treatment of SMEI, are not FDA-approved for this use in the US. Sleep deprivation and illness can exacerbate seizures. Persons with epilepsy should be made aware of motor vehicle driving laws.

What is the prognosis?

The phenotype varies even among family members with the same mutation. Figure 1 shows a pedigree that demonstrates variable expressivity. As a result of this variable expressivity, lthe ong-term prognosis is difficult to predict.

People with epilepsy need to take special precautions when traveling. The Traveller’s Handbook for People with Epilepsy, which has recently been updated, is a very useful booklet for anyone undertaking a trip. It contains general advice for travelers, covering such topics as accommodation, insurance, driving and getting around. It also includes a set of First Aid instructions, reminders regarding medication and a very practical selection of phrases.

What are the options?

Yes, a person with epilepsy can obtain a driver’s license, however, Florida, like every state in the United States, has specific guidelines concerning driver’s licensure for medically impaired individuals including those with epilepsy. Considerable differences exist among the states in length of time a patient must be seizure free in order to drive. In some states, the person must wait two years; in others, only six months.

Florida guidelines

• The guideline states that applicants and licensed drivers should be seizure free for a period of two years before being approved for licensing; but if under regular medical supervision, may apply at the end of six months for review by the Medical Advisory Board. Absence seizures, partial and complex partial seizures will also follow these guidelines. The isolated seizure with a normal electroencephalogram may be reviewed at the end of three months.
• Applicants and licensed drivers who have been approved after six months seizure free may be required to submit follow-up reports at the end of one year from date of approval.
• Applicants and licensed drivers who had a chronic recurring seizure disorder (or have been treated for such for one year) and medications have been discontinued will not be licensed to drive during the period of drug withdrawal and for a period of three months following complete cessation of treatment. If the patient has seizures during this period, licensing may be considered after a three-month seizure-free interval upon return to adequate therapy.
• If there is a question about the seizure type or the medication the applicant or licensed driver is on, it is the prerogative of the Medical Board to question the treating Physician further in an effort to clarify the nature of the seizures.
• Blood levels below therapeutic levels are to be considered on an individual basis.
• Applicants and licensed drivers with only chronic nocturnal seizures will be considered on an individual basis.
• Applicants and licensed drivers with syncopal episodes who have no clear diagnosis established will be considered on an individual basis.

Procedures

The procedure is the same for all residents of Florida. Application is made at the local driver license office. A Road Signs and Road Rules examination will be given. When a person indicates on there application that he has had a seizure within the past two years a medical form is mailed to the applicant from Tallahassee. The applicant is responsible for getting this form to his physician. The Physician should complete the form and return it to the Medical Review section in Tallahassee. If the applicant passes both the written and the driving portion of the exam and approval is received from the Division of Driver Licenses, then a drivers license will be issued.
Who decides?
The final decision about a person’s medical ability to drive is made by the division of Driver Licenses. The decision, based upon the recommendation of the Department’s Medical Advisory Board, is made after the review of information provided by the physician treating the person with epilepsy.

Physician requirements

In Florida, Physicians are not required to report a patient with epilepsy to the Division of Driver Licenses. The moral obligation is left to the patient. It is very important for the person with epilepsy to discuss driving recommendations with his Physician.

Denied driving privileges

If driving privileges are denied, the applicant has a right to appeal. This process consists of a hearing, during which the applicant may present evidence that he is capable of driving and may bring witnesses to testify on his behalf. The request for appeal must be made in writing to the Division of Driver Licenses.

Medical updates

Yes. The Division reviews cases of drivers with seizure disorders at varying periods of time.

Classroom driver education for students with epilepsy

Students with epilepsy interested in taking drivers education should contact the local school board office and obtain class requirements before enrolling in the class. In some areas, a restricted license may be required to obtain full credit for the class.

Recommendations

• Avoid driving when tired and do not drive for prolonged periods of time. Employment that requires long hours of driving is not recommended.
• Take care not to go for long periods of time without food or sleep.
• Take seizure medication regularly. If treatment is changed or stopped, be aware that this may interfere with your fitness to drive. At the time such changes are made, it may be wise to limit driving or to stop altogether for a period of time. Discuss this with your physician.
• Seizures of short duration can be as important as those of longer duration when it comes to driving. All episodes should be reported to your physician.
• It is important for someone with epilepsy to drive under the best conditions. Anyone with a history of epilepsy should be sure to avoid drinking alcohol before driving. Not only does alcohol impair driving but it may also cause a seizure which might not occur otherwise.