Landau-Kleffner syndrome

The article "Multiple subpial transection for Landau-Kleffner syndrome" is the basis for this AORN Journal independent study. The behavioral objectives and examination for this program were prepared by Janet S. West, RN, BSN, CNOR, clinical editor, with consultation from Susan Bakewell, RN, MS(N), professional education specialist, Center for Perioperative Education.

A minimum score of 70% on the multiple-choice examination is necessary to earn two contact hours for this independent study. Participants receive feedback on incorrect answers. Each applicant who successfully completes this study will receive a certificate of completion. The deadline for submitting this study is Sept 30, 1996.

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BEHAVIORAL OBJECTIVES

After reading and studying the article on multiple subpial transection (MST) for Landau-Kleffner syndrome (LKS), the nurse will be able to (1) discuss signs and symptoms of LKS in children, (2) describe preoperative evaluation testing for children with LKS, (3) discuss perioperative care for children undergoing MST procedures, and (4) describe perioperative nurses' roles when caring for children undergoing MST procedures.

Landau-Kleffner syndrome (LKS) in children was first described in a 1957 clinical study as an acquired childhood epileptic aphasia associated with seizure activity and severe electroencephalogram (EEG) abnormalities.(1) Two epileptologists studied five children who previously had normal speech but then developed stuttering onsets of aphasia with striking EEG abnormalities. A follow-up clinical study of the original five children and four additional children reported that four of nine children had spontaneous speech recovery and were able to lead normal lives.(2)

More recent clinical studies, however, suggest that most children with LKS do not have spontaneous speech recovery, and if speech is recovered, the children suffer severe language disorders.(3) Traditional medical treatment for LKS has been pharmacological management, but a review of the literature suggests that anticonvulsant medications have little effect on speech recovery.(4) In 1988, epilepsy team members at Rush-Presbyterian-St Luke's Medical Center, Chicago, implemented a technique for surgical treatment of LKS known as multiple subpial transection (MST).(5)

PEDIATRIC PATIENT PROFILE

Landau-Kleffner syndrome commonly appears between the ages of three and seven years in children who already have developed age-appropriate speech.(6)

Acquired aphasia. The first symptom parents report is that their children do not respond appropriately to language (ie, receptive aphasia). Parents often initially attribute this lack of response to inattentiveness. Eventually, the lack of verbal response becomes severe enough that parents suspect hearing loss. Speech output eventually is affected, and over time, children lose their ability to communicate effectively. Some children even lose their ability to recognize nonverbal sounds, such as a telephone's ring, a knock on the door, or a dog's bark (ie, auditory agnosia).(7)

Brain wave activity. This loss of language function is correlated with severe EEG abnormalities. The EEG pattern of children diagnosed with LKS is most virulently abnormal in the deep stages of sleep. The pattern is best described as a continuous or near continuous bilateral spike and wave activity primarily focused in the posterior temporal regions of the brain.(8) Epileptologists do not understand how this continuous spike and wave activity affects brain development, however, they do know that the constant abnormal electrical activity during sleep interrupts the brain's ability to develop normal language function.

Seizures. Clinical seizure activity has been reported in most children diagnosed with LKS.(9) The type of seizure activity varies, but most children are described as displaying eye blinking, brief ocular deviations, head dropping, or as having occasional generalized, tonic-clonic seizures.(10) There does not seem to be any relationship between the type of seizures and the loss of language function.(11)

Seizure activity may begin at the onset of children's loss of language functions, or parents may notice seizure activity after their children have been diagnosed with LKS. Seizure activity may be so subtle that parents do not notice their children are undergoing seizures. Seizure activity in children with LKS has a benign course that is treated easily with anticonvulsant medications, and seizures usually cease in adolescence.(12) Although clinical seizures and gross EEG abnormalities may end during adolescence, language function rarely returns to normal, and EEG abnormalities leave devastating developmental disabilities that do not improve with age. Most children with LKS become permanently disabled and unable to lead normal lives.(13)

TRADITIONAL MEDICAL TREATMENT

Traditional medical treatment for LKS has been pharmacological management.

Antiepileptic medications. Physicians traditionally have treated children diagnosed with LKS with anticonvulsant medications (eg, carbamazepine, divalproex sodium). Several researchers suggest that seizure activity in children with LKS has a good response to anticonvulsant medications.(14) Most anti-convulsant medications, however, do not eliminate the continuous spike and wave activity of EEGs seen in slow-wave sleep activity and do not improve children's language functions.

