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He had was to become the Princess Margaret Rose the capacity for a great buy cheap meloxicam 15 mg online arthritis in lower back, continuing but quiet hap- Orthopaedic Hospital were already on the piness generic meloxicam 7.5 mg overnight delivery arthritis pain medicine for dogs. The concept conceived by Robert Some of his happiest times were spent with the Jones and developed at Oswestry was to be Monks of St cheap meloxicam 7.5mg on line arthritis lumbar. The orthopedic hospital is on the dines in monks’ habit on beer and sausages cheap 15mg meloxicam otc arthritis triggers, regal- edge of the city, surrounded by trees, facing south ing each other with tales and humorous verse. He over the Pentland Hills, and is perhaps the loveli- was in all ways a gentle man, aware of the human est site of any orthopedic hospital in Britain. It dignity of his patients, even the most fractious or has now grown to nearly 300 beds. In his early years he wrote a book of though a traditionalist and conservative to the fairy stories. In Wyn he of orthopedics, if one could argue to his satisfac- found a completion to his life, a fulfilment of his tion that it was necessary. He had seemed a confirmed bachelor; was held by his colleagues was reflected in his in reality he was an idealist who was prepared to election as vice president of the British Orthope- wait. Having all his life per- leagues tirelessly traveled over the many counties suaded the crippled that disablement was not a brought into their ambit. At one time, 95 clinics disability, as one might expect, he learned to walk were in being in southern Scotland. He became a appearance of tuberculosis, poliomyelitis, rickets nimble septuagenarian, drove his car, became an and many other crippling diseases of children and enthusiastic gardener and attended public dinners. Derick Strange was an articulate and enthusi- astic teacher, an original thinker rather than a regurgitator of the views of others. His initiative resulted in the beginning of postgraduate teach- ing at the Kent and Canterbury Hospital and the eventual opening of the Kent Postgraduate Medical Centre. His most important contribution to the devel- opment of orthopedic surgery was probably the publication, in 1965, of his monograph entitled The Hip. It ran to only 284 pages, but Norman Capener, in his review of it in The Journal of Bone and Joint Surgery, said that “in a special way, this book... Clair Strange’s ability to convey complex ideas simply by his own drawings. He was a member of the STRANGE original group of ABC Traveling Fellows, and, at 1911–2002 86 years of age, published The History of the ABC Club of Traveling Fellows. Clair Strange was born on book will surely be a valued possession of every July 22, 1911, in Moh Kan San, near Shanghai, member, and is a very good “read” for any ortho- to Dr. Charles Frederick Strange, of the Church pedic surgeon in the English-speaking world, Missionary Society, and Olive Cecilia Strange if only for the insight it gives into the earlier (née Harrison). He was educated at Rugby School exploits of some of the present-day leaders of the and the London Hospital Medical College. Strange won the Robert Jones Gold Medal and After a number of junior hospital posts, he spent Association Prize for his essay on amputations the years of World War II as senior surgeon at in 1943, and was a Hunterian Professor in 1948. Dunston Hill Hospital, near Newcastle, where he He was a member of the editorial board of The had sole charge of 200 beds for wounded service Journal of Bone and Joint Surgery from 1964 to personnel. He has been president of the orthopedic He was appointed to the Kent and Canterbury section of the Royal Society of Medicine, and and Ramsgate Hospitals as an honorary consult- vice president of the British Orthopedic Associa- ant in 1947, a year before the beginning of the tion. At this time, fractures four surgeons who were honorary fellows of the were managed by general surgeons and orthope- British Orthopedic Association and who were dic clinics were organized by county councils or British by birth. Strange realized the For nearly 30 years, he was honorary surgeon need for a long-stay hospital for orthopedic to the Kent County Cricket Club, and served as patients in East Kent and established an orthope- honorary civilian consultant to the army from dic unit at the Royal Sea Bathing Hospital in 1967 to 1976. His East Kent colleagues inaugu- Margate, which was then being used for the man- rated an annual FG St. He died during the night preceding the 2002 success of the treatment of this disease by drugs, lecture. He was only par- 327 Who’s Who in Orthopedics Georg Friedrich Louis Ernest Adolph Gustav Gottfried STROMEYER STRÜMPELL 1804–1876 1853–1925 Georg Friedrich Louis Stromeyer of Hanover, at Ernest Adolph Gustav Gottfried Strümpell of times professor at various German universities Leipzig published a two-volume edition of the and surgeon general of the Hanoverian army, was Lehrbuch der Speciellen Pathology and Therapie one of the most powerful influences in develop- der inneren Krankheiten in 1883–1884. He was ing the surgical aspects of orthopedic surgery in among the leading internists and pathologists of the mid-nineteenth century. The masterpiece went through tribution was the popularizing of