eMedicine Specialties > Neurology > Pediatric Neurology
Sturge-Weber Syndrome: Treatment & Medication
Updated: Dec 8, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
This includes anticonvulsants for seizure control, symptomatic and prophylactic therapy for headache, glaucoma treatment to reduce the IOP, and laser therapy for PWS.
- Seizures: Since the seizures are typically focal, an anticonvulsant with efficacy in focal seizures is preferable (see anticonvulsant medications).
- Glaucoma: The goal of treatment is control of IOP to prevent optic nerve injury (please see the articles on glaucoma in eMedicine Ophthalmology journal). Medications either decrease the production of aqueous fluid or promote the outflow of aqueous fluid. Beta-antagonist eye drops reduce the production of aqueous fluid, adrenergic eye drops and miotic eye drops reduce IOP by promoting drainage, and carbonic anhydrase inhibitors decrease IOP by decreasing production of aqueous fluid.
- Headaches: Recurrent headaches can be treated with symptomatic and prophylactic medications (see Migraine Headache).
- Kossoff et al evaluated 68 patients with SWS regarding headaches, identified through the Sturge-Weber Foundation.74 Mean onset of the headaches was 8 years. Fifty-five of the 68 patients had epilepsy as well. Twenty-two of these patients perceived that the headaches were a more significant problem compared to their epilepsy. A positive family history of headaches was seen in 37 of these patients.
- Most of the patients were using only abortive treatment, mainly acetaminophen and ibuprofen, while only 15 were tried on preventative agents, including gabapentin, valproate, and amitriptyline (none were on beta-adrenergic blockers). The authors suggested that the headaches may be undertreated.
- Kossoff et al also reported on 104 patients with SWS and migraine headaches, regarding self-reported treatment patterns through a questionnaire. In SWS, triptans and preventative agents appear to be effective for the headaches.75
- Stroke-like events: Aspirin has been used for headaches and to prevent vascular disease, although it typically is used in patients who have had neurologic progression or recurrent vascular events.76 Aspirin needs to be used with extreme caution in children because of the concern with Reye syndrome, and the risks and benefits need to be carefully weighed. Thomas-Sohl, Vaslow, and Maria have recommended 3-5 mg/kg/d of aspirin for stroke-like events, and they also recommended varicella and yearly influenza immunizations because of association of varicella and influenza infections in Reye syndrome.22 Maria et al reported a decreased incidence of strokelike events in 20 patients who received aspirin14 ; of 119 strokelike events, 31 occurred in patients treated with aspirin, whereas 88 of these events occurred in those not treated with aspirin. The authors suggested further investigation of aspirin treatment in SWS.
- PWS: These need to be evaluated within the first week of life and differentiated from hemangioma.
- PWS are treated with laser therapy, which should start as soon as possible, since multiple treatments are needed and earlier treatment may reduce the number needed. Also, the smaller the lesion initially, the fewer the laser flashes needed to remove the lesion.77,78
- Troilius et al reported on the potential psychological benefits from early treatment of PWS.79 In a survey of patients with PWS, 75% reported that the PWS had affected their lives negatively, 62% were convinced that their lives would improve if the PWS were removed, 47% suffered low self-esteem, and 28% said that the PWS made their school life and education more difficult. No persistent pigmentation changes or posttreatment scarring were reported after laser therapy.
Surgical Care
Surgery is desirable for refractory seizures, glaucoma, and specific problems related to various associated disorders, such as scoliosis.80
- Seizures, refractory seizures
- Surgical options are available for seizures refractory to medical treatment, especially for focal seizures81 . Surgical procedures include focal cortical resection, hemispherectomy, corpus callosotomy, and recently, vagal nerve stimulation (VNS).82 SWS is considered one of the catastrophic epilepsies which, according to Holmes, result in poor seizure control and developmental outcome if not controlled early83 ; however, criteria for medical intractability should be fulfilled before considering surgery.