Corticosteroids. The use of corticosteroids (eg, prednisone) in children with LKS results in improved EEGs and speech.(15) If children are treated long enough with corticosteroids, many of them show improved control of the epileptic activity and improvement in speech. Unfortunately, long-term use of corticosteroids has multiple and serious side effects, such as * growth retardation, * cushingoid features, * voracious appetites, * development of steroid-induced diabetes, * osteoporosis, and * development of myopathy and cataracts.

If physicians attempt to discontinue the use of corticosteroids in children with LKS, abnormal EEG activity returns and worsens and is followed by deterioration of language functions.(16)

SURGICAL TREATMENTS

The most common surgical treatment of focal epilepsy is surgical resection of the seizure focus; however, this procedure is not an option when the focus is located in a critical area of the cerebral cortex (eg, language function). The MST procedure was developed to provide an alternative to surgical excision of brain tissue, to preserve critical areas of higher neurological functions, and to provide patients a surgical alternative when focal resection is not an option. The MST procedure eliminates the capacity of cortical tissue to generate seizure activity but preserves normal cortical functions (eg, speech, movement, primary sensation, memory).(17)

PREOPERATIVE PATIENT EVALUATION

Children diagnosed with LKS undergo extensive preoperative evaluations to establish the appropriateness of MST procedures. Electroencephalographic evaluations involve clinical electrical brain activity recordings (ie, seizures, waking and prolonged-sleep EEGs). Preoperative evaluations require several days of hospitalization and frequently involve physician-supervised, gradual withdrawal of children's antiepileptic medications to enhance seizure activity.

Nursing care. The preoperative evaluation period is a stressful time for the child and family members. Open communication between health care providers and family members decreases some of the anxiety of the hospital admission. The neurology unit nurse is the primary coordinator for communication between health care providers and family members, and the same nurse is assigned to the child throughout his or her hospital stay.

Before the child is admitted to the hospital, a clinical nurse specialist from the epilepsy center and the head nurse or the clinical nurse coordinator from the neurology unit have frequent contacts with family members to facilitate open communication. Preadmission telephone calls from the clinical nurse coordinator allow family members to ask questions and provide primary care nurses with information for individualized patient care plans. The clinical nurse coordinator also is responsible for contacting health care providers in the child life and dietary departments about the child's admission date so they can be available to meet the special needs of a child with LKS.

Preoperative teaching. The clinical nurse specialist from the epilepsy center and the neurology unit nurses address the education and emotional needs of the child and family members throughout the hospitalization period. These nurses use visual aids, involve the child in decision making whenever feasible, and give appropriate emotional support to the child and his or her family members. For example, they explain every step of preoperative evaluation testing to decrease the child's and family members' anxiety levels. In addition, nurses address family members' concerns about the child's sedation for preoperative evaluation testing. If family members identify medications that have been used successfully in the past to sedate the child, the nurse assesses the appropriateness of family members' request for a particular sedative for an evaluation procedure, assesses the child's need for sedation, and gives his or her recommendations to the physician.

Neurology unit nurses prepare the child and family members the night before the MST procedure by allowing them to express their fears about surgery and by discussing preoperative preparation (eg, child's shower, NPO status). Nurses also spend time helping family members understand what to expect when they see the child after surgery (eg, presence of invasive lines, head dressing). If family members are made aware that the child will have a bandaged head, be deeply sedated, and actually may seem to be worse after the initial postoperative 24 hours, they will be more tolerant of the child's recovery period in the hospital. It is not unusual for a child's receptive/expressive aphasia to continue after an MST procedure; therefore, the clinical nurse specialist from the epilepsy center, the epileptologist, and the neurology unit nurses explain to the parents that the child will require speech therapy and that it may take several months for the child to redevelop his or her language skills.

Assessments. Neurology unit nurses continually monitor the child for * changes in neurological status, * increased anxiety, * decreased respiratory status with sedation during evaluation testing, * changes in hydration status and skin condition, and * the appearance of other medical problems.