subcutaneous more than 30 editions, translated into several lan- tenotomy and the encouragement of its practice guages, with the first in English appearing in throughout the western surgical world. The volumes are still valid references in in the preface to his masterful and influential pathology with a wealth of forgotten and redis- book, he gives credit to his predecessors who covered musculoskeletal material pertinent to attempted the procedure sporadically, Stromeyer contemporary studies. His name is eponymic with applied it to deformities wherever contracted spondylitic syndrome. To appreciate the importance of subcutaneous teno- tomy, the horrendous proportion and devastating effects of open surgery in pre-Listerian days must be kept in mind. As becomes obvious in this preface, the technic of Stromeyer’s procedure was used as a wedge for the development of muscu- loskeletal surgery vis-a-vis the brace and stretch- ing era of the early days of our specialty. His first paper on the subject was published in 1833; however, the classic of Stromeyer was his book on Operative Orthopedics. Even within his lifetime, the Syme amputation was recognized as a major technical advance. Hudson, The Mechanical Surgery, described the Syme amputation as follows: “No amputation of the inferior extremity can ever compare in its value to the subject with that of the ankle joint originated by Mr. That same year he resigned the chair James Syme was born in Edinburgh in November at the University of Edinburgh, to which he had 1799, the son of well-to-do parents. He school days he was fascinated with the subject of was buried in the family vault at St. In the Scotsman Philosophy a new solvent for India rubber derived newspaper of June 20, 1870, there is a resume of from coal tar. Syme’s character and achievements as a Glasgow manufacturer named Macintosh, and surgeon. It is believed to be from the pen of had Syme followed the advice of his friends, our Joseph Lister: “The most prominent feature of rainproof garments today might be referred to as Mr. Syme’s character was uncompromising truth- “Symes” and not “Macintoshes. In 1833 he was appointed to the chair of clinical surgery at the University of Edinburgh and was given an appointment on the staff of the Royal Infirmary. In 1853 Joseph Lister migrated from London to Edinburgh and established a warm relationship with James Syme. By his marriage with Agnus, Syme’s eldest daughter, Lister became the son-in- law of the distinguished professor and acted as his assistant and substitute on many occasions. Syme introduced conservative alternatives to major amputations and is best remembered for his contribution of ankle disarticulation with preser- vation of the heel pad as an alternative to below- knee amputation. Since cartilage is more resistant to infection, the postoperative healing with this new operation, reported by Syme in 1844, was much better than with other traditional types of 329 Who’s Who in Orthopedics he was fully alive to the advantages of medical training, and sent all his five sons to a medical school—surely a unique occurrence for one family. The eldest and the youngest, Hugh Owen and John Lewis, became widely known, one as a pioneer of orthopedic surgery and the other as a leader in gynecology.

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Infants tend to have less of a physiologic safety factor compared to older children and adults proven meloxicam 15 mg arthritis in dogs licking. Junctional failure at a single synapse is an all-or-nothing process cheap 7.5mg meloxicam with visa arthritis in neck cause sore throat, but at the level of the motor neuron it is graded order meloxicam online pills arthritis in fingers relieve, as some muscle fibers may fail to contract while others respond normally purchase meloxicam 15mg with mastercard arthritis pain in elbow. The specifics of physiologic testing for neuromuscu- lar junction failure are complex and beyond the scope of this chapter, but excellent reviews exist. General features of enhancing the diagnosis of specific disorders of neuromuscular disorders in children are discussed below. SPECIFIC DISORDERS Autoimmune Myasthenia Gravis Autoimmune myasthenia gravis (MG) is by far the single most common disorder of the NMJ in children. This disorder is more common in adults, but can affect children as early as late infancy. In children, the general incidence correlates with age; MG is rarest in the youngest. It tends to affect orbital and bulbar-innervated muscles more than appendicular muscles, but there is wide individual variation. Muscle power may be normal after a period of rest, but with repeated activation available power drops quickly. The MG can manifest over a range from trivial to cata- strophic; the course can be stable and predictable or extraordinarily capricious. In general, the severity and course of any one individual with MG will declare itself relatively early in the course. For example, more than half of affected children have symptomatic weakness restricted to orbital muscles; if early eye muscle involvement does not spread more widely for 6 months it is unlikely to do so thereafter. Similarly, the tendency for minor intercurrent illness to trigger a sudden exacerbation that requires urgent medical intervention usually manifests within the first year. Therapy for Neuromuscular Junction Disorders 