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Adolescent Medicine: State of the Art Reviews Subspecialty Update April 2013 ? Volume 24 ? Number 1
By Donald E. Greydanus
American Academy of PediatricsCopyright © 2013 American Academy of Pediatrics
All rights reserved.
Neurological Problems in the Adolescent Population
Venu Parachuri, MD, Christopher Inglese, MD
Adolescent patients pose a unique challenge to physicians taking care of them. Although ultimately treatment decisions are made by parents, it is essential to consider and honor the opinion of the adolescent patient as treatment options are offered. This not only helps to gain their confidence and establish a successful physician-patient relationship, but also helps eliminate any misconceptions they might have about the disease or therapies. One needs to be considerate about the sensitivities adolescents may harbor and suggest a private interview, independent of parents, because teens might not be forthcoming about sexual activity and drug abuse, which might have an effect on the response to therapies being offered.
There are a number of neurological disorders that present in childhood and persist through adolescence, such as cerebral palsy, epilepsy, and muscular dystrophies, and some disorders, such as Guillain-Barre syndrome, tumors, and stroke, that can occur in any age group. There are some disorders like juvenile myoclonic epilepsy (JME) that occur almost exclusively in the adolescent age group.
Discussing pathophysiology and clinical features of all the neurological problems that occur in adolescents is beyond the scope of this article. We plan to discuss recent updates on the most common neurological disorders that are encountered by adolescent medicine physicians. We also briefly discuss recent neuroimaging techniques, deep brain stimulation and its application in the adolescent age group, and factors affecting the transition of care to an adult neurologist. There are many drugs that are used by pediatric neurologists that are not approved by the Food and Drug Administration (FDA) for neurological disorders, but have been safely and effectively used for many years based on anecdotal experience. They are discussed in other sections of this article and are mentioned as such.
MIGRAINES IN ADOLESCENT POPULATION
Venu Parachuri, MD, Christopher Inglese, MD
The overall prevalence of migraines in children and adolescent populations is about 7.7%, with a higher incidence in females. Evaluation of a child with headache begins with a thorough history. The interview should focus on the context of the headaches, precipitants and relieving factors, and a systems review to provide an impression as to whether the headaches are primary or symptomatic of a medical disorder or a concerning intracranial process. Hydration, nutrition, sleep, and recreation are important topics to discuss. It is important to address the possibility of nicotine, caffeine, and recreational substance abuse in an adolescent patient. Athletes must be asked to be forthright about the possibility of concussion. Paradoxically, post-concussive headache is inversely proportional to the severity of impact.
A detailed physical examination with emphasis on fundoscopic examination, visual acuity, and visual fields is important. CT or MRI is not routinely indicated in children with episodic headaches and a normal neurologic examination. Neuroimaging should be considered in children with an abnormal neurologic examination, particularly if there is unexplained impaired mentation, somnolence, and persistently depressed mood. Recent onset of severe debilitating progressive headache and a change in the type of headache qualify as indication for an advanced imaging study (Table 1).
Tables 2 and 3 summarize the criteria for pediatric migraine with and without aura.
The American Academy of Neurology (AAN) has recently updated the guidelines in the management of migraines in adults (Table 4, Table 5). However, there have not been any updates on the guidelines in the management of migraine in the child and adolescent population since 2004 despite the availability of new medication approved by FDA for use in adolescent population (Table 6, Table 7).
Treatment of headaches in children can be categorized into acute treatment, preventive treatment, and biobehavioral therapy. Acute treatment includes stepped analgesia, migraine specific abortive agents, and rescue strategies. Ibuprofen and acetaminophen can be safely used for acute treatment of mild, moderate, and severe migraine. When NSAIDs are not effective, triptans alone or a combination of triptan/NSAID can be used. Almotriptan is the only triptan approved by the FDA for treatment of acute migraine, but it is only available in an oral preparation.
When the patient is unable to tolerate oral medication, zolmitriptan or sumatriptan nasal spray is an alternative. A recently approved and marketed subcutaneous delivery device for sumatriptan is now available that claims to be painless and more readily tolerated.
There are no FDA-approved medications that can be used for prophylaxis of migraines in children and adolescents. There are many studies on various classes of medications showing their efficacy and safety when used for prophylaxis of migraines in children. Choice of prophylactic medication is based on the data extrapolated from adult studies and personal experience. It is important to discuss this frankly with parents, documenting caretaker understanding of this.
Parents should be neither frightened nor persuaded by this balanced discussion. Realistic expectations should be reviewed before initiating a daily preventive agent. It is unrealistic to expect a prophylactic compound to completely eliminate the occurrence of significant cephalalgia. Counseling should focus on the notion that significant reduction of debilitating attacks and the timely use of migraine-specific medications in adequate doses to prevent analgesic overuse can improve the quality of life of the migraine sufferer.
Amitriptyline followed by cyproheptadine and propranolol are the most commonly prescribed medications for migraine prophylaxis in children and are well tolerated.