Postictal examination. Postictal examinations are the responsibility of neurology unit nurses throughout the child's hospital stay. Postictal examinations provide physicians with important information (ie, child's language, cognition, motor abilities, memory, level of consciousness) immediately after seizure activity and help physicians localize the epileptic focus. Postictal examination of a child with LKS is difficult because the child usually has little or no language ability and often cannot follow directions. To compensate for the child's lack of speech, the neurology unit nurse identifies activities the child can readily engage in and uses these activities to complete his or her postictal examination. For example, if the child liked to work with a particular puzzle, the nurse would use this puzzle to test the child's postictal motor skills, cognitive abilities, and his or her ability to follow directions.

Electroencephalographic monitoring. After admission to the hospital, children who are being considered for MST procedures are placed in specially equipped video-monitoring rooms that allow continuous (ie, 24 hour) EEG recordings and on-line television monitoring of the children's behaviors. Epileptologists receive EEG recordings and view online television monitoring through split-screen displays, which also are delivered continuously to the nursing station on the neurology monitoring unit.

Children are observed continuously by EEG technicians and neurology unit nurses for several days. The primary care nurse ensures that the child and family members understand the procedure for continuous EEG monitoring (eg, lead placement, activity restrictions, postictal examinations) and reinforces explanations as needed. Preoperative EEG recordings establish that children with LKS have continuous or near-continuous spike and wave activity during sleep and that clinical seizures arise from a single focus. Children with LKS may have bilateral epileptic discharges from the posterior regions of their temporal lobes, which are seen on EEG recordings.

Methohexital suppression testing. If epileptic activity appears bilateral on EEG recordings, methohexital suppression testing is performed to document that the epileptic abnormality arises from a unilateral focus and that the epileptic activity in one side of the brain is driving the epileptic activity in the other region of the brain. Methohexital suppression testing also allows epileptologists to identify sources of epileptic foci.

During methohexital suppression testing, anesthesia care providers administer methohexital sodium in sufficient amounts to cause electrocerebral silence on EEG recordings. Epileptic cortical lesions are resistant to anesthesia-induced inhibition; therefore, cortical lesions' independent epileptic activities stand out against otherwise flat EEG recordings.(18) An anesthesia care provider's presence is required during methohexital suppression testing because the administration of methohexital sodium may cause these children to become apneic with deep anesthesia, and they may require mechanical assistance with their breathing.

Seizure protocol. The primary care nurse ensures the child will be safe during seizure activity by padding the side rails of his or her hospital bed, by allowing the child out of bed only with the assistance of health care providers or family members, and by providing emergency oxygen and suction setups at the bedside. Neurology unit nurses reinforce the initial seizure precaution instructions given by the primary care nurse with the child and family members at the beginning of each shift. If a clinical seizure occurs during preoperative monitoring, an alarm is triggered at the nursing station. A nurse who has undergone special training in treating epileptic seizures immediately enters the child's room and assesses the child's safety. He or she notifies the physician and then conducts a brief neurological screening of the child.

Neuropsychological and speech evaluations. Children undergo thorough preoperative neuropsychological and speech evaluations. Neuropsychological testing is performed by psychologists who are specially trained to differentiate between global cognitive delays (ie, as seen with pervasive developmental delays) and specific language-related deficits (ie, as seen with LKS). Speech testing is performed by speech therapists who are specially trained to establish the existence of acquired aphasia as opposed to speech delays related to other childhood syndromes.

Tomography and body scans. Other tests conducted during preoperative evaluations include magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT), or positron emission tomography (PET) scans. The MRI scan is used to rule out structural abnormalities, and the PET scan measures cerebral glucose metabolism rates (ie, children with focal epileptic lesions may display decreased local cerebral metabolism rates). The SPECT scan assesses cerebral blood flow (ie, interictally, focal epileptic areas have decreased blood flow, and ictally, there is increased blood flow).(19) These tests provide epileptologists further information about the precise location of each child's epileptic focus.

Patient selection criteria. Preoperative evaluation testing (eg, EEG monitoring, methohexital suppression testing, speech evaluations, tomography and body scans) allows neurosurgeons to determine if children meet inclusion criteria for MST procedures. A child is selected for an MST procedure if * the acquired aphasia has an acute onset, * he or she developed age-appropriate language before being diagnosed with LKS, and * the severe epileptiform activity is maximal in the temporoparietal region of the child's brain and is continuous or nearly continuous in slow-wave sleep recordings. A child is excluded from consideration for an MST procedure if he or she has * a normal sleep EEG, and/or * a global developmental delay that involves capacities other than those dependent on language.(20)

PREOPERATIVE PATIENT CARE

The circulating nurse meets the child and his or her family members before surgery to explain the MST procedure and the sequence of perioperative events. The nurse reassures the child's family members that communication will be continuous throughout the intraoperative period. The anesthesia care provider orders a mild preoperative sedative for the child before he or she is transported from the preoperative holding area to the OR. The type of sedation administered to a child undergoing an MST procedure is important because many sedatives inhibit electrical brain activities. For example, the preoperative administration of secobarbital can interfere with cortical EEG activity during surgery. Anesthesia care providers usually give narcotics such as fentanyl because narcotics do not interfere with electrical brain activities.

