Shahram Khoshbin

The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes

OBJECTIVE To develop a standardized nomenclature system for the neuropsychiatric syndromes of systemic lupus erythematosus (NPSLE). METHODS An international, multidisciplinary committee representing rheumatology, neurology, psychiatry, neuropsychology, and hematology developed case definitions, reporting standards, and diagnostic testing recommendations. Before and after the meeting, clinician committee members assigned diagnoses to sets of vignettes randomly generated from a pool of 108 NPSLE patients. To assess whether the nomenclature system improved diagnostic agreement, a consensus index was developed and pre- and postmeeting scores were compared by t-tests. RESULTS Case definitions including diagnostic criteria, important exclusions, and methods of ascertainment were developed for 19 NPSLE syndromes. Recommendations for standard reporting requirements, minimum laboratory evaluation, and imaging techniques were formulated. A short neuropsychological test battery for the diagnosis of cognitive deficits was proposed. In the postmeeting exercise, a statistically significant improvement in diagnostic agreement was observed. CONCLUSION The American College of Rheumatology (ACR) Nomenclature for NPSLE provides case definitions for 19 neuropsychiatric syndromes seen in SLE, with reporting standards and recommendations for laboratory and imaging tests. It is intended to facilitate and enhance clinical research, particularly multicenter studies, and reporting. In clinical settings, consultation with other specialists may be required. It should be useful for didactic purposes but should not be used uncritically or as a substitute for a clinical diagnosis. The complete case definitions are available on the ACR World Wide Web site: http://www.rheumatology .org/ar/ar.html.

The Teratogenicity of Anticonvulsant Drugs

BACKGROUND The frequency of major malformations, growth retardation, and hypoplasia of the midface and fingers, known as the anticonvulsant embryopathy, is increased in infants exposed to anticonvulsant drugs in utero. However, whether the abnormalities are caused by the maternal epilepsy itself or by exposure to anticonvulsant drugs is not known. METHODS We screened 128,049 pregnant women at delivery to identify three groups of infants: those exposed to anticonvulsant drugs, those unexposed to anticonvulsant drugs but with a maternal history of seizures, and those unexposed to anticonvulsant drugs with no maternal history of seizures (control group). The infants were examined systematically for the presence of major malformations, signs of hypoplasia of the midface and fingers, microcephaly, and small body size. RESULTS The combined frequency of anticonvulsant embryopathy was higher in 223 infants exposed to one anticonvulsant drug than in 508 control infants (20.6 percent vs. 8.5 percent; odds ratio, 2.8; 95 percent confidence interval, 1.1 to 9.7). The frequency was also higher in 93 infants exposed to two or more anticonvulsant drugs than in the controls (28.0 percent vs. 8.5 percent; odds ratio, 4.2; 95 percent confidence interval, 1.1 to 5.1). The 98 infants whose mothers had a history of epilepsy but took no anticonvulsant drugs during the pregnancy did not have a higher frequency of those abnormalities than the control infants. CONCLUSIONS A distinctive pattern of physical abnormalities in infants of mothers with epilepsy is associated with the use of anticonvulsant drugs during pregnancy, rather than with epilepsy itself.

Formal art observation training improves medical students' visual diagnostic skills.

BackgroundDespite evidence of inadequate physical examination skills among medical students, teaching these skills has declined. One method of enhancing inspection skills is teaching “visual literacy,” the ability to reason physiology and pathophysiology from careful and unbiased observation.ObjectiveTo improve students’ visual acumen through structured observation of artworks, understanding of fine arts concepts and applying these skills to patient care.DesignProspective, partially randomized pre- vs. post-course evaluation using mixed-methods data analysis.ParticipantsTwenty-four pre-clinical student participants were compared to 34 classmates at a similar stage of training.InterventionTraining the Eye: Improving the Art of Physical Diagnosis consists of eight paired sessions of art observation exercises with didactics that integrate fine arts concepts with physical diagnosis topics and an elective life drawing session.MeasurementsThe frequency of accurate observations on a 1-h visual skills examination was used to evaluate pre- vs. post-course descriptions of patient photographs and art imagery. Content analysis was used to identify thematic categories. All assessments were blinded to study group and pre- vs. post-course evaluation.ResultsFollowing the course, class participants increased their total mean number of observations compared to controls (5.41 ± 0.63 vs. 0.36 ± 0.53, p < 0.0001) and had increased sophistication in their descriptions of artistic and clinical imagery. A ‘dose-response’ was found for those who attended eight or more sessions, compared to participants who attended seven or fewer sessions (6.31 + 0.81 and 2.76 + 1.2, respectively, p = 0.03).ConclusionsThis interdisciplinary course improved participants’ capacity to make accurate observations of art and physical findings.