- Early surgery has been advocated specifically in SWS to improve outcome and prevent refractory seizures, developmental delay, and hemiparesis. In the era prior to modern neuroimaging, Alexander and Norman and, later, Alexander suggested exploratory craniotomy and lobectomy if the diagnosis was confirmed, even before seizures started, because they found that early onset seizures were associated with mental retardation.84
- Hoffman et al and then Ogunmegan et al later advocated early hemispherectomy for seizures.85,86 Therefore, the need for surgery, its timing, and the appropriate surgical procedure are important considerations. Erba and Cavazzuti estimated that 40% of patients with SWS could become epilepsy surgery candidates, excluding those with either good seizure control or bilateral disease.23
- The chance of achieving seizure control with medical therapy in SWS varies. Depending on the series, complete seizure control has been achieved in 10-50% of patients, and refractory seizures occur in 11-83% (Table 4). Results vary by the patient population seen at different centers, with a higher incidence of medical failures reported by surgical centers. However, according to Arizmanoglou, even data from the surgical centers indicate that good seizure control is achieved in one third to one half of the patients seen at these centers.87 Table 4. Seizure Control in Sturge-Weber Syndrome
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Study Complete Partial Refractory/No Control Gilly et al 88 NA* NA 37% Sujanski and Conradi 35
(adults)27% 49% 24% Sujanski and Conradi 35, 16 (all ages) 50% 39% 11% Pascual-Castroviejo et al 33 47% 12% 28% Oakes 25 10% NA 83% Sassower et al 69 NA NA 43% Arzimanoglou and Aicardi 87 NA NA 39% Erba and Cavazzuti 23 50% NA NA Toronto 72, 85 NA NA 32% *NA = not availableStudy Complete Partial Refractory/No Control Gilly et al 88 NA* NA 37% Sujanski and Conradi 35
(adults)27% 49% 24% Sujanski and Conradi 35, 16 (all ages) 50% 39% 11% Pascual-Castroviejo et al 33 47% 12% 28% Oakes 25 10% NA 83% Sassower et al 69 NA NA 43% Arzimanoglou and Aicardi 87 NA NA 39% Erba and Cavazzuti 23 50% NA NA Toronto 72, 85 NA NA 32% - The age of seizure onset may be a prognostic sign for ultimate seizure control.
- Roach believes that seizure onset in patients younger than 2 years is more likely to be associated with refractory seizures and developmental problems.18 The data from Bebin and Gomez, Oakes, Pascual-Castroviejo et al, and Sujansky and Conradi support this.32,25,33,16 However, Maria et al divided their patients into 2 groups by age for a longitudinal study—those aged 1-3 years versus those aged 10-22 years—and found no difference in clinical outcomes with early onset seizures.14
- Even with seizure onset within the first year, Erba and Cavazzuti reported satisfactory control in 50%, with 30% seizure free for at least 2 years, and in the others, 17% had an average of 1 seizure per month, and 33% were considered to have poorly controlled seizures, defined as greater than 1 seizure per week.23 Therefore, early seizures may not predict either the severity of subsequent epilepsy or severe mental retardation.
- Predictors of poor outcome include the extent of the LA, a refractory seizure disorder, and relapsing or permanent motor deficits. Factors predicting a poor outcome (or indicating surgery) include the following:
- Early seizure onset
- Extensive LA
- Medically refractive seizures
- Relapsing or permanent motor deficits
- Headaches or mild trauma associated with transient motor deficits
- Evidence of progressive neurologic damage
- Focal seizures with subsequent generalization
- Increasing seizure frequency and duration
- Increasing duration of postictal deficits
- Increasing focal or diffuse atrophy
- Progressive atrophy or calcifications
- Development of hemiparesis
- Deterioration in cognitive functioning (loss of intellectual abilities)
- Erba and Cavazzuti recommended surgery when seizures, as well as other neurologic events, such as headaches or mild head trauma, are associated with functional neurologic deficits, the presence of which indicates an impairment in cortical perfusion.23
- Arzimanoglou and Aicardi treat seizures initially with anti-epileptic drugs (AEDs), no matter what the age of onset, and recommend surgery when seizures are intractable or when evidence of progressive cortical damage is noted. The appropriate surgical procedure is determined individually by clinical course, EEG, and neuroimaging.87,89
- Factors suggesting a progressive course include (1) initial focal seizures progressing to frequent secondarily generalized seizures, (2) increasing seizure frequency and duration despite AEDs, (3) increasing duration of a transient postictal deficit, (4) increase in focal or diffuse atrophy determined by serial neuroimaging, (5) progressive increase in calcifications, (6) development of hemiparesis, and (7) deterioration in cognitive functioning.
- Outcome of epilepsy surgery in SWS: Three centers have reported on groups of more than 10 patients—Hoffman et al from Toronto, Arzimanoglou and Aicardi from Paris, and the author's series from Children's Hospital, Boston (Table 5). Of the 32 patients from these groups who have had limited resection, 18 are seizure free, 10 have had an improvement, and 4 have had no improvement. Of 26 treated with hemispherectomy, 24 have been seizure free.
- The group from Toronto has evaluated the relationship between seizure control and developmental outcome in 74 patients72 . Of these, 53 patients had seizures, which were refractory in 17 (32%) patients. The authors compared the ultimate developmental outcome (determined by intelligence quotient [IQ] score) of medical and surgical therapies in 50 patients, 17 patients who underwent surgery and 33 who were given medical therapy. Normal or borderline functioning was more common after surgical treatment (10 of 17 [58.8%] patients) than in medical treatment (11 of 33 [33.3%] patients, P <0.05).
- When surgery is considered, choice of appropriate procedure must be the main consideration. The epileptogenic region is located in cortex adjacent to the angioma, and electrocorticography (ECOG) may be needed. However, the LA usually covers the entire hemisphere, and even areas without angioma may be epileptogenic and therefore need resection to achieve seizure control. A focal cortical resection (a more limited resection) is done when the LA and, therefore, the epileptogenic region is smaller and more localized. This can be demonstrated preoperatively by localizing the area of seizure onset, either with surface EEG or ECOG (if invasive monitoring has been done), with a combination of both structural and functional neuroimaging, and with intraoperative ECOG.
