Alison Suzanne Clay, MD
https://medicine.duke.edu/faculty/alison-suzanne-clay-md
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Early intervention can prevent epilepsy-induced disabilities, especially in children and adolescents (2). Moreover, refractory epilepsy is associated with higher mortality from sudden unexpected death than nonepileptic patients (79). Early control of epileptic seizures once a patient is considered drug refractory is crucial in preventing irreversible psychological disabilities and progressive cerebral dysfunction, as well as epilepsy-related death. The pooled proportions weighted by number of patients are also represented by the large vertical bar. The authors found that 58% of patients in the surgical group were seizure free at 1 year compared with 8% in the medical group (P 0. The authors also found that compared to the medical group, patients in the surgery arm had better scores for quality of life, rates of employment, and school attendance. Finally, they reported an unexpected death for one patient in the medical group and no deaths in the surgical cohort. Another study has reported that a larger proportion of patients who had temporal resections were seizure free in comparison with medically treated patients (72% versus 23%) (85). These studies included patients with variable pathologic substrates, and the time period covered by the authors and the number of incorporated papers were similar (Table 82. As might be expected, seizure outcomes reported by the different reviews were very similar, with seizure freedom after surgery averaging from 65% to 68% of patients. Evidence Report/Technology Assessment on Management of Treatment-Resistant Epilepsy, 2003. In two systematic reviews, the authors reported a higher proportion of long-term seizure freedom in patients with tumors when compared with other etiologies (86,87). In patients with temporal lobe tumors, two studies report that 65% of patients remain seizure free with follow-up of 9 years or more (21,64).
Other series also suggested that early epilepsy surgery in infants with catastrophic epilepsy may allow the resumption of developmental progression during critical stages of brain development and maturation (95,96). Mental development tends to progress in the majority of children after epilepsy surgery, in particular in those with initially no measurable development. Another series also showed a statistically significant increase in developmental levels at an average age of 21 months after surgery as compared to presurgical results (94). Early treatment and seizure control appear to be the key to improved developmental outcome. Consideration regarding the eloquent cortex involved and onset and type of lesion provide clues towards the necessary techniques for assessment of plasticity of function in the pediatric brain. Noninvasively presurgically obtained maps help guide further evaluation with subdural and depth electrodes, and may in the future even replace invasive monitoring in selected patients. There is increasing evidence that earlier treatment of epileptic seizures leads to improved developmental outcome. Functional magnetic resonance imaging in anesthetized patients: a relevant step toward real-time intraoperative functional neuroimaging. Brain mapping in sedated infants and young children with passive-functional magnetic resonance imaging. Reorganisation of descending motor pathways in patients after hemispherectomy and severe hemispheric lesions demonstrated by magnetic brain stimulation. Visually evoked blood flow response assessed by simultaneous two-channel transcranial Doppler using flow velocity averaging. Language and spatial attention can lateralize to the same hemisphere in healthy humans. Changes in cerebral blood flow induced by passive and active elbow and hand movements. Transcranial doppler assessment of cerebral flow velocity during perception and recognition of melodies. Bilateral simultaneous assessment of cerebral flow velocity during mental activity. Determination of language dominance: Wada test confirms functional transcranial Doppler sonography. Functional corticospinal projections are established prenatally in the human foetus permitting involvement in the development of spinal motor centres.
By 2002, figures showed that the annual use of dietary supplements had risen to 18. The low Herbal medicine use in specific patient groups (a) Cancer patients Certain groups of patients are known, or thought to have, a higher incidence of supplement usage than others. It is generally thought that cancer patients, for example, have an exceptionally high intake of herbal and nutritional supplements. The most commonly used herbal products for this purpose in 2005 were flaxseed, green tea and vitamins (C and E). Use of nonprescription dietary supplements for weight loss is common among Americans. Consumption of herbal remedies and dietary supplements amongst patients hospitalized in medical wards. Palliative patients tended to show more frequent herbal use than curative patients (78% versus 67%), whereas curative patients used herbal remedies much more often to relieve adverse effects (31% versus 3%). Whereas about 25% of the Asian and Hispanic elderly used herbal medicines, only about 10% of the black and white elderly used them; the herbs used, and the reasons for doing so, also differed according to ethnicity. The risk for adverse interactions was assessed in a Medicare population, using a retrospective analysis of Cardiovascular Health Study interview data from four different years. Of 5052 participants, the median age at the beginning of the study was 75 years, 60. Combinations thought to be potentially risky were noted in 393 separate interviews, with most (379 reports in 281 patients) involving a risk of bleeding due to use of garlic, ginkgo or ginseng together with aspirin, warfarin, ticlopidine or pentoxifylline. In a predominantly white (91%) elderly cohort, the use of dietary supplements was surveyed each year from 1994 to 1999 for an average of 359 male (36%) and female (64%) participants aged 60 to 99 years. By 1999, glucosamine emerged as the most frequently used (non-vitamin, non-mineral) supplement followed by ginkgo, chondroitin and garlic.