203 Diagnosis The diagnosis of MG is sometimes simple, sometimes extraordinarily difficult. Fortunately, however, the general difficulty of establishing a definitive diagnosis is inversely related to the need for that diagnosis. In those with severe illness the diag- nosis is usually straightforward, while the diagnosis in those with subtle transient symptoms may never be established with certainty. Clinical suspicion of MG should be increased whenever weakness is variable from time to time or with fatiguing effort. A frequent clinical test is to examine for decreasing range of extraocular mobility or ptosis with sustained up gaze. Another test is to look for early fatigue while sustaining a horizontal anteverted posture of the arms. In addition, four lines of evidence can contribute to the diagnosis: A positive test for antibodies against the acetylcholine receptor (anti-AChR antibodies) has the best predictive value of any single test. Unfortunately, anti-AChR antibodies are less often found in children than adults with MG, and much less often found in those with weakness restricted to the orbital muscles. In the practice of pediatrics, the antibody test thus often operates as does a ‘‘one way ratchet’’—useful if positive but not if negative. Electrodiagnostic testing, demonstrating a characteristic decremental response of muscle action potential amplitude—generally considered as anything greater than 10–15%—decreases from the first to the fourth or fifth response at 2–5 Hz stimulation, can be very useful, particularly if it can be shown to improve following rest or anticholinesterase treatment. Unfortunately this is an insensitive test, particularly in children and when weakness is transient. It is absolutely necessary to obtain a stable baseline and repeated testing, which are difficult to obtain in a less than fully cooperative child. When done poorly, false interpretation of testing to be abnormal is a risk. Sedating the child will improve the sensitivity of the test somewhat and substantially improve test specificity, but sedation of the child with enhanced potential for bulbar muscle weakness must be done in a controlled environment and carefully followed, increasing the morbidity and emotional and financial costs of the test. Very specialized electrodiag- nostic testing with a stimulated single fiber technique, looking for abnormal jitter and block, is occasionally useful. Evaluation of the clinical response to short-term anticholinesterase inhibi- tor medications can be very useful, but care must be taken to insure a qual- ity test. To be useful as a test for mysathenia, the child must manifest partial and easily testable weakness in a muscle at the time of treatment. A positive test is one where dramatic improvement can be demonstrated within seconds of infusion and the improvement then lapses back to baseline over a period of minutes. Because edriphonium clearly produces autonomic symptoms, care must be taken not to interpret as positive responses that could be attributable more simply to vigorous stimulation—such as the resolution of ptosis in a sleepy infant. In very subtle cases of transient symptoms—such as in children with vari- able ptosis, the sensitivity of each of the above methods may be limited. Use of a randomized paired on=off trial testing standard oral anticholines- terase medications with a blinded observer can be useful in some such cir- cumstances. One parent can give the medication, while the other is instructed to score whether or not the symptom of concern is improved. The observer should also be asked to iden- tify what were the most distinctive features—with post hoc reporting of observed anticholinergic features diminishing the power of the test. Treatment Treatment for children with confirmed MG should be tailored to clinical need. The range of required therapy is vast, with some requiring no therapy and some necessi- tating substantial intervention and tolerance for its associated morbidity (Table 1). Therapeutic modalities include short-term treatment of symptoms with acetylcholi- nesterase inhibiting agents, intermediate term treatment of the antibody response with plasmapheresis and IVIG, and long-term modifiers of the disorder with corti- costeroids, other immunosuppressives, and thymectomy. Oral acetylcholinesterase inhibitor therapy with pyridostigminine bromide can minimize weakness transiently. The onset of action is rapid, and duration of action is approximately 3–4 hr. Dosage can be increased slowly, titrating for benefit and the absence of side effects, usually abdominal cramping and diarrhea. Too high a dose may increase weakness, so the dose should not be increased too rapidly. Dosing is best at 4 hr intervals, with 3, 4, or 5 doses daily with meals to minimize GI side effects. An extended form is available for those who have symptoms in the morning before taking their dose. This come at only 180 mg doses, however, which is generally useful for the adult or child taking 60 mg or more with each dose. Use of the timespan form of pyridostigmine other than at night is contraindicated because of wide variation in daytime absorption and elimination pharmacokinetics. Intrave- nous neostigmine by continuous infusion can be substituted for oral mestinon in an ICU setting during crises, to tailor dosage and effect, and when oral medications cannot be tolerated.