- Hemispherectomy is done when an extensive, unilateral epileptogenic region exists. When the epileptogenic region is smaller, a focal cortical resection (ie, a more limited resection) is preferable, since it is less likely to cause a neurologic deficit. Hoffman reports that focal disease responds well to resection, ECOG identifies adjacent epileptogenic cortex, and hemispherectomy produces a significant improvement in outcome, leading to normal intelligence and a chance of becoming seizure free greater than 90%.
- Residual seizures, however, are more likely with a more limited resection than with hemispherectomy. Gilly et al reported a 30% failure rate after limited resection88 , and in the combined data from 3 surgical centers, 12.5% (Table 5) of those patients who underwent a limited resection had no improvement. Table 5. Surgical Results of Hemispherectomy and Limited Resection from 3 Centers
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Center Hemispherectomy Seizure Free Limited resection Seizure Free Improved Toronto 12 11 11 8 2 Paris 5 5 15 7 8 Boston 9 8 6 3 0 Total 26 24 32 18 10 24 of 26 patients with hemispherectomy - Seizure free 28 of 32 patients with limited resection - Seizure free or improved Center Hemispherectomy Seizure Free Limited resection Seizure Free Improved Toronto 12 11 11 8 2 Paris 5 5 15 7 8 Boston 9 8 6 3 0 Total 26 24 32 18 10 24 of 26 patients with hemispherectomy - Seizure free 28 of 32 patients with limited resection - Seizure free or improved
- Kossoff et al evaluated the outcome of hemispherectomy in 32 patients with SWS, using a questionnaire; these patients were identified through the Sturge-Weber Foundation90
- Although this study was limited because of the volunteer basis of the returned questionnaires, still a larger number of patients were included to the previous studies. Patients had hemispherectomy between 1979 and 2001, and mean age of seizure onset was 4 months, median age of surgery was 1.2 years. Sixteen had anatomical hemispherectomy, 14 had functional hemispherectomy, and 2 had hemidecortications performed in 18 different centers throughout the world. Fifteen had complications in the immediate postoperative period, including hemorrhage, infection, and severe headaches, and they underwent reoperation due to persistent seizures, shunting, or hypertension. No deaths occurred.
- In this study, 81% became seizure free, with 53% off antiepileptic drugs. The type of surgery (anatomical hemispherectomy vs functional hemispherectomy vs hemidecortication) did not influence outcome. Age of seizure onset did not predict seizure freedom, while older age of surgery had a positive correlation. Postoperative hemiparesis was not worse compared with before the surgery. Cognitive outcome was not related to age at surgery, side of surgery, or seizure freedom.
- The Toronto group suggested that hemispherectomy is more successful if done during infancy, since earlier seizure control helps to preserve the function of the normal hemisphere.72,85 They now perform a hemispherectomy, resulting in better neurologic recovery, even with some residual finger movement. Alternatively, if the patient is not a candidate for a limited resection or hemispherectomy, such as when disease is bilateral, corpus callosotomy can be done or VNS can be administered. VNS has been shown to be effective for focal seizures; its mechanism of action is a putative increase in CNS inhibitory activity.
- In order to address these issues, the Sturge-Weber Foundation recruited a task force to evaluate epilepsy surgery in SWS. The following is a summary of recommendations for surgery in SWS, modified to include VNS:
- Hemispherectomy should not be done in every patient with SWS solely because of the emphasis on increasingly early surgery. Surgery is appropriate only for medically refractory seizures.
- Patients with intractable seizures and very localized lesions should have a limited resection that preserves as much normal tissue as possible.
- Video EEG and both structural and functional neuroimaging should be used to define the extent of the lesion and the site of seizure origin.
- Corpus callosotomy is reserved for patients with intractable atonic or tonic seizures leading to secondary injury who are not candidates for more definitive surgery.
- Surgery should be done only in a center with an ongoing pediatric epilepsy surgery program.
- Although the benefit of surgery for refractory seizures is accepted generally, additional work is needed to evaluate the natural history of the syndrome and the potential benefits and risks of surgery.
- VNS can be done in those who are not candidates for other surgical procedures.
- Summary: Data on the natural history of the disease are not yet sufficient to advocate hemispherectomy unless refractory seizures occur.
- Glaucoma surgery39 : If medications are unable to lower IOP, surgery may be beneficial. Trabeculectomy increases the release of aqueous fluid from the anterior chamber and opens the outflow pathway. Goniotomy is similar but is done through the eye. A Molteno valve can be placed (similar to a shunt), and cyclodestructive procedures with either freezing or laser decrease the production of aqueous fluid.
Consultations
Primary-care providers should be educated about SWS. Consultations are needed from a neurologist, an epileptologist (especially if seizures are intractable), a dermatologist, a plastic surgeon, a psychologist, a psychiatrist, a neuropsychologist, and a neuroendocrinologist.
