Mary Coleman

Infantile spasms associated with 5‐hydroxytryptophan administration in patients with Down's syndrome

ANIMAL EXPERIMENTS and anticonvulsant drug studies have suggested that variations in the levels of serotonin (5-hydroxytryptamine, 5HT) , a biogenic amine present in midline nuclei in the human brainstem, may be relevant to seizure phenomena. Yet a direct relationship of 5-HT to a clinical seizure disorder has not been demonstrated. Patients with Down’s syndrome are receiving the precursor of 5-HT, 5-hydroxytryptophan (5-HTP), in our clinic in a study which began after low levels of platelet 5-HT were demonstrated in these patients.l.2 Some patients received the L form of 5-HTP, the optical isomer form of the amino acid normally used by the body. Other patients were given a D,L racemic mixture of 5-HTP. In the infant mongol, depression of platelet 5-HT closely correlates with the amount of hypotonia, buccolingual dyskinesia, and decreased activity level of each patient. Platelet 5-HT comes from the gastrointestinal tract, not from cerebral sources; this hypotonia-platelet level correlation can be understood by the Pletscher theory that the platelet may be a partial functional model of the serotonergic neuron.3 Oral administration of 5-HTP to patients with Down’s syndrome results in improved muscular tone4 and activity level as well as in suppression of the buccolingual dyskinesias. However, in 15% of the 60 patients receiving the amino acid on a continuous basis, 5-HTP also appears to induce a seizure disorder resembling the infantile spasm syndrome. This report discusses the factors that may have contributed to this onset of seizures and reviews the subject of serotonin and seizures. Case histories of the two most severely affected patients are presented in detail.

Preliminary remarks on the L‐dopa therapy of dystonia

DYSTONIA is a rare symptom but may be one of the most ancient; the German literature contains a paper on severe torticollis or dystonic twisting of the neck-in dinosaurs.1 In spite of its antiquity, a satisfactory treatment for dystonia in all patients has not yet been developed, although there are the major contributions of neurosurgery described earlier in these proceedings. Our study of the biochemical abnormalities of inferior intelligence in retarded patients has stimuIated further interest in possible biochemical correlations of superior intelligence. Therefore, we noted the report that persons with at least one form of dystonia tend to have superior intelligence.2 We had deduced that some of the dystonias probably are metabolic or biochemical diseases because of the controversy that exists among pathologists. Dr. Zeman gave us an excellent description of the controversies surrounding the pathology of dystonia and suggested that the pathology is nonspecific. The hallmark of a metabolic disease often is heated discussions between pathologists. In 1964, Hirschmann and Mayer administered L-dopa to a patient with retrocollis or torticollis and reported limited improvement.3 This was confirmed in 1966 by Pazzagli and Amaducci in Italy.4 Early in 1969, my group began administering L-dopa to patients with various forms of torsion dystonia or dystonia musculorum deformans.5 We had considered this treatment for some time because of the effectiveness of L-dopa in the Parkinson syndromes, regardless of etiology. Neurosurgical therapy in Parkinson’s disease was also effective in several forms

Serum dopamine-β-hydroxylase levels in Down's syndrome

Serum dopamine‐β‐hydroxylase (DBH) and serum immunoreactive (IR) DBH levels were measured in patients with Downs syndrome. Serum DBH activity was markedly reduced in Downs syndrome patients as compared with age matched, normal controls or non‐mongoloid, disturbed children. Serum IR‐DBH levels were also markedly reduced in Downs syndrome. The possible factors responsible for the observed reduction in serum DBH levels (active and inactive enzyme levels) in Downs syndrome were investigated.

A familial study in serum dopamine‐β‐hydroxylase levels in torsion dystonia

Dopamine-β-hydroxylase activity and immunoreactive dopamine-β-hydroxylase levels were measured in the serum of patients with the autosomal recessive and autosomal dominant forms of torsion dystonia, as well as in unaffected members of their families. The enzyme levels do not differentiate between the two forms of the disease. The elevated serum dopamine-β-hydroxylase levels in some patients with torsion dystonia reflect genetic influences rather than clinical symptomatology. Patients with torsion dystonia could not be classified with the autosomal dominant form or autosomal recessive form according to the serum dopamine-β-hydroxylase levels.

Education Research: Neurology training reassessed The 2011 American Academy of Neurology Resident Survey results

Objective: To assess the strengths and weaknesses of neurology resident education using survey methodology. Methods: A 27-question survey was sent to all neurology residents completing residency training in the United States in 2011. Results: Of eligible respondents, 49.8% of residents returned the survey. Most residents believed previously instituted duty hour restrictions had a positive impact on resident quality of life without impacting patient care. Most residents rated their faculty and clinical didactics favorably. However, many residents reported suboptimal preparation in basic neuroscience and practice management issues. Most residents (71%) noted that the Residency In-service Training Examination (RITE) assisted in self-study. A minority of residents (14%) reported that the RITE scores were used for reasons other than self-study. The vast majority (86%) of residents will enter fellowship training following residency and were satisfied with the fellowship offers they received. Conclusions: Graduating residents had largely favorable neurology training experiences. Several common deficiencies include education in basic neuroscience and clinical practice management. Importantly, prior changes to duty hours did not negatively affect the resident perception of neurology residency training.

Results of the American Academy of Neurology resident survey.