New AAN guidelines recommend the following antiepileptic drugs (AED) for prophylaxis of migraines in adults: divalproex sodium, sodium valproate, and topiramate, but data is conflicting about the use of AEDs in pediatric population.
Few studies have shown the efficacy of valproate and topiramate for use in migraine prophylaxis in children. The choice of a preventive strategy should, when possible, take in to consideration comorbidities. For example, propranolol for an anxious patient, valproate for a subject with a mood disturbance or primary generalized epilepsy, and topiramate for an overweight child. Both valproate and topiramate should be avoided in pregnancy because of the risk of teratogenicity.
There is an increased risk of ischemic stroke in patients with migraine with aura, but the risk is less in patients younger than 35 years and who do not smoke. Migraine with aura is not an absolute contraindication for use of oral contraceptive pills and may be considered on case-by-case basis if benefits outweigh risks.
New AAN guidelines on complementary treatments for episodic migraine prevention in adults recommend butterbur for migraine prophylaxis. Riboflavin, magnesium, and feverfew are probably effective and coenzyme Q-10 (CoQ10) is possibly effective. In an open-label study performed on 108 children between the ages of 6 and 17 years by Pothmann and Danesch, headaches improved in 91% after 4 months of butterbur usage. CoQ10 and magnesium may be helpful and studies on riboflavin yielded mixed results. There are no trials on feverfew usage in pediatric migraines. Botulinum toxin A (Botox) was approved by FDA in adults with chronic migraine but is not yet approved for usage in pediatric age group. Botox was effective in reducing headache frequency in adolescents but more studies are needed.
Medication Overuse Headache
Medication overuse headache (MOH) is defined as headache present for more than or equal to 15 days/month or more often than not associated with the regular use of 1 or more acute/symptomatic drugs for longer than 3 months. During this period of time headaches have markedly worsened. Resolution or return to its previous pattern within 2 months after discontinuation of overused medication is an additional criterion. Most common factors associated with MOH are intake of more than 30 doses/month of an abortive or analgesic agent, smoking, alcohol consumption, and non-adherence to the prescribed regimen. Combination products and opiates may be more likely to engender MOH.
There are no established guidelines to manage MOH. Data is conflicting about abrupt withdrawal of the offending medications and when to initiate prophylactic medications. Management involves a multidisciplinary approach, patient education about the mechanism of medication overuse, and motivating them to reduce the frequency of the usage of offending medications. Corticosteroids and amitriptyline may be effective in counteracting the withdrawal symptoms.
Status migrainous (SM) is defined as a debilitating migraine attack lasting for more than 72 hours. There are no established guidelines for treatment of SM. Patients should be aggressively hydrated and antiemetics may be used for symptomatic relief of nausea. Intravenous valproate has similar efficacy to dihydroergotamine (DHE) and is better tolerated, but headache relief was greater with DHE/metoclopramide combination at 24 hours. Pregnancy test should be done in all adolescent females before DHE administration.
Biobehavioral therapy is an important aspect in the management of migraines. It complements pharmacologic therapy and may even help reducing the dose of preventive medications. It involves identifying triggers, lifestyle modifications, biofeedback, and relaxation techniques. The most common triggers of migraine in children and adolescents were stress, lack of sleep, climate change, and video games.
IDIOPATHIC INTRACRANIAL HYPERTENSION
Venu Parachuri, MD, Christopher Inglese, MD
Idiopathic intracranial hypertension (IIH) is a syndrome of elevated intracranial pressure in the absence of intracranial pathology and normal cerebrospinal fluid (CSF) composition. Although pseudotumor cerebri and benign intracranial hypertension are synonymously used, the term IIH is preferred since the condition is not benign and the word "tumor" in pseudotumor is misleading.
Annual incidence is 1 to 2 per 100,000 population. IIH occurs at an equal incidence in prepubertal boys and girls, whereas its incidence is higher in adolescent girls (70%). Obesity was observed in 64% of adolescent patients, whereas it was noted in only 26% of prepubertal patients.
Most adolescents present with headache followed by transient visual obscuration, vomiting, diplopia, somnolence, and tinnitus. Some patients can be asymptomatic and papilledema can be an incidental finding. Neurologic exam is usually normal, although patients can have visual field deficits and paresis of 3rd, 4th, or 6th nerve. Papilledema is observed in most of the patients with IIH;however, its absence does not rule out the diagnosis.
Conditions that are associated with elevated intracranial pressure (ICP) include, but are not limited to Addison disease, hypoparathyroidism, obstructive sleep apnea, polycystic ovarian disease, lupus, iron deficiency anemia, cerebral sinus venous thrombosis, and intracranial tumor. Commonly used medications in adolescents that can elevate ICP are tetracycline compounds (doxycycline, minocycline), vitamin A (isotretinoin), oral contraceptive pills, nalidixic acid, growth hormone, and anabolic steroids.
Normal CSF opening pressure in adults is less than 25 cm H2O, and in adolescents it can be confounded by technique, weight, pain, anxiety, sedatives, and analgesics used during the procedure. Classic radiological findings in IIH include empty sella, posterior globe flattening, vertical tortuosity of optic nerve, and optic nerve sheath distension. In a recent study, Butros et al proposed to include widening of the foramen ovale as an additional marker for IIH.