Diet
No special diet is needed.
Activity
No restrictions are needed except as mandated by associated conditions.
Medication
Please refer to the various articles that describe anticonvulsant treatment of partial seizures.
Anticonvulsants
These agents are used to terminate clinical and electrical seizure activity as rapidly as possible and prevent seizure recurrence.
Carbamazepine (Tegretol)
Anticonvulsant effective for treatment of complex partial seizures. Appears to act by reducing polysynaptic responses and blocking posttetanic potentiation. Major mechanism of action is reduction of sustained high-frequency repetitive neural firing.
Adult
200 mg PO bid (100 mg qid of suspension); increase every wk by <200 mg/d PO tid/qid (bid with extended release) until best response obtained; not to exceed 1600 mg/d
Pediatric
<6 years: 10-20 mg/kg/d PO bid/tid (qid with suspension); increase every wk to achieve optimal clinical response administered tid/qid
6-12 years: 100 mg PO bid (50 mg qid of suspension); increase every wk by adding 100 mg/d PO tid/qid (bid with extended release) until response obtained; not to exceed 1000 mg/d
>12 years: Administer as in adults; not to exceed 1000 mg/d in children aged 12-15 years or 1200 mg/d if >15 years
Do not administer with MAOIs; danazol within last 30 d may increase serum levels significantly (avoid whenever possible); cimetidine may increase toxicity, especially if taken in first 4 wk of therapy; may decrease primidone and phenobarbital levels (their coadministration may increase carbamazepine levels)
Documented hypersensitivity; history of bone marrow depression; MAOIs within last 14 d
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Do not use to relieve minor aches or pains; caution with increased IOP; obtain CBCs and serum iron baseline level prior to treatment, during first 2 mo, and yearly or every other year thereafter; can cause drowsiness, dizziness, and blurred vision; caution while driving or performing other tasks requiring alertness
Phenytoin (Dilantin)
Primary site of action of hydantoins, such as phenytoin, appears to be motor cortex, where may inhibit spread of seizure activity. May reduce maximal activity of brainstem centers responsible for tonic phase of grand mal seizures. Dosing should be individualized. If daily dosing cannot be divided equally, larger dose should be given before retiring. Phosphorylated formulation, fosphenytoin, available for parenteral use and may be given IM or IV.
Adult
100 mg (125 mg suspension) PO/IV tid initially; maintenance dose 300-400 mg/d PO/IV divided tid, or qd/bid if using extended release; increase to 600 mg/d (625 mg/d suspension) may be necessary; not to exceed 1500 mg/24h
Pediatric
<6 years: 5 mg/kg/d PO/IV divided bid/tid initially; maintenance dose 4-8 mg/kg PO/IV divided bid/tid
>6 years may require minimum adult dose (300 mg/d); not to exceed 300 mg/d
Amiodarone, benzodiazepines, chloramphenicol, cimetidine, fluconazole, isoniazid, metronidazole, miconazole, phenylbutazone, succinimides, sulfonamides, omeprazole, phenacemide, disulfiram, ethanol (acute ingestion), trimethoprim, and valproic acid may increase toxicity
Barbiturates, diazoxide, ethanol (chronic ingestion), rifampin, antacids, charcoal, carbamazepine, theophylline, and sucralfate may decrease effects
May decrease effects of acetaminophen, corticosteroids, dicumarol, disopyramide, doxycycline, estrogens, haloperidol, amiodarone, carbamazepine, cardiac glycosides, quinidine, theophylline, methadone, metyrapone, mexiletine, oral contraceptives, valproic acid
Documented hypersensitivity; sino-atrial block; sinus bradycardia; second- and third-degree AV block; Adams-Stokes syndrome
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Perform blood counts and urinalyses when therapy is begun and at monthly intervals for several mo thereafter to monitor for blood dyscrasias; discontinue use if skin rash appears, and do not resume use if rash is exfoliative, bullous or purpuric; rapid IV infusion may result in death from cardiac arrest, marked by QRS widening; caution in acute intermittent porphyria and diabetes (may elevate blood sugars; discontinue use if hepatic dysfunction occurs
Valproic acid (Depakote, Depakene, Depacon)
Chemically unrelated to other drugs used to treat seizure disorders. Although mechanism of action unknown, activity may be related to increased brain levels of GABA or enhanced GABA action. Also may potentiate postsynaptic GABA responses, affect potassium channels, or have direct membrane-stabilizing effect. For conversion to monotherapy, concomitant AED dosage ordinarily can be reduced by approximately 25% every 2 wk. This reduction may be started at initiation of therapy or delayed by 1-2 wk if concern that seizures are likely to occur with reduction. Monitor patients closely during this period for increased seizure frequency. As adjunctive therapy, divalproex sodium may be added to patient's regimen at dosage of 10-15 mg/kg/d. Dosage may be increased by 5-10 mg/kg/wk to achieve optimal clinical response. Ordinarily, optimal clinical response achieved at daily doses <60 mg/kg/d.