Background: To assess the effect of neurology residency education as trainees advance into independent practice, the American Academy of Neurology (AAN) elected to survey all graduating neurology residents at time of graduation and in 3-year cycles thereafter. Methods: A 22-question survey was sent to all neurology residents completing residency training in the United States in 2007. Results: Of 523 eligible residents, 285 (54.5%) responded. Of these, 92% reported good to excellent quality teaching of basic neurology from their faculty; however, 47% noted less than ideal training in basic neuroscience. Two-thirds indicated that the Residency In-service Training Examination was used only as a self-assessment tool, but reports of misuse were made by some residents. After residency, 78% entered fellowships (with 61% choosing a fellowship based on interactions with a mentor at their institution), whereas 20% entered practice directly. After adjustment for the proportion of residents who worked before the duty hour rules were implemented and after their implementation, more than half reported improvement in quality of life (87%), education (60%), and patient care (62%). The majority of international medical graduates reported wanting to stay in the United States to practice rather than return to their country of residence. Conclusions: Neurology residents are generally satisfied with training, and most entered a fellowship. Duty hour implementation may have improved resident quality of life, but reciprocal concerns were raised about impact on patient care and education. Despite the majority of international trainees wishing to stay in the United States, stricter immigration laws may limit their entry into the future neurology workforce.

International electives in neurology training: A survey of US and Canadian program directors

Objective: To ascertain the current status of global health training and humanitarian relief opportunities in US and Canadian postgraduate neurology programs. Background: There is a growing interest among North American trainees to pursue medical electives in low- and middle-income countries. Such training opportunities provide many educational and humanitarian benefits but also pose several challenges related to organization, human resources, funding, and trainee and patient safety. The current support and engagement of neurology postgraduate training programs for trainees to pursue international rotations is unknown. Methods: A survey was distributed to all program directors in the United States and Canada (December 2012–February 2013) through the American Academy of Neurology to assess the training opportunities, institutional partnerships, and support available for international neurology electives. Results: Approximately half of responding programs (53%) allow residents to pursue global health–related electives, and 11% reported that at least 1 trainee participated in humanitarian relief during training (survey response rate 61%, 143/234 program directors). Canadian programs were more likely to allow residents to pursue international electives than US programs (10/11, 91% vs 65/129, 50%, p = 0.023). The number of trainees participating in international electives was low: 0%–9% of residents (55% of programs) and 10%–19% of residents (21% of programs). Lack of funding was the most commonly cited reason for residents not participating in global health electives. If funding was available, 93% of program directors stated there would be time for residents to participate. Most program directors (75%) were interested in further information on global health electives. Conclusions: In spite of high perceived interest, only half of US neurology training programs include international electives, mostly due to a reported lack of funding. By contrast, the majority of Canadian programs that responded allow international electives, likely due to clearer guidelines from the Royal College of Physicians and Surgeons of Canada compared to the Accreditation Council of Graduate Medical Education. However, the number of both Canadian and US neurology trainees venturing abroad remains a minority. Most program directors are interested in learning more information related to global health electives for neurology residents.

Status of neurology medical school education Results of 2005 and 2012 clerkship director survey

Objective: To survey all US medical school clerkship directors (CDs) in neurology and to compare results from a similar survey in 2005. Methods: A survey was developed by a work group of the American Academy of Neurology Undergraduate Education Subcommittee, and sent to all neurology CDs listed in the American Academy of Neurology database. Comparisons were made to a similar 2005 survey. Results: Survey response rate was 73%. Neurology was required in 93% of responding schools. Duration of clerkships was 4 weeks in 74% and 3 weeks in 11%. Clerkships were taken in the third year in 56%, third or fourth year in 19%, and fourth year in 12%. Clerkship duration in 2012 was slightly shorter than in 2005 (fewer clerkships of ≥4 weeks, p = 0.125), but more clerkships have moved into the third year (fewer neurology clerkships during the fourth year, p = 0.051). Simulation training in lumbar punctures was available at 44% of schools, but only 2% of students attempted lumbar punctures on patients. CDs averaged 20% protected time, but reported that they needed at least 32%. Secretarial full-time equivalent was 0.50 or less in 71% of clerkships. Eighty-five percent of CDs were “very satisfied” or “somewhat satisfied,” but more than half experienced “burnout” and 35% had considered relinquishing their role. Conclusion: Trends in neurology undergraduate education since 2005 include shorter clerkships, migration into the third year, and increasing use of technology. CDs are generally satisfied, but report stressors, including inadequate protected time and departmental support.

Practice and payment trends in neurology in 2012

SummaryThis article describes practice and payment trends among neurologists. Data from the 2012 Practice and Payment Trends survey were compared to results from the 2010 Medical Economics survey. Both surveys were sent to a random sample of 1,000 US practicing neurologists, with a response rate of 32%. Since 2010, there has been an 8% increase in the percent of neurologists working in academic medical centers. Nearly half of neurologists reported working for a hospital-affiliated practice. Wait times have increased 40% for a new patient visit. Only 19% of neurologists indicated procedures as the primary focus of their practice. New delivery models have not yet gained traction with neurologists but the majority (>80%) of neurologists currently use electronic health records in their practice.

Survey of current neurohospitalist practice

Summary Neurohospitalists represent a new approach to inpatient neurologic care. In order to characterize this practice, we surveyed both a general neurology sample as well as a sample of pertinent American Academy of Neurology sections. Of the section sample, 42% defined themselves as neurohospitalists, compared to 16% of the general sample. The majority of neurohospitalists are in an academic setting and share call responsibilities with non-neurohospitalists. Many are concerned about the possibility of burnout in their current practice setting. This representative sample of neurohospitalists reveals a diverse group facing a number of unanswered questions and challenges, including concerns for burnout, ideal practice setting, and defining the core curriculum for a neurohospitalist.