Multimodality evoked potentials and blink reflex in multiple sclerosis

Thirty patients with definite multiple sclerosis and 50 patients in whom the diagnosis was suspected but not proved (indefinite multiple sclerosis) were studied with visual evoked potentials from each eye, somatosensory evoked potentials from each limb, brainstem auditory evoked potentials from each ear, and the blink reflex. The purpose was to organize a battery of clinically practical tests. The battery found 100% abnormality in the definite patients and 82% in the indefinite patients. Of the individual tests, somatosensory evoked potentials were most often abnormal: 86% in definite patients, 58% in indefinite patients, and 77% in asymptomatic definite patients. Visual evoked potentials were abnormal in 76%, 55%, and 40% of the same three categories. Brainstem auditory evoked potentials and the blink reflex were abnormal less often but contributed to the efficacy of the battery.

LONG-TERM FOLLOWUP OF NEWBORNS WITH MYELODYSPLASIA AND NORMAL URODYNAMIC FINDINGS: : IS FOLLOWUP NECESSARY?

PURPOSE A subset of newborns with myelodysplasia have normal bladder function on urodynamic assessment. We analyzed long-term followup in this population to determine the necessity for subsequent urological surveillance. MATERIALS AND METHODS We retrospectively analyzed the records of 25 of 204 newborns (12%) with myelodysplasia in whom neurourological evaluation was normal after surgical repair of the spinal defect. Initial assessment included complete urodynamic study, renal ultrasound, urinalysis and urine culture. These patients were reevaluated every 3 months until age 3 years, semiannually until age 6 years and yearly thereafter. The longest followup was 18.6 years. RESULTS Of the 25 newborns 22 had myelomeningocele and 3 had meningocele. During a mean followup of 9.1 years urodynamics subsequently showed neurourological deterioration in 8 children (32%). No changes in urodynamics were observed in any patient older than 6 years. All children with neurourological deterioration underwent magnetic resonance imaging, which confirmed a tethered spinal cord that was then surgically corrected. After the untethering procedure 2 patients (25%) regained normal voiding function, whereas in 6 (75%) mild or moderate neurogenic bladder dysfunction persisted. CONCLUSIONS Newborns with myelodysplasia and initially normal urodynamic studies are at risk for neurological deterioration secondary to spinal cord tethering, especially during the first 6 years of life. Close followup of these children is important for the early diagnosis and timely surgical correction of tethered spinal cord, and for the prevention of progressive urinary tract deterioration.

Urodynamic assessment of children with cerebral palsy.

More than a third of the children with cerebral palsy present with dysfunctional voiding symptoms. Clinical evaluation and urodynamic study of cerebral palsy patients were performed to characterize the symptoms, to define the type of neurological deficit and to document its effect on voiding dynamics. We evaluated flow rate, and cystometrographic and external sphincter electromyographic findings in 57 children with cerebral palsy. Upper and lower motor neuron lesions were defined by standard criteria. Of the children 86 per cent had evidence of a pure upper motor neuron injury and 11 per cent manifested electromyographic findings suggestive of incomplete lower motor neuron sphincteric injury. The latter deficit could not be predicted on the basis of clinical neurological findings but it was suggested by a history of neonatal cyanosis. Treatment protocols achieved continence in more than 75 per cent of the children. The voiding dynamics in children with cerebral palsy have not been assessed previously. We have defined the lower urinary tract dysfunction in these patients, and provide a rational and effective plan of management.

MR imaging of paraneoplastic limbic encephalitis

Paraneoplastic limbic encephalitis is a rare disorder that has been previously diagnosed on clinical and pathologic grounds without good radiologic correlation. We present the case of a 42-year-old woman who developed gradually progressive limbic dysfunction 4 years after undergoing mastectomy for breast cancer. Although CT scans were normal, magnetic resonance (MR) imaging showed signal abnormalities in the medial portions of both temporal lobes, the amygdaloid nuclei, and the hypothalamus. An MR-guided temporal lobe biopsy confirmed the presence of encephalitis.

Urinary Undiversion in Myelodysplasia: Criteria for Selection and Predictive Value of Urodynamic Evaluation

Urinary undiversion was done in 15 children with myelodysplasia and ileal conduits. The process of evaluation and the criteria for selection are described. Neurologic and psychologic factors have an important role. Electromyography of the external urethral sphincter is helpful in predicting which children would be continent after undiversion and in managing the neurogenic bladder postoperatively. Over-all, a successful outcome has been achieved in two-thirds of the children chosen for undiversion.

Prevalence of Migraine in Patients With Systemic Lupus Erythematosus

Objective.—To determine the prevalence of migraine in patients with systemic lupus erythematosus (SLE), and to examine the relationships between headache type and other clinical, serologic, and treatment features of the disease.

Evaluation and management of children with sacral agenesis

Sacral agenesis is a rare congenital anomaly of the lower vertebral column which usually produces lower urinary tract dysfunction. Sixteen children with varying degrees of sacral agenesis and urinary symptoms were extensively evaluated. The neurologic lesion varied from no denervation to a complete loss of sacral motor and sensory function. The treatment instituted was individualized and based on specific urodynamic and radiologic findings. Overall, 12 of the 16 children (75%) achieved continence. Clues to the diagnosis including symptoms, physical findings, and the relationship to maternal diabetes are discussed.