Adult
Monotherapy: 10-15 mg/kg/d PO qd or divided tid; increase by 5-10 mg/kg/wk until seizures controlled or adverse effects prevent further increases; if total daily dose >250 mg, give in divided doses; not to exceed 60 mg/kg/d
Pediatric
Administer as in adults
Cimetidine, salicylates, felbamate, and erythromycin may increase toxicity; rifampin may reduce levels significantly; in pediatric patients, salicylates decrease protein binding and metabolism; may result in variable changes of carbamazepine concentrations with possible loss of seizure control; may increase diazepam and ethosuximide toxicity (monitor closely); may increase phenobarbital and phenytoin levels, while either may decrease valproate levels; may displace warfarin from protein-binding sites (monitor coagulation tests); may increase zidovudine levels in HIV-seropositive patients
Documented hypersensitivity; hepatic disease/dysfunction
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Thrombocytopenia and abnormal coagulation parameters have occurred; risk of thrombocytopenia increases significantly at total trough plasma concentrations >110 mcg/mL in females and 135 mcg/mL in males; at periodic intervals and prior to surgery, determine platelet counts and bleeding time before initiating therapy; reduce dose or discontinue therapy if hemorrhage, bruising, or hemostasis/coagulation disorder occurs; hyperammonemia may occur, resulting in hepatotoxicity; monitor patients closely for appearance of malaise, weakness, facial edema, anorexia, jaundice, and vomiting; may cause drowsiness
Gabapentin (Neurontin)
Has properties in common with other anticonvulsants. However, exact mechanism of action unknown. Structurally related to GABA but does not interact with GABA receptors. Increases in daily dose are best tolerated when done slowly.
Adult
100 mg PO tid or 300 mg PO hs on day 1; on day 2 increase dose to 400 mg PO tid; after 3 d at this dose, titrate prn; not to exceed 1200 mg
Pediatric
<12 years: Not established
>12 years: Administer as in adults
Antacids may significantly reduce bioavailability (administer > 2 h following antacids); may increase norethindrone levels significantly
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in severe renal disease
Lamotrigine (Lamictal)
Triazine derivative useful in treatment of both seizures and neuralgic pain. Inhibits release of glutamate and inhibits voltage-sensitive sodium channels, which stabilizes neuronal membrane. Follow manufacturer's recommendation for dose adjustments.
Adult
Adjunctive therapy with enzyme-inducing anticonvulsant: 50 mg/d PO first 2 wk, followed by 100 mg/d divided bid for 2 additional wk; for maintenance, may increase by 100 mg/d q1-2wk to 300-500 mg/d divided bid
Adjunctive therapy with an anticonvulsant regimen containing valproate: 25 mg PO qod for first 2 wk, followed by 25 mg/d for 2 additional wk; for maintenance, may increase doses by 25-50 mg/d q1-2wk to 100-200 mg/d qd or divided bid
Conversion from single enzyme-inducing anticonvulsant to lamotrigine monotherapy: 50 mg/d PO for first 2 wk, followed by 100 mg/d PO divided bid for 2 additional wk; for maintenance, may increase by 100 mg/d q1-2wk to 300-500 mg/d divided bid; enzyme-inducing anticonvulsant gradually withdrawn over 4-wk interval in 20% decrements per wk
Pediatric
Adjunctive therapy with an enzyme-inducing anticonvulsant
2-12 years: 0.6 mg/kg/d PO divided bid, rounded down to nearest 5 mg for first 2 wk; followed by 1.2 mg/kg/d divided bid, rounded down to nearest 5 mg for 2 additional wk; for maintenance, increase by 1.2 mg/kg/d (round down to nearest 5 mg) q1-2wk and add this amount to previously administered daily dose; average maintenance 5-15 mg/kg/d; not to exceed 400 mg/d divided bid
>12 years: 50 mg/d PO for first 2 wk, followed by 100 mg/d divided bid for 2 additional wk; for maintenance, increase dose by 100 mg/d q1-2wk; average maintenance dose 300-500 mg/d divided bid
Concomitant therapy with valproic acid
2-12 years: 0.15 mg/kg/d PO qd or divided bid, rounded down to nearest 5 mg for first 2 wk; if initial calculated daily dose 2.5-5 mg, take 5 mg on alternate days for first 2 wk, followed by 0.3 mg/kg/d qd or divided bid, rounded down to nearest 5 mg for additional 2 wk; for maintenance, increase subsequent doses by 0.3 mg/kg/d q1-2wk, round down to nearest 5 mg, and add this amount to previously administered qd dose; average maintenance dose 1-5 mg/kg/d; not to exceed 200 mg/d qd or divided bid
>12 years: 25 mg PO qod for first 2 wk, followed by 25 mg qd for 2 additional wk; for maintenance, increase by 25-50 mg/d q1-2wk; average maintenance dose 100-400 mg/d qd or divided bid
Acetaminophen increases renal clearance, decreasing effects; similarly, phenobarbital and phenytoin increase metabolism, causing decrease in levels; valproic acid increases half-life
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in impaired renal or hepatic function; associated with rash in 5% of patients; children who take lamotrigine with valproate have significantly increased risk of severe allergic drug reactions
Topiramate (Topamax)
Sulfamate-substituted monosaccharide with broad spectrum of antiepileptic activity that may have state-dependent sodium channel– blocking action. Potentiates inhibitory activity of neurotransmitter GABA. May block glutamate activity. Not necessary to monitor plasma concentrations to optimize therapy. On occasion, addition to phenytoin may require adjustment of phenytoin dose to achieve optimal clinical outcome.