INTRAOPERATIVE PATIENT CARE

The circulating nurse acts as a patient advocate throughout the intraoperative period. Specific areas of concern include * maintaining patient safety by checking for proper positioning of pressure-point padding, the safety strap, and an electrosurgical unit dispersive pad; * ensuring the availability of necessary surgical instruments and supplies; * Updating the child's family members during the surgical procedure; and * limiting traffic flow in the OR during the surgical procedure.

The circulating nurse places egg crate foam on the OR bed before the child arrives in the OR. He or she also ensures that a temperature regulating blanket and overhead warming lights are available. The anesthesia care provider may or may not use the overhead warming lights depending on the child's age and weight. The scrub person assembles all necessary surgical instruments and supplies, which include a craniotomy tray, a powered neurosurgical drill, a fixation system for the cranial bone flap, and a subpial transector.

Induction and intubation. After the anesthesia care provider and the circulating nurse transport the child to the OR, they place the child in a supine position on the OR bed for the induction of general anesthesia. Two circulating nurses are present during the induction and intubation period. The primary circulating nurse assists the anesthesia care provider with patient positioning, attachment of hemodynamic monitors (ie, blood pressure cuff, electrocardiogram [ECG], pulse oximetry), and induction of general anesthesia, and the assisting circulating nurse attends to the needs of the scrub person.

The primary circulating nurse and the anesthesia care provider intubate the child with a single-lumen endotracheal tube. After verifying the proper positioning of the endotracheal tube, the anesthesia care provider places an esophageal temperature probe near the endotracheal tube. He or she then inserts a radial arterial line and a peripheral IV line into the child's wrist and forearm. The assisting circulating nurse leaves the OR after the induction of general anesthesia.

Positioning and prepping. The circulating nurse inserts a Foley catheter, connects the catheter tubing to a urine collection bag, and positions the urine collection bag to gravity drainage near the anesthesia care provider. The circulating nurse applies elastic bandages to the child's legs as an antiembolism precaution. The surgeon preps the child's scalp with povidone-iodine, if the child is not allergic to iodine. If the child is allergic to povidone-iodine, the surgeon preps the child's scalp with chlorhexidine gluconate.

After the neurosurgeon shaves the child's head, surgical team members (ie, surgeons, anesthesia care provider, circulating nurse) place the child in a modified three-point fixation device attached to a neurosurgical head rest. Surgical team members then place the child in a semilateral position by using blankets to support the child's thoracolumbar region. The circulating nurse places egg crate foam or a pillow between the child's legs, depending on the child's size and weight. Scrubbed team members drape the child's head for a craniotomy incision.

Goal of multiple subpial transection. The basic neuronal organizational structure of the cerebral cortex is columnar (Figure 1). Neural input and output and vascular supply are oriented vertically to the pial surface of the cerebral cortex. There also are horizontal synaptic communications between neurons in the cerebral cortex. Experimental evidence indicates that the synchronization of cellular discharges on which epileptic activity is based depends on these horizontal connections. Surgical disruption of the horizontal synaptic contacts with a transecting surgical instrument (Figure 2) does not seem to interfere significantly with neural input and output. The MST procedure is designed to surgically eliminate horizontal intracortical fibers greater than 5 mm in length and to preserve vertical fibers that are necessary to send and receive neural messages.(21)

Intraoperative electrocorticography. Neurosurgical team members (ie, neurosurgeon. neurologist, epileptologist, EEG technician) work together to perform an intraoperative electrocorticography procedure. Intraoperative electrocorticography records electrical impulses from the child's brain through electrodes that are placed directly on the cerebral cortex. After the neurosurgeon opens the dura, he or she places a sterile 20-contact grid on the child's cerebral cortex. While the EEG technician and the epileptologist monitor the electrocorticography recordings, the neurologist maps the area of electrical abnormality, and the neurosurgeon identifies the affected area of abnormal electrical activity on the cerebral cortex. The neurosurgeon uses corresponding numbers on the 20-contact grid to mark the affected areas on the cerebral cortex (Figure 3). The neurologist uses several montages (ie, different EEG configurations) until he or she is satisfied that the epileptic zone has been identified correctly.