Electrocardiographic monitoring and echocardiography are frequently more valuable than electroencephalography in establishing the diagnosis. Narcolepsy and Cataplexy Narcolepsy is a state of excessive daytime drowsiness causing rapid brief sleep, sometimes during conversation or play; the patient usually awakens refreshed. Narcolepsy also includes sleep paralysis (transient episodes of inability to move on awakening) and brief hallucinations on arousal along with cataplexy, although not all patients demonstrate the complete syndrome. Cataplexy produces a sudden loss of tone with a drop to the ground in response to an unexpected touch or emotional stimulus such as laughter. Basilar Migraine Most common in adolescent girls, basilar migraine begins with a sudden loss of consciousness followed by severe occipital or vertex headache. Dizziness, vertigo, bilateral visual loss, and, less often, diplopia, dysarthria, and bilateral paresthesias, may occur. A history of headache or a family history of migraine is helpful in making the diagnosis. Children may respond to classic migraine therapy or antiepileptic drugs (105,106). Tremor An involuntary movement characterized by rhythmic oscillations of a particular part of the body, tremor may appear at rest or with only certain movements. Consequently, it is occasionally mistaken for seizure activity, particularly when the movement is severe and proximal such as in the "wing-beating tremor" of Wilson disease or related basal ganglia disorders. Examination at rest and during activities, possibly by manipulating the affected body part while observing the tremor, usually can define the movement by varying or obliterating the tremor. The electroencephalogram is unchanged as the tremor escalates and diminishes (107). Panic Disorders Panic attacks may occur as acute events associated with a chronic anxiety disorder or in patients suffering from depression or schizophrenia.
However, the soya milk brand taken in the case report did not contain vitamin K,3 and another reference 359 source lists soya milk as containing just 7. Why this product decreased the effect of warfarin is therefore open to speculation. Note that soy sauce made from soya and wheat is reported to contain no vitamin K, and soft tofu made from the curds by coagulating soya milk contains only low levels (2 micrograms per 100 g). In addition, the bacteria might continue to act in the gut to increase the synthesis and subsequent absorption of vitamin K2. Importance and management the interaction between warfarin and fermented soya bean products is established, marked and likely to be clinically relevant in all patients. Patients taking coumarin and probably indanedione anticoagulants should be advised to avoid natto, unless they want to consume a regular, constant amount. Although information is limited, it appears that soya protein might also modestly reduce the effect of warfarin. In particular, complete substitution of animal protein for soya protein appears to reduce the effect of warfarin. Case reports suggest that soya milk and soya oil may also interact, and therefore some caution would be prudent with these products. On the basis of known vitamin K content, whole soya beans could potentially reduce the effect of warfarin, whereas soy sauce should not. This would seem particularly important if they decide to change their intake of soya-related products. Effect of vitamin K intake on the stability of oral anticoagulant treatment: dose-response relationships in healthy subjects. Warfarin resistance associated with intravenous lipid administration: discussion of propofol and review of the literature. Bioavailability from varying formulations and extracts appears to be low, giving variable steady-state plasma concentrations. Flavonoids, which include kaempferol, quercetin, luteolin, hyperoside, isoquercitrin, quercitrin and rutin; biflavonoids, which include biapigenin and amentoflavone, and catechins are also present. Other polyphenolic constituents include caffeic and chlorogenic acids, and a volatile oil containing methyl-2-octane.
The skin should be examined for evidence of neurocutaneous disorders and the fundi for a cherry-red macula suggestive of a storage or mitochondrial disorder or for chorioretinitis indicating possible transplacental infection. Nearly half of all of the etiologic diagnoses are established or suspected by the historical and physical data. In the remaining 30% of cases, an etiology will be established for no more than one third, leaving about 10% of cases in which a diagnosis is determined by results of lumbar puncture or metabolic or genetic testing. Delayed myelination in 27% of patients did not appear to be associated with any specific etiology (Table 17. A trial of folinic acid is warranted (108), as is a 100-mg intravenous pyridoxine bolus to rule out pyridoxine-dependent seizures. Complete blood count, electrolytes (looking for an anion gap), and glucose determinations are appropriate. Nevertheless, such screening is not routine in all countries, and measurement of urine amino acid levels will detect phenylketonuria and maple-syrup urine disease, as well as other, rarer, metabolic diseases. Zellweger syndrome and neonatal adrenoleukodystrophy are other rare causes of hypsarrhythmia that can be diagnosed with the serum very-longchain fatty acid test (Table 17. In the 45 years since that report, efficacy has been repeatedly confirmed, but agreement is still lacking on the most appropriate dose and duration of treatment. Dosing is complicated by the existence of natural and synthetic forms of corticotropin. Studies of the synthetic product generally used much lower doses than studies of the natural product. Most children develop a Cushing syndrome with obesity, plethora, hypertension, and intense irritability. Corticotropin and, as of 2009, vigabatrin are approved by the Food and Drug Administration for use in the United States.