Adult
50 mg/d PO; titrate by 50 mg/d at 1-wk intervals to target dose of 200 mg bid; not to exceed 1600 mg/d
Pediatric
Not established
Phenytoin, carbamazepine, and valproic acid can decrease levels significantly; reduces digoxin and norethindrone levels; carbonic anhydrase inhibitors may increase risk of renal stone formation and should be avoided; may have additive effects with CNS depressants in CNS depression, as well as other cognitive or neuropsychiatric adverse events
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Risk of developing kidney stone increased 2-4 times that of untreated population; risk may be reduced by increasing fluid intake; caution in renal or hepatic impairment
Tiagabine (Gabitril)
Mechanism of action in antiseizure effect unknown. However, believed to be related to its ability to enhance activity of GABA, major inhibitory neurotransmitter in CNS. May block GABA uptake into presynaptic neurons, permitting more GABA to be available for receptor binding on surfaces of postsynaptic cells and possibly prevents propagation of neural impulses that contribute to seizures by GABA-ergic action. Dosing modification of concomitant AEDs not necessary unless clinically indicated.
Adult
4 mg PO qd in 2-4 divided doses; increase by 4-8 mg/wk until clinical response achieved or until total daily dose of 56 mg/d administered; effects of doses >56 mg/d have not been evaluated systematically in adequate well-controlled trials
Pediatric
<12 years: Not established
12-18 years: 4 mg PO qd and increase by 4 mg after 2 wk; total daily dose may be increased by 4-8 mg/wk thereafter until clinical response achieved or 32 mg/d administered; >32 mg/d tolerated in small number of adolescent patients for relatively short duration
Cleared more rapidly in patients treated with carbamazepine, phenytoin, primidone, or phenobarbital than in patients who have not received these drugs
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Patients receiving valproate monotherapy may require lower doses or slower dose titration of tiagabine for clinical response; moderately severe to incapacitating generalized weakness has been reported following administration of tiagabine in as many as 1% of patients with epilepsy; weakness may resolve after reduction in dose or discontinuation of tiagabine; should be withdrawn slowly to reduce potential for increased seizure frequency
Felbamate (Felbatol)
Oral antiepileptic agent with weak inhibitory effects on GABA-receptor binding and benzodiazepine-receptor binding. Has little activity at MK-801 receptor-binding site of NMDA receptor-ionophore complex. However, is antagonist at strychnine-insensitive glycine-recognition site of NMDA receptor-ionophore complex. Not indicated as first-line antiepileptic treatment. Recommended for use only in patients whose epilepsy is so severe that benefits outweigh risks of aplastic anemia or liver failure. Most adverse effects during adjunctive therapy resolve as dosage of concomitant AEDs decreased.
Adult
Monotherapy: 1200 mg/d PO divided tid/qid initially; titrate to 2400 mg/d with 600 mg increments q2wk and to 3600 mg/d if clinically indicated
Conversion to monotherapy: 1200 mg/d divided PO tid/qid initially; reduce dosage of concomitant AEDs by one third at initiation of felbamate therapy; after first wk, increase dosage to 2400 mg/d while reducing dosage of other AEDs by additional one third of their original dosage; following wk 2, increase felbamate dosage up to 3600 mg/d and continue to reduce dosage of other AEDs prn
Adjunctive therapy: 1200 mg/d PO; after first wk reduce dose of concomitant AEDs by one third; following first wk, administer 2400 mg/d and reduce original AED dose by another third; 3600 mg/d after third wk and reduce other AEDs as clinically indicated
Pediatric
Monotherapy
<14 years: Not established
>14 years: Administer as in adults
Adjunctive therapy
2-14 years: 15 mg/kg/d PO divided tid/qid; reduce other AEDs by 20%; titrate felbamate dose with 15 mg/kg/d increments qwk to 45 mg/kg/d
>14 years: Administer as in adults
May increase steady-state phenytoin levels (40% dose-reduction of phenytoin may be necessary in some patients); phenytoin may double clearance, resulting in more than 45% decrease in steady-state levels; may increase phenobarbital plasma concentrations; phenobarbital may reduce plasma levels; may decrease steady-state carbamazepine levels and increase steady-state carbamazepine metabolite levels; may increase steady-state valproic acid levels
Documented hypersensitivity; blood dyscrasia; hepatic dysfunction
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Associated with marked increase in incidence of aplastic anemia (monitor CBC periodically); marked increase in fatal hepatic failure—perform liver function testing (ALT, AST, bilirubin) before felbamate therapy and at 1- to 2-wk intervals during therapy; discontinue immediately if liver abnormalities detected during treatment
Phenobarbital (Luminal, Barbita)
Exhibits anticonvulsant activity in anesthetic doses and can be administered orally. If IM route chosen, inject into large muscle such as gluteus maximus, vastus lateralis, or other areas where little risk of encountering nerve trunk or major artery. Injection into or near peripheral nerves may result in permanent neurological deficit. Restrict IV use to conditions in which other routes are not feasible, either because patient unconscious, as in cerebral hemorrhage, eclampsia, or status epilepticus, or because prompt action imperative.