Multiple subpial transection procedure. The neurosurgeon completes the initial skin incision and creates a large temporoparietal cap and bone flap. He or she makes a pinhole-sized opening in the pial layer of the cortical cortex by using the point of a #11 surgical blade. The neurosurgeon introduces a subpial transector (ie, a right-angled hook made of heavy surgical steel wire) through the pial opening and sweeps the transector forward, dipping underneath the gyral crown. He or she uses the transector to dissect large horizontal intracortical fibers in the pia mater by raising the transector's tip at the far end of the gyrus so that it is visible beneath the pia mater. He or she then gently draws the subpial transector straight back across the gyrus (Figure 4).

The surgeon repeats the subpial transections every 5 mm in the cortical area mapped by intraoperative electrocorticography. The repeat transections eliminate the horizontal fibers and reduce the possibility of synchronized cell discharge (ie, seizure activity).(22) Elimination of horizontal fibers interrupts the ability of the epileptic focus to spread or evolve into a clinical attack because neighboring neural cells no longer can be recruited. Normal cortical functions remain intact because the neurosurgeon preserves vertical fibers.(23)

After the neurosurgeon completes the MSTs, he or she places a small piece of absorbable gelatin sponge soaked in topical thrombin over the entry site, covers the absorbable gelatin sponge with a small neurosurgical sponge, and applies a slight amount of pressure over the entry site. The EEG technician and epileptologist perform another set of electrocorticography recordings. If the neurosurgeon and the epileptologist determine that no further MSTs are needed, the neurosurgeon closes the wound.

The anesthesia care provider removes the child's ET tube and awakens the child. He or she administers a loading dose of IV phenytoin (ie, 20 mg/kg) before the child leaves the OR. The circulating nurse performs a general assessment of the child's pressure points and skin integrity and applies soft restraints to the child's wrists to prevent the child from removing his or her dressings or tubes. The circulating nurse gives a report to the surgical intensive care unit (SICU) nurses, and surgical team members (ie, surgeons, anesthesia care provider, circulating nurse) transfer the child to the SICU. After the patient is transferred to the SICU, the circulating nurse visits family members in the surgery waiting area to answer questions about the MST procedure and to provide them reassurance.

POSTOPERATIVE PATIENT CARE

The SICU nurses monitor the child's vital signs (ie, blood pressure, pulse, ECG, pulse oximetry, respiratory rate, temperature), initiate seizure precautions, and perform neurological and pain assessments at least every two hours for 12 hours and then every four hours thereafter. The child is attached to a cardiac/apnea monitor for the first 24 hours after surgery to assess him or her for early signs of neurological deterioration. The SICU nurses also assess the child for signs of dehydration by monitoring the child for decreased urine output and changes in laboratory test results (eg, electrolyte imbalances, decreased hematocrit levels) and monitor the child for signs of upper respiratory or urinary tract infections (eg, increases in temperature, white blood cell counts).

Seizure precautions. The epileptologist instructs the SICU nurses to administer a loading dose of phenobarbital (ie, 10 to 20 mg/kg) over a one-hour period. The child is kept on IV phenytoin and phenobarbital until he or she can take oral medications and be switched to preoperative dosage levels of his or her prescribed antiepileptic medication (eg, 600 mg to 800 mg oral carbamazepine per day). The epileptologist prescribes phenytoin to prevent the spread of epileptic discharges and phenobarbital to quiet focal disturbances. The SICU nurses monitor the child's vital signs for cardiac arrhythmias and initiate patient safety measures (eg, padded bed rails, soft restraints) to prevent injury during seizure activity.

Neurological assessment. The SICU nurses perform frequent neurological assessments of the child that include the following: * level of consciousness, * pupillary size and direct light reaction, * eye movement, * motor function, * selected reflexes (eg, corneal, gag) as needed, and * vital signs.(24) Early recognition of subtle changes in any of these factors allows physicians and nurses to take definitive action to reverse possible neurological deterioration in the child before a downhill course of events occurs that does not allow time for intervention.(25)

The SICU nurses perform frequent neurological assessments of the child to detect signs of increased intracranial pressure. Signs of increased intracranial pressure include * decreased level of consciousness; * increased irritability, headaches, and/or pain; * increased blood pressure and/or decreased pulse; * photophobia (ie, shuddering or withdrawal when light is introduced); and * pupillary size changes.