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In addition to his membership in local and regional orthopedic societies purchase meloxicam 7.5mg overnight delivery arthritis in back causing hip pain, he belonged to the Inter- urban Club and the Société Internationale de Chirurgie Orthopédique et de Traumatologie (SICOT) buy generic meloxicam 7.5mg line rheumatoid arthritis ulnar nerve. His ability to see the essence of a problem order 15mg meloxicam amex arthritis relief knuckles, a situation buy meloxicam 15mg line arthritis hands medication, or an issue quickly and to express it suc- cinctly and often humorously made him a favorite with patients and residents alike. Always a gentleman, he was considerate and kind to even the youngest of medical students, and many young residents had their egos boosted by Andrew Hoyt CRENSHAW being asked their opinions by “The Editor of 1920–1991 Campbell’s. His last 2 years of retirement allowed Presbyterian College in Clinton, South Carolina, him to spend time with her; his children, Andrew, and his MD degree from Emory University in Tom, Sarah, and Jeannie; and his five grand- Atlanta. He finally had time for mowing the lawn service in the United States Army Medical Corps, and walking with McGregor, his Scottish terrier from July 1945 through January 1947. That he chose to spend completion of an orthopedic residency at the a large part of retirement editing the most recent Campbell Foundation, he joined the staff of the edition of “The Book” reflects his commitment to Campbell Clinic in 1951. Crenshaw’s skill as a surgeon and Throughout his 37 years as an orthopedic acumen as a diagnostician were quickly evident, surgeon, Dr. Crenshaw personified the old- his partners soon discovered another talent. After fashioned values of duty, loyalty, integrity, and contributing two chapters to the third edition of excellence. His death on October 18, 1991, left us Campbell’s Operative Orthopedics in 1956, he with one fewer of the “giants” on whose experi- became the editor of the fourth edition in 1963. He served in that capacity for five editions of this voluminous text, completing work on the eighth edition only days before his death. A colleague described his work as follows: “Hoyt reads what I’ve written, then writes what I meant to say. Crenshaw was an associate editor of The Journal of Bone and Joint Surgery from 1963 through 1972, as well as of the AAOS Bulletin and the Journal of Continuing Education in Orthopedics. He contributed numerous articles to the orthope- dic literature, many of them now classics. Crenshaw gave fully of his time and talents to orthopedic specialty societies. Among his duties, he served as librarian–historian of the 82 Who’s Who in Orthopedics On the basis of this work, he can be considered to be one of the originators of the technique of compression plating. Robert DANIS 1880–1962 Robert Danis was born in a small town in the Belgian province of Tournai. After classical studies at Anvers and Louvain, he studied medicine at the William DARRACH University of Brussels, from which he received his degree in 1904. He became associated with 1876–1948 Antoine Depage and moved up through the system, becoming professor of operative surgery Dr. William Darrach was born in Germantown, and gynecology in 1919 and succeeding his chief Pennsylvania, the son of William and Edith as professor of clinical surgery in 1925. School and was graduated from Yale University He made significant contributions to the field in 1897. Following his graduation from the of local and regional anesthesia, especially sacral College of Physicians and Surgeons in 1901, he blocks. His interest in vascular surgery led to the served an internship at the Presbyterian Hospital. Beginning appointed Associate Attending Surgeon at the in 1925, his work gradually focused on trauma- Presbyterian Hospital, and continued to serve tology and especially on the operative treatment there in varying capacities until his death. Darrach went overseas Danis pursued his interest in the laboratory as with Base Hospital 2, serving as Chief of Surgi- well as the clinic. He studied the biology of frac- cal Service, later as Consulting Surgeon for the ture healing and demonstrated that primary union First Army, and, still later, for the Third Army. He (soudure autogene) of fractures could occur was discharged, after serving with distinction, without callus formation when the fracture frag- with the rank of Colonel. His final conditions, he designed a complete armamen- service to the government was in the post of tarium of compression plates (coapteurs) and Director of Education and Research at the screws. The results of his clinical and laboratory Kingsbridge Veterans Administration Hospital. After his retirement as Dean, fol- lowing the completion of the great Columbia– Presbyterian Medical Center, which towers over Washington Heights, his colleagues attested in formal resolution that he, above all others, had been responsible for the successful alliance between the Presbyterian Hospital and other hos- pitals and the School of