Diseases
This might be related to the duration of the epileptic abnormality prior to surgery (78). In reports by Patel and the Devinsky groups, a moderate improvement in language, social and behavioral function with a significant improvement in seizure frequency was reported (79). In four patients, the targeted seizures were eliminated but the progression of the disease continued. Consequently, patients are expected to experience transient dysfunction of transected cortex, with ensuing neurological deficits lasting for 2 to 3 weeks. We have reported previously a neurological complication rate of 15% with 7% suffering a permanent deficit. These included foot drop in 2%, language deficit in 2%, and a parietal sensory loss in 1%. Both procedures offer therapeutic options in patients previously rejected for more traditional resective surgery. The efficacy of corpus callosotomy has now been demonstrated in multiple centers around the world. Additional experimental and clinical studies are needed before this surgical procedure is fully integrated into the therapeutic armamentarium at all major epilepsy centers. Much of the success of both procedures depends on the proper selection of patients and the experience of the neurologic and neurosurgical teams. A learning curve should be expected whenever these procedures are newly implemented at a center. Surgical division of commissural pathways in the corpus callosum: relation to spread of an epileptic attack. The generalized convulsive seizures induced by daily electrical stimulation of the amygdala in split brain cats.
Summary: the evidence suggests that phenobarbitone and phenytoin are not different in efficacy outcomes for neonatal seizures. Although response rates were not statistically different, the evidence is insufficient to prove equivalence in the treatment of generalized, tonic-clonic and partial seizures in adults and children with phenytoin better tolerated. Carbamazepine vs Valproate Marson et al120 performed a meta-analysis comparing valproate and carbamazepine therapy in the treatment of epilepsy in children and adults in which a misdiagnosis of epilepsy could not be ruled out. Assessed outcomes included the retention time, time to first post-randomization seizures and time to 12-month remission. In the setting of generalized-onset seizures no difference was found between treatments. Summary: Evidence, which may be confounded by misclassified seizure type, finds both carbamazepine and valproate were equally efficacious in the treatment of generalized and partial-onset epilepsy in adults and children. Methsuximide 59 Methsuximide was prospectively studied in an open-label protocol of 112 children with epilepsy refractory to first line antiepileptic drugs or combinations or antiepileptic drugs. Methsuximide serum concentrations were positively correlated with reversible ataxia and leukopenia when >45 mg/L. Tennison et al122 found methsuximide efficacious when added to the current regiment of children with intractable epilepsy on maximal, combination therapy. A reduction in seizure frequency was achieved in >50% of children and was maintained for a mean of 19 months although no patient achieved complete seizure remission. Dasheiff et al123 compared clorazepate, methsuximide and valproate treatment in 66 patients with medically refractive, complex partial seizures with secondary generalization or partial seizures with aura who failed phenytoin, carbamazepine or phenobarbital therapy. The elimination of seizures with tolerated side effects occurred in small numbers of patients similarly with each medication. Methsuximide and valproate use resulted in gastrointestinal and mental status changes and valproate therapy resulted in impaired coordination. Summary: In children and adults with refractory epilepsy who failed therapy with multiple medications, limited, low quality evidence suggests methsuximide can reduce the seizure frequency in ~ 1/3 of patients, although the effects do not appear to be long-lived. A small trial suggests methsuximide efficacy may compare favorably with clorazepate and valproate. Methsuximide is associated with gastrointestinal toxicity and mental status changes and at least some of methsuximide adverse effects are dose related (ataxia, leukopenia). Acetazolamide Acetazolamide was assessed for utility as adjunct therapy in 37 Japanese children with refractory epilepsy complicated by mental retardation currently failing therapy to a combination of at least two of the following medications, carbamazepine, clonazepam or sodium valproate124.