Pain assessment. Pain assessment often is difficult because the child's language deficit does not allow him or her to express discomfort easily. Nurses use a visual pain rating scale (eg, Wong/Baker Pain Rating Scale, Nine Face Scale) that the child can point at to indicate his or her pain level (eg, 1 or A = minimal pain, 10 or I = severe pain). Nurses also assess the child for increased pain or discomfort by looking for signs of * irritability, * crying or grimacing, * fatigue or lack of interest in play, * sensitivity to light, * decreased appetite, or * withdrawn appearance.

Physicians prescribe codeine or other narcotics for management of pain during the initial postoperative period; however, these medications are not continued after the child is transferred from the SICU because of their potential for causing gastrointestinal upset or constipation. The child usually experiences adequate pain relief with nonsteroidal anti-inflammatory medications, and nurses routinely medicate the child every four to six hours for the first 48 to 72 hours after surgery.

The child remains in the SICU for 24 hours after surgery. When the child is able to take oral fluids, the neurologist orders the SICU nurses to remove the child's Foley catheter and discontinue the IV line, and the child is transferred to the neurology monitoring unit. Nurses in the neurology unit continue to assess the child's oral intake and output status, and they ambulate the child as tolerated. The total postoperative hospital stay typically is seven days.

RECENT PATIENT OUTCOMES

A recent clinical study conducted by Frank Morrell, MD, and his colleagues at Rush-Presbyterian-St Luke's Medical Center, Chicago, documented the following patient outcomes for 14 children with LKS who underwent MST procedures. * Before surgery, all 14 children were mute or had only single-word utterances. None of the children was enrolled in a normal classroom and most were in classrooms for developmentally delayed students. * After MST procedures, seven of the 14 children have been able to enroll in normal classes and now are able to engage in normal age-related activities. * Four of the 14 children have made substantial postoperative recoveries but continue to require extensive speech therapy sessions. * Three of the 14 children have had no changes in their preoperative conditions.(26)

CONCLUSION

We believe MST procedures are successful in treating children who have LKS. At our institution, we continue to see many children diagnosed with LKS each year, and we have performed more than 20 MST procedures on these children. Although their recoveries take several months and they must continue to undergo speech therapy for some time after surgery, our observations suggest that their recoveries are related directly to the elimination of abnormal electrical discharges in their cerebral cortexes. The MST procedure appears to be a good therapeutic option for children with LKS.

Janice M. Buelow, RN, MS, CNRN, is a clinical nurse specialist in The Rush Epilepsy Center at Rush-Presbyterian-St Luke's Medical Center, Chicago.

Pat Aydelott, RN, CNOR, is a clinical nurse coordinator for neurosurgery in the OR at Rush-Presbyterian-St Luke's Medical Center, Chicago.

Deborah M. Pierz, RN, BSN, is the acting neurology unit director at Rush-Presbyterian-St Luke's Medical Center, Chicago.

Barbara Heck, RN, BSN, is the clinical nurse coordinator for neurology at Rush-Presbyterian-St Luke's Medical Center, Chicago.

The authors wish to acknowledge Frank Morrell, MD, professor of neurology in the department of neurological sciences at Rush-Presbyterian-St Luke's Medical Center and director of clinical neurophysiology at The Rush Epilepsy Center, Chicago; and Michael C. Smith, MD, associate professor of neurology in the department of neurological sciences at Rush-Presbyterian-St Luke's Medical Center and associate director for clinical programs at The Rush Epilepsy Center, Chicago, for their guidance with this article; and Amy E. Hall, CPA, section secretary at The Rush Epilepsy Center, Chicago, for her editorial assistance with this article.

NOTES

(1.) W Landau, F Kleffner, "Syndrome of acquired aphasia with convulsive disorder in children," Neurology 7 (August 1957) 523-530.

(2.) J F Montovani, W Landau, "Acquired aphasia with convulsive disorder: Course and prognosis," Neurology 30 (May 1980) 524-529.

(3.) D V M Bishop, "Age of onset and outcome in acquired aphasia with convulsive disorder (Landau-Kleffner syndrome)," Developmental Medicine and Child Neurology 27 (January 1985) 705-712.