Medicine, resulting in the Medical Center. Darrach then returned from administration to his real abiding interest—surgery—and estab- lished the fracture service. To the mending of broken bones, he brought his keen analytical mind, his mature judgment, and rare skill. Many of them caught some of the fire of his inspiration, and, through those who did, his influence will continue. Darrach was distinguished in every stage of Robert Merle D’AUBIGNÉ his career, and his pre-eminence was recognized by countless honors. He served as President of the 1900–1989 Association of the American Medical Colleges, of the American Surgical Association, and of the Society of Clinical Surgery. He was a Regent of Robert Merle d’Aubigné was born in 1900 at the American College of Surgeons. He was an Neuilly, just upstream from Paris, into a family honorary member of the American Academy of with a long Huguenot tradition; his father was the Orthopedic Surgeons. He received many honorary degrees practice at skiing and boating, while many family from universities at home and abroad. He wrote contacts across the Channel led to a mastery of numerous articles, and always spoke clearly, spoken English. Some remarkable wartime tutors intelligently, and with a delightful sense of humor. Robert just He was a devoted, radiant, and generous person, missed active service in 1918 but soon turned to loved by countless friends, among whom were his adventurous mountaineering, which eventually many patients and associates. William Darrach died on May 21, 1948, experience of a fractured femur treated by trac- after a brief illness. Early on in the rigorous training in general surgery of that period, Robert found that his pro- fessors paid scant personal attention to fractures and other lesions of the limbs. It was only in his fourth year as a junior resident aged 27 that he came under the influence of a surgeon, Paul Lecène, with a deep interest in skeletal disorders. Two years later Lecène proposed coauthorship of a work on elective orthopedic surgery but almost at once died tragically from typhoid fever. The next year Robert made a long pilgrimage to Böhler in Vienna and then another to Putti in Bologna. These visits were the main source of a recurrent dream of such treatment equally well organized all over France. Meanwhile he had to concentrate hard on general surgery in order to 84 Who’s Who in Orthopedics attain the coveted rank of chirurgien des hôpitaux elled widely, especially in Great Britain and North in 1936. America, always seeking advances, often lectur- The declaration of war in 1939 and the long ing and generally acting as a roving orthopedic retreat of 1940 found Merle d’Aubigné as Captain ambassador for France. Not only did tion he worked in Paris under the double strain of it provide every facility for practice and for teach- belonging to the resistance and of protecting his ing, but also accommodation for an excellent first wife, Bibka, from deportation.

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Given our current state of knowledge cheap meloxicam 7.5 mg line arthritis relief medication, a rational plan of treatment focuses on counseling and on preventing prolonged febrile convulsions generic meloxicam 15 mg with amex arthritis knee numbness. Prolonged febrile convulsions are the only ones that have been causally implicated with adverse outcomes order meloxicam 15 mg line rheumatoid arthritis tmj. While the data are reassuring effective 7.5mg meloxicam arthritis treatment pdf, the clinician needs to recognize that febrile seizures are a very frightening event. Parents need to be reassured that the child will not die during a seizure, a fear that seems to be widespread. They also need to be provided information about the prognosis and management of febrile seizures. Prac- tice parameter: the neurodiagnostic evaluation of the child with a simple febrile seizure. American Academy of Pediatrics, Committee on Quality Improvement, Subcommittee on Febrile Seizures. Practice parameter: long-term treatment of the child with simple febrile seizures. Berg AT, Shinnar S, Darefsky AS, Holford TR, Shapin ED, Salomon ME, Gain EF, House WA. Long-term intellectual and behavioral outcomes of children with febrile convulsions. INTRODUCTION Defined by Gastaut in 1966, but first recognized in 1939 by Lennox, Lennox– Gastaut syndrome (LGS) has attracted considerable interest in the last half century. Responsible for less than 5% of childhood epilepsies, this catastrophic epilepsy con- sists of a typical triad of intractable seizures (tonic axial, atonic, myoclonic, and atypical absence), characteristic EEG abnormalities (bursts of slow spike and wave during the awake state and 10 Hz—fast rhythms with bursts of slow polyspikes during sleep state), and cognitive delay. Its disabling course and refractory nature give it an importance out of proportion to its incidence. DIAGNOSIS The diagnosis of LGS is clinical and rests on a constellation of clinical characteristics and electrographic findings. Characteristic Seizures Children with LGS have multiple seizure types, and their epilepsy