Non-pharmacologic treatment options include eliminating offending agent, low salt diet, and weight loss in obese patients. Acetazolamide can be started at a dose of 0.5 to 1 gram per day in divided doses and may be increased up to 3 to 4 grams per day. If vision loss is severe, surgical options are available that include optic nerve sheath fenestration and CSF shunting.
Topiramate is a reasonable alternative to acetazolamide given its secondary benefit in patients with headaches and also because of its weight loss properties. Visual acuity and visual fields should be monitored to assess the response to treatment. Younger patients seem to have favorable visual outcome. Recurrence may occur in first 18 months after diagnosis.
JUVENILE MYOCLONIC EPILEPSY
Venu Parachuri, MD, Christopher Inglese, MD
Juvenile myoclonic epilepsy (JME) is the most common idiopathic generalized epilepsy in the adolescent age group, with a frequency of 5% to 10 % of patients with epilepsy. It occurs with an equal frequency in males and females, with the onset between 12 and 18 years. Although a gene locus on chromosome 15 has been identified in patients with JME, its mode of inheritance remains uncertain. Clinical features include myoclonic jerks or generalized tonic-clonic (GTC) seizures usually on awakening from sleep. About one-third of patients present with absence seizures, which usually precede myoclonic jerks.
There is usually a precipitating factor in many patients, which include sleep deprivation, fatigue, photosensitivity, and menstruation in females. Neurologic examination is normal with the exception of a mild tremor noted in about one-third of the patients. Imaging studies are usually not indicated; however, a recent study demonstrated white matter abnormalities in the frontal lobe that might contribute to frontal lobe dysfunction in patients with JME. Interictal EEG is characteristic of generalized discharges of spike/double spike or polyspike and slow wave.
Valproate remains the drug of choice for treating JME, and the usual dose is 1 to 2 grams/day in divided doses. Treatment is lifelong; however, a recent 25-year long-term follow-up study showed that nearly 67% of patients became seizure free and antiepileptic medications were discontinued in 28% of patients. Appropriate choice of antiepileptic drugs (AED) is important since certain AEDs can aggravate or worsen absence seizures and myoclonic jerks.
Although not as effective as valproic acid, newer AEDs such as lamotrigine, topiramate, levetiracetam, and zonisamide can be considered an alternative to valproate to avoid potential teratogenic effect in child bearing age.
Patients should be counseled about adequate sleep, photosensitivity, and avoidance of alcohol and recreational drugs. Particular attention should be paid to interaction with other medications, especially oral contraceptive pills.
NEWER ANTIEPILEPTIC DRUGS IN ADOLESCENT EPILEPSY
Akshat Katyayan, MD, Venu Parachuri, MD, Christopher Inglese, MD
Epilepsy is a common pediatric neurological disorder affecting about 0.5% to 1% of the population. Most patients with epilepsy are treated with antiepileptic drugs (AEDs). Traditional AEDs have included phenobarbital, ethosuximide, primidone, phenytoin, carbamazepine, and valproic acid, most of which have been in use for many decades. Newer AEDs have included leviteracetam, topiramate, lamotrigine, and zonisamide, most of which were approved after the mid-1990s. The more recently introduced AEDs appear to provide a better side effect profile, fewer drug interactions, and less monitoring of serum AED levels.
The table on page 14 lists newer AEDs that have been approved by FDA in the past 4 to 5 years.
TICS AND TOURETTE SYNDROME IN ADOLESCENTS
Vidyashree Chikkaramanjegowda, MD, Venu Parachuri, MD, Katie M. Spangler, MD, Christopher Inglese, MD
A tic is a sudden, rapid, recurrent, nonrhythmic, stereotyped movement or vocalization. Tourette syndrome (TS) is a combination of motor tics and vocal tics and has other defining characteristics for diagnosis. The tics may appear simultaneously or at different periods during the illness and are not caused by a general medical condition or the effects of a substance or prescribed therapy.
Tic disorder (TD) may be transient or chronic. Transient tic disorder lasts anywhere between 4 weeks and 12 months. If the condition lasts longer than 12 months without a tic-free period of more than 3 consecutive months, it becomes chronic tic (CT) disorder. Not only are tics characterized according to duration, but they can also be characterized according to type.
Simple motor tics may manifest as eye blinking, shoulder shrug, head jerk, or nasal twitching. Complex motor tics are complex sequences of movements such as repetitive head banging, hand slapping, or neck jerking. Simple vocal or phonic tics range from simple throat clearing, grunting, humming, barking, and sniffing or can be more complex such as phrases, echolalia, and coprolalia.
Excerpted from Adolescent Medicine: State of the Art Reviews Subspecialty Update April 2013 ? Volume 24 ? Number 1 by Donald E. Greydanus. Copyright © 2013 American Academy of Pediatrics. Excerpted by permission of American Academy of Pediatrics.
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