Adult
60-100 mg/d PO; alternatively, 200-320 mg IV/IM q6h prn
Pediatric
3-6 mg/kg/d PO; alternatively, 4-6 mg/kg/d IV/IM for 7-10 d to blood level of 10-15 mcg/mL, maximum dose of 10-15 mg/kg/d
May decrease effects of chloramphenicol, digitoxin, corticosteroids, carbamazepine, theophylline, verapamil, metronidazole, and anticoagulants (patients stabilized on anticoagulants may require dosage adjustments if added to or withdrawn from their regimen); alcohol may produce additive CNS effects and death; chloramphenicol, valproic acid, and MAOIs may increase toxicity; rifampin may decrease effects; induction of microsomal enzymes may result in decreased effects of oral contraceptives in women (must use additional contraceptive methods to prevent unwanted pregnancy; menstrual irregularities may occur)
Documented hypersensitivity; severe respiratory disease; marked impairment of liver function; nephritis
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
In prolonged therapy, evaluate hematopoietic, renal, hepatic, and other organ systems; caution in fever, hyperthyroidism, diabetes mellitus, and severe anemia since adverse reactions can occur; caution in myasthenia gravis and myxedema
Oxcarbazepine (Trileptal)
Pharmacological activity primarily by 10-monohydroxy metabolite. Studies indicate that this drug may block voltage-sensitive sodium channels, inhibit repetitive neuronal firing, and impair synaptic impulse propagation. This drug's anticonvulsant effect may occur by affecting potassium conductance and high-voltage activated calcium channels. Drug pharmacokinetics are similar in older children (>8 y) and adults. Young children ( <8 y) have 30-40% increased clearance compared with older children and adults. Children <2 years have not been studied in controlled clinical trials.
Adult
Monotherapy: 600 mg/d divided PO bid initially; increase dose by 300 mg/d q3d to 1200 mg/d; monitor patients for anticonvulsant adverse effects
Conversion to monotherapy: 600 mg/d PO divided bid initially; gradually reduce dose of concomitant AEDs in about 3-6 wk and gradually increase oxcarbazepine dose in 2-4 wk; may increase oxcarbazepine dose as needed by maximum increment of 600 mg/d at weekly intervals; monitor patients closely during this transition phase for anticonvulsant adverse effects
Adjunctive therapy: 600 mg/d PO divided bid initially; may increase by maximum of 600 mg/d at weekly intervals; recommended daily dose 1200 mg/d; monitor patients for anticonvulsant adverse effects
Pediatric
Adjunctive therapy (age 4-16 years): 8-10 mg/kg/d PO divided bid, not to exceed 600 mg/d; gradually increase to target dose over 2 wk; target dose based on body weight as follows:
20-29 kg: 900 mg/d PO
29.1-39 kg: 1200 mg/d PO
>39 kg: 1800 mg/d PO
May decrease levels of dihydropyridine calcium antagonists and oral contraceptives; can reduce serum concentrations of carbamazepine, phenobarbital, phenytoin, and valproic acid; when given in doses >1200 mg/d may increase phenytoin and phenobarbital serum concentrations significantly; can reduce serum concentrations of oral contraceptives and make oral contraceptives ineffective; can increase clearance of felodipine
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Can cause cognitive adverse effects (eg, psychomotor slowing, impaired concentration, impaired speech, impaired language); decrease initiation dose by 50% with renal impairment (CrCl <30 mL/min) and increase dose more slowly; oxcarbazepine can cause hyponatremia (sodium <125 mmol/L); among persons with hypersensitivity to carbamazepine, 25-30% will have hypersensitivity to oxcarbazepine; rapid withdrawal of oxcarbazepine can cause exacerbation of seizures; observe for side effects and monitor plasma levels of concomitant anticonvulsants during dose titration
Zonisamide (Zonegran)
Indicated for adjunct treatment of partial seizures with or without secondary generalization. Evidence that is effective in myoclonic and other generalized seizure types as well.