The attitude towards this definition is often not positive because it is felt that patients with seizures arising from multiple sites in the brain are usually not helped by surgery. Based on this thinking, one could easily make the argument that such patients are not candidates for surgery. However, an alternative and compelling theory is that in multifocal epilepsy, seizures are actually spreading rapidly between brain regions such that the true ictal-onset zone eludes detection by current methods. Correlating the outcome and electrographic ictal patterns in 26 patients with neocortical epilepsy, Kutsy et al. Intriguingly, a critical review of the literature reveals several examples of successful epilepsy surgery in which a targeted, focal, surgical approach was utilized to treat apparent multifocal epilepsy, rather than resections in multiple areas of the brain. The challenge, therefore, is to utilize technological advances in anatomic imaging and functional studies to unmask the possibility of an occult seizure focus that may not yet be revealed in a seemingly multifocal seizure network. The goal of treatment in patients with multifocal epilepsy is the elimination of seizures. Especially in children, one should strive to eliminate seizures as soon as possible, in order to optimize the neurologic setting for improved cognitive development, education, and quality of life (16). Of course, any decision one makes about multifocal resections should be made together with members of a comprehensive team and the family, weighing all the possible risks and potential benefits as they pertain to the individual patient. A review of the literature on multifocal resections for epilepsy does not provide a clear understanding of the indications and outcome of this approach. In fact, very few 957 958 Part V: Epilepsy Surgery investigators have directly studied this group of patients as an independent entity. Moreover, the available outcome data on the likelihood of becoming seizure-free after resections in multifocal epilepsy are disappointing. For example, only 37% of patients with seizures secondary to viral encephalitis, a classic etiology for multifocal epilepsy, were seizure-free at long-term follow-up (29).
Importance and management There is a good body of evidence, which suggests that soya products or soya isoflavones might increase the dose required of thyroid hormone replacement therapy. It would seem prudent to closely monitor the resolution of primary hypothyroidism in infants receiving soya formula, and expect to use higher dose of levothyroxine than anticipated in these individuals. Monitor thyroxine levels, and either discontinue the soya formula or further increase the dose if necessary. Similar precautions would seem prudent if patients receiving levothyroxine wish to take soya supplements; however, remember that the intake of soya supplementation will need to remain relatively constant. Use of soy protein supplement and resultant need for increased dose of levothyroxine. Persistent hypothyroidism in an infant receiving a soy formula: case report and review of the literature. Abnormal thyroid function tests in infants with congenital hypothyroidism: the influence of soy-based formula. However, other soya products such as dried textured soya protein and fresh soya beans are unlikely to contain important amounts of tyramine. Successful treatment of a monoamine oxidase inhibitor-tyramine hypertensive emergency with intravenous labetolol [sic]. Tyramine content of preserved and fermented foods or condiments of Far Eastern cuisine. Effects of soy protein and soybean isoflavones on thyroid function in healthy adults and hypothyroid patients: a review of the relevant literature. However, other soya products such as dried textured soya protein and fresh soya beans are unlikely to contain important amounts of tyramine. Clinical evidence A 33-year-old woman taking tranylcypromine 10 mg four times daily presented to an emergency department with global headache and stiffness of the neck and was found to have a blood pressure of 230/140 mmHg and bradycardia of 55 bpm. Tyramine is an indirectly acting sympathomimetic amine, one of its actions being to release noradrenaline (norepinephrine) from the adrenergic neurones associated with blood vessels, which causes a rise in blood pressure by stimulating their constriction. Significant amounts of tyramine may be present in fermented or preserved soya products such as soy sauce and tofu, and it may be prudent to avoid these Soya + Nicotine For discussion of a study showing that soya isoflavones (daidzein and genistein) caused a minor decrease in the metabolism of nicotine, see Isoflavones + Nicotine, page 261.
Frithjof, 40 years: During the second stage, neurons migrate away from their place of origin toward the pial surface and settle within the cortical plate. Diazepam rectal gel is effective in preventing subsequent seizures during seizure clusters (170172) and can reduce the frequency of emergency department visits (113). Hyponatremia, defined as a serum sodium level lower than 115 mEq/L, is one of the most frequently reported metabolic abnormalities, affecting 2.
Givess, 29 years: Despite some progress, the crisis the Commission found in 2003 remains, and the federal government continues to fail to support adequately the social and economic wellbeing of Native Americans. Visual hallucinatory seizures are occasionally characterized by complex visual perceptions. The effect of echinacea (Echinacea purpurea root) on cytochrome P450 activity in vivo.
Saturas, 57 years: Feeling bad about themselves occurs more often in children and adolescents with epilepsy when compared to other children and adolescents with or without chronic illnesses. Predicting long-term seizure outcome after resective epilepsy surgery: the multicenter study. In the infant with diffuse, very high intracranial pressure, lumbar puncture should be delayed until antibiotics and pressure-reducing measures are initiated.
References
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