is medically intractable with high frequency of seizures. The onset is in childhood, typically between 1 and 8 years with greatest frequency between 3 and 5 years. Seizures most commonly include tonic axial seizures, often nocturnal during non-REM sleep and associated with autonomic phenomena. Frequently described seizures include atonic and tonic seizures resulting in drop attacks, and prolonged atypical absence with automatisms sometimes accompanied by generalized seizures. Other seizure types may occur and predominate, such as in the myoclonic variant, or evolve, such as the increased frequency of generalized tonic–clonic convulsions in adolescence. Seizures usually occur multiple times a day, are usually brief but may be repetitive or prolonged. Tonic or 79 80 Vanderver and Gaillard atypical absence status may occur frequently in some patients. Frequent falls because of repeated seizures are disabling in many children with LGS and may result in injury. Characteristic EEG Findings Electroencephalographic tracings are not diagnostic, as in some epilepsy syndromes, but are characteristic of the diagnosis. The background is abnormal, with lower than age appropriate frequency of the posterior basic rhythm and intrusion of slow activ- ity generally. During wakefulness, generalized, bisynchronous slow spike-and-wave and polyspike-and-wave discharges (1–2. These may occur in bursts or as near continuous activity, and are most often irregular in frequency, distribution, and amplitude. This pattern may be asymmetric and intermixed with bursts of faster activity. Hyperventilation may increase the fre- quency of slow spike-and-wave discharges, but photic stimulation produces no par- oxysmal activation. During sleep, bursts of generalized fast spikes are seen at 10 Hz or more, maximal in non-REM sleep, and are the electrographic correlate of noctur- nal tonic seizures. They may obscure normal sleep architecture and, while not the pattern of electrographic status epilepticus of sleep (ESES), may occupy up to 50% of the recording. Generalized spikes, and in some patients multifocal spikes, can be seen throughout the recording. Neuropsychologic Disturbances Mental retardation is almost universal in LGS, with fewer than 10% of patients pre- serving near-normal intellectual functioning. Children may appear normal or near normal at onset with an abrupt deterioration following the onset of uncontrolled sei- zures, or may have a preceding encephalopathy, including infantile spasms. Overall, cognition progressively deteriorates, compounded by repeated trauma from falls and the effects of multiple anticonvulsants. Behavior problems abound and include autis- tic spectrum disorders, aggressiveness, and hyperactivity. EVALUATION—ETIOLOGY The differentiation of LGS from other catastrophic onset childhood epilepsies is important for prognosis and management. The seizure pattern may not appear char- acteristic initially and suggestive EEG patterns may not appear for several months. Therefore, it is not unreasonable to pursue diagnostic testing that may be suggested by the individual’s presentation—for example, that of progressive myoclonic epi- lepsy in a patient in whom the initial presentation includes myoclonic seizures. Very few patients with LGS, however, have documented abnormalities of metabolism. Concerns that LGS may be related to a developmental channelopathy or be an immune-mediated process have yet to be substantiated. In some series, 17–30% of patients with LGS have a history of infantile spasms. Therefore, central nervous system insults known to predispose to infantile spasms have been implicated in the pathogenesis of LGS. These include congenital infec- tions, sequela of neonatal hypoglycemia, hypoxic–ischemic encephalopathy, and traumatic brain injury. Lennox–Gastaut Syndrome 81 Structural abnormalities are the most common underlying etiology of sympto- matic LGS. Rarely, brain tumors have been known to cause LGS, although this raises the difficulty of differentiating true LGS from secondary bilateral synchrony. Tuberous sclerosis or other neurocutaneous syndromes are found in children with LGS, but not as frequently as in association with infantile spasms. Developmental brain malformations are the most common structural lesion to be reported in LGS, especially subcortical band heterotopia, bilateral perisylvian syndrome, and focal cortical malformations. It is therefore important to perform magnetic reso- nance imaging when considering the diagnosis of LGS, as there are reported cases of seizure reduction with focal surgical resection of lesions. Other types of neuroima- ging, including PET and SPECT scans, have not been uniformly helpful and are cur- rently better research than diagnostic tools. The majority of cases of LGS remain cryptogenic despite extensive metabolic evaluation and neuroimaging. Evaluation aimed at maximizing supportive care, such as neuropsychological assessment to identify baseline neurodevelopmental state and aid in appropriate educational placement are also important.

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