Ed Susman

Anthony, a teenager, and Olivia, a mother of two young children, had a few things in common: They had epilepsy that was difficult to control, and they both died alone, probably as a result of a seizure.  

Their stories, said Elizabeth J. Donner, MD, assistant professor of neurology, University of Toronto, are representative of the devastating cases of sudden, unexpected death in persons with epilepsy (SUDEP). 

“Sudden death is 24 times more likely in someone with epilepsy than in the normal population,” Dr. Donner said in her plenary lecture “Sudden Unexplained Death in Epilepsy,” at the 61st Annual Meeting of the American Academy of Neurology.   

She described the case history of Anthony who had an uncomplicated, unremarkable delivery but began developing staring spell-type seizures at age 3 years. By age 12 years, Anthony was experiencing multiple seizures, including generalized tonic clonic seizures as often as 8 times a day. He was diagnosed with Lennox-Gastaut Syndrome and proved medically intractable. Although also autistic and still suffering from cluster headaches, Anthony was attending school in a special needs 10th grade class. At age 16 years, his mother found him face down in his bed one morning. Resuscitation failed.  

Olivia also had an uncomplicated pregnancy and was well until seizures were experienced when she was age 11 years. By age 19 years, her seizures were worsening, requiring multidrug treatment, but she was less than compliant, due to adverse effects of the medicine. Despite the seizures, which were poorly controlled, Olivia married, had two children, and held an office manager position. A room was set aside for her use if she felt poorly. When she was age 32 years, she went to the room, and when she didn’t return after two hours, co-workers went to the room and found her dead.   

Dr. Donner said that while some people succumb to epilepsy because the seizure occurs while swimming or doing some other activity in which the seizure is responsible for the death, Anthony and Olivia appear to be cases of SUDEP. 

“Epilepsy affects 1% of children,” Dr. Donner said, “but SUDEP accounts for 34% of sudden deaths in childhood.” And she said that SUDEP may be under-reported because coroners and medical examiners may not be aware of the condition. Autopsy rates in these cases are low, she noted.  

She said her studies have estimated that SUDEP occurs in 0.2 per 1000-patient years in children.  

Dr. Donner said risk factors of SUDEP include epilepsy severity, increased frequency of generalized tonic clonic seizures, longer duration of epilepsy, the use of polytherapy, and the lack of medication.  

“Over the last few generations, we have emphasized that people with epilepsy can live nearly normal lives,” said Andrew Wilner, MD, an epileptologist in Newport, RI, “but we have to remember that people do die of this disease.”  

Dr. Wilner emphasized that “those at most risk of fatality are people with intractable seizures.”

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"Sudden death is 24 times more likely in someone with epilepsy than in the normal population," Elizabeth J. Donner, MD, assistant professor of neurology, University of Toronto said in her plenary lecture "Sudden Unexplained Death in Epilepsy."

Sudden Death Still a Threat to People with Epilepsy

In a striking case report, the stimulant modafinil was able to dramatically improve neurological functioning in an elderly woman suffering from bilateral thalamic infarction, a disorder that often is fatal.  

“This woman slept for long hours and even when she was awake, she was very lethargic,” said Kristina Darnauer, MD, a resident in family practice at the Research Medical Center, Kansas City, MO.  

Lethargy, extended sleep, sleepiness and coma often occur in patients with bilateral thalamic infarction caused by hypoperfusion of the thalamo-perforating arteries, explained Dr. Darnauer at her poster “

Bilateral Thalamic Infarction Treatment with Modafinil

,” presented at the 61st Annual Meeting of the American Academy of Neurology.  

The disorder interferes with the body’s arousal on switch. “People go to sleep, and they just don’t wake up,” Dr. Darnauer said. The condition results in a high degree of mortality and morbidity from stupor and coma.  

In the case she presented, an 82-year-old woman was hospitalized after being observed in a stuporous condition for two days. The women had a history of diabetes, hypertension, and peripheral artery disease, but her serum chemistries and arterial blood chemistries were normal. A magnetic resonance imaging study detected acute infarction of the medial thalamus.  