(4.) C Marescaux et al, "Landau-Kleffner syndrome: A pharmacologic study of five cases," Epilepsia 31 (November/December 1990) 768-777.

(5.) F Morrell, W W Whisler, T P Bleck, "Multiple subpial transection: A new approach to die surgical treatment of focal epilepsy," Journal of Neurosurgery 70 (February 1989) 231-239.

(6.) Bishop, "Age of onset and outcome in acquired aphasia with convulsive disorder (Landau-Kleffner syndrome)," 708; Montovani, Landau, "Acquired aphasia with convulsive disorder: Course and prognosis," 525; F Morrell et al, "Landau-Kleffner syndrome: Treatment with subpial intracortical transection," Brain 118 (December 1995) 1529-1546.

(7.) A J Cole et al, "The Landau-Kleffner syndrome of acquired epileptic aphasia: Unusual clinical outcome, surgical experience, and absence of encephalitis," Neurology 38 (January 1988) 31-38; E Hirsch et al, "Landau-Kleffner syndrome: A clinical and EEG study of five cases," Epilepsia 31 (November/ December 1990) 756-767.

(8.) Morrell et al, "Landau-Kleffner syndrome: Treatment with subpial intracortical transection," 1529-1546.

[9.] Ibid.

[10.] Bishop, "Age of onset and outcome in acquired aphasia with convulsive disorder (Landau-Kleffner syndrome)," 708; Cole et al, "The Landau-Kleffner syndrome of acquired epileptic aphasia: Unusual clinical outcome, surgical experience and absence of encephalitis," 31-38; Hirsch et al, "Landau-Kleffner syndrome: A clinical and EEG study of five cases," 756-767; Morrell et al, "Landau-Kleffner syndrome: Treatment with subpial intracortical transection," 1529-1546; Landau, Kleffner," syndrome of acquired aphasia with convulsive disorder in children," 523-530.

[11.] Morrell et al, "Landau-Kleffner syndrome: Treatment with subpial intracortical transection," 1529-1546.

[12.] Ibid.

[13.] Ibid.

[14.] T Deonna, "Acquired epileptiform aphasia in children (Landau-Kleffner syndrome)," Journal of Clinical Neurophysiology 8 (July 1991) 3-4; Hirsch et al, "Landau-Kleffner syndrome: A clinical and EEG study of five cases," 757-761.

[15.] Hirsch et al, "Landau-Kleffner syndrome: A clinical and EEG study of five cases," 773; Montovani, Landau, "Acquired aphasia with convulsive disorder: Course and prognosis," 528; Morrell et al, "Landau-Kleffner syndrome: Treatment with subpial intracortical transection," 1529-1546; G Patry, S Lyagoubi, C A Tassinari, "Subclinical `electrical status epilepticus' induced by sleep in children," Archives of Neurology 24 (March 1971) 250-251; I M S Sawhney et al "Acquired aphasia with epilepsy -- Landau-Kleffner syndrome," Epilepsia 29 (May/June 1988) 283-287.

[16.] Hirsch et al, "Landau-Kleffner syndrome: A clinical and EEG study of five cases," 759-766; Marescaux et al, "Landau-Kleffner syndrome: A pharmacologic study of five cases," 768-777; Morrell et al, "Landau-Kleffner syndrome: Treatment with subpial intracortical transection," 1529-1546.

[17.] Morrell et al, "Landau-Kleffner syndrome: Treatment with subpial intracortical transection, 1529-1546.

[18.] Morrell, Whisler, Bleck, "Multiple subpial transection: A new approach to the surgical treatment of focal epilepsy," 237.

[19.] M Smith, W Whister, F Morrell, "Neurosurgery of epilepsy," Seminars in Neurology 9 (September 1989) 233-235.

[20.] Morrell, Whisler, Bleck, "Multiple subpial transection: A new approach to the surgical treatment of focal epilepsy," 233.

[21.] Ibid.

[22.] Ibid, 233.

[23.] Ibid.

[24.] J V Hickey, The Clinical Practice of Neurological and Neurosurgical Nursing, third ed (Philadelphia: J B Lippincott, Co, 1992) 276-278.

[25.] Ibid, 278.

[26.] Morrell et al, "Landau-Kleffner syndrome: Treatment with subpial intracortical transection,: 1529-1546.

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