Adult
100-600 mg/d PO effective dose
100 mg/d PO for 2 wk initial dose; increase 100 mg q2wk; >400 mg/d not shown to be of benefit
Pediatric
Not established
May increase serum carbamazepine levels; carbamazepine may increase zonisamide concentrations; phenobarbital may decrease zonisamide levels
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May cause drowsiness, weight loss, ataxia, nausea, and slowing of mental activity; pediatric patients have an increased risk for oligohidrosis and hyperthermia
Levetiracetam (Keppra)
Used as add-on therapy for partial seizures. Mechanism of action unknown. Has favorable adverse effect profile, with no life-threatening toxicity reported.
Adult
500 mg PO bid initial dose; increase by 1000 mg q2wk; typical dose 1000-3000 mg
Pediatric
Not established
None reported
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal impairment; major side effects include somnolence, asthenia, incoordination, mild leukopenia (3%) and behavioral changes such as anxiety, hostility, emotional lability, depression and psychosis (1-2%), and depersonalization
Pregabalin (Lyrica)
Structural derivative of GABA. Mechanism of action unknown. Binds with high affinity to alpha2-delta site (a calcium channel subunit). In vitro, reduces calcium-dependent release of several neurotransmitters, possibly by modulating calcium channel function. FDA approved for neuropathic pain associated with diabetic peripheral neuropathy or postherpetic neuralgia and as adjunctive therapy in partial-onset seizures.
Adult
75 mg PO bid or 50 mg PO tid initially; if needed, may increase dose to maximum of 600 mg/d
Pediatric
Not established
May cause additive effects on cognitive and gross motor functioning when coadministered with drugs that cause dizziness or somnolence
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Discontinue gradually (over a minimum of 1 wk) to minimize increased seizure frequency in patients with seizure disorders; may cause insomnia, nausea, headache, or diarrhea with abrupt withdrawal; common adverse effects include dizziness, somnolence, blurred vision, weight gain, and peripheral edema; may elevate creatinine kinase level, decrease platelet count, and increase PR interval; doses >300 mg/d associated with higher rate of adverse effects and treatment discontinuation; decrease dose with renal impairment (ie, CrCl <60 mL/min)
Clonazepam (Klonopin)
Long-acting benzodiazepine that increases the presynaptic GABA inhibition and reduces the monosynaptic and polysynaptic reflexes. Suppresses muscle contractions by facilitating inhibitory GABA neurotransmission and other inhibitory transmitters. Has multiple indications, including suppression of myoclonic, akinetic, or petit mal seizure activity and focal or generalized dystonias (eg, tardive dystonia). Reaches peak plasma concentration at 2-4 h after oral or rectal administration.
Adult
Initial dosing: 1.5 mg PO divided tid
Maintenance dosing: Increase initial dose by 0.5-1 mg PO q3d to a dose range of 0.05-0.2 mg/kg in divided doses; not to exceed 20 mg/d
Pediatric
<10 years: Initial dosing: 0.01-0.03 mg/kg/d PO bid/tid
Maintenance dosing: Increase initial dose by 0.5 mg PO q3d to a range of 0.1-0.2 mg/kg/d divided tid; not to exceed 0.2 mg/kg/d
>10 years: Administer as in adults
Phenytoin and barbiturates may reduce effects; coadministration of CNS depressants increase toxicity
Documented hypersensitivity; severe liver disease, and acute narrow-angle glaucoma
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in chronic respiratory disease or impaired renal function; withdrawal symptoms can result from abrupt discontinuation of the medication
Lorazepam (Ativan)
Sedative hypnotic with short onset of effects and relatively long half-life.
By increasing the action of gamma-aminobutyric acid (GABA), which is a major inhibitory neurotransmitter in the brain, may depress all levels of CNS, including limbic and reticular formation.
Important to monitor patient's blood pressure after administering dose. Adjust as necessary.
Adult
Status epilepticus;
4 mg/dose IV slowly over 2-5 min and repeat in 10-15 min prn; cumulative dose of 8 mg/d typically considered maximum
1-10 mg/d PO/IV/IM divided bid/tid
Pediatric
Status epilepticus;
Infants and children: 0.1 mg/kg IV slowly over 2-5 min; repeat prn in 10-15 min at 0.05 mg/kg; not to exceed 4 mg/dose
Adolescents: 0.07 mg/kg IV slowly over 2-5 min and repeat in 10-15 min prn; not to exceed 4 mg/dose
Toxicity of benzodiazepines in CNS increases when used concurrently with alcohol, phenothiazines, barbiturates, and MAO inhibitors
Documented hypersensitivity; preexisting CNS depression, hypotension, and narrow-angle glaucoma; reversal agents (eg, flumazenil) contraindicated when lorazepam used for life-threatening conditions (eg, control of intracranial pressure or status epilepticus)
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in renal or hepatic impairment, myasthenia gravis, organic brain syndrome, or Parkinson disease
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Further Reading
Keywords
Sturge-Weber syndrome, encephalotrigeminal angiomatosis, encephalofacial angiomatosis, Sturge-Weber-Dimitri syndrome, SWS, neurocutaneous disorder, angiomas, leptomeningeal angiomas, port-wine stain, PWS, cutaneous angioma