On the third day of hospitalization, doctors began administering modafinil. “In the next few days, the patient showed improvement,” Dr. Darnauer said. “She was able to stay awake, engage in conversations, and became independent in all her activities of daily living.”  

The treatment appears to be necessary for normal functioning, she said. “At one point, the woman did not receive modafinil for two days and she reverted back to deep stupor,” Dr. Darnauer said. When modafinil was re-instituted, the woman recovered.  

She said the cause of bilateral thalamic infarction is basically due to atherosclerosis/embolism in the vertebro-basilar system. The thalamo-perforating arteries are branches of the vertebro-basilar arteries. “The clinical phenomenon may vary by the location of the infarction,” she said, “but somnolence is invariably present. Hypersomnolence is mediated by damage to common pathways of arousal that traverse through the thalamus.  

“Drugs such as modafinil and other stimulants causes wakefulness through the intricate adrenergic/hypocritin system, and can result in meaningful recovery,” she added.  

The cerebrovascular accidents that create bilateral thalamic infarction are rare events, Dr. Darnauer said. “However, with treatment the sleep/wake cycle can be normalized, resulting in better neurological and functional outcomes.”

In a striking case report, the stimulant modafinil was able to dramatically improve neurological functioning in an elderly woman suffering from bilateral thalamic infarction, a disorder that often is fatal.

Modafinil Shakes Patients from Thalamic Infarction Lethargy

Getting people who are having an acute stroke to the hospital in time for thrombolytic therapy—a window of opportunity that is about 3-4.5 hours wide&mdash;appears to be thwarted by a variety of barriers, including the desire of a the patient to avoid being embarrassed an ambulance showing up at the front door.   

“Sometimes, patients just don’t seem to think their condition is serious enough to go to the hospital, or they think the symptoms will go away on their own,” said Amanda B. Castle, BA, a physician’s assistant student at Emory University in Atlanta, GA, in her poster, “

Barriers in Seeking Early Stroke Treatment: Assessment of an Urban Underserved Population

Castle and colleagues at Washington Hospital Center in Washington, DC, interviewed 100 patients within 48 hours after being admitted to the hospital for stroke symptoms to ask them reasons for their delay in getting medical help. “Only 32% of the patients interviewed thought they were having a stroke,” Castle said.  

Overall, 70% of the patients said that one reason for not seeking immediate help was that they didn’t think their symptoms were serious. She said that 68% of the patients thought their symptoms would get better without seeking medical help. About 29% of the patients said they didn’t call 911 because they would have been embarrassed to have emergency medical services and an ambulance appear at their home. About 23% of the people didn’t think that it would make any difference if they got to the hospital early or not. And 19% called their primary care physician first and waited for the doctor or a nurse to call back with instructions.  

“These are very familiar reasons for failing to get treatment early,” said Andrew McKeon, MD, a fellow in neurology at the Mayo Clinic, Rochester, MN. “For many people, stroke is painless. When you have crushing chest pain, you tend to call for help more quickly.”  

Dr. McKeon said that stroke patients who have sudden vision loss are more likely to call for an ambulance than those individuals who have a numbness or weakness in an arm, and put off calling for help until the stroke progresses to the point that they cannot walk or have other symptoms.  

He said that continued education campaigns will be necessary to get patients to the hospital sooner so that they arrive within the window of opportunity for thrombolytic therapy. 

“By identifying these barriers,” Castle said, “we can tailor interventions that have the potential to significantly increase the number of patients receiving effective acute therapies.”  

She noted that only about 3-5% of stroke patients get to a hospital soon enough to even be eligible for thrombolytic treatment.

Getting people who are having an acute stroke to the hospital in time for thrombolytic therapy appears to be thwarted by a variety of barriers, including the desire of a the patient to avoid being embarrassed an ambulance showing up at the front door. 

Ed Susman

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