Paula M. Brna

Manganese Toxicity in a Child With Iron Deficiency and Polycythemia

A previously healthy 5-year-old girl presented with pica, emotional lability, and marked gait abnormalities. She had concurrent severe iron deficiency and polycythemia. Her magnetic resonance imaging (MRI) scan showed increased signal in the basal ganglia on T1-weighted images consistent with manganese neurotoxicity. Manganism was subsequently confirmed as her blood manganese levels were extremely elevated. Chelation therapy resulted in improvement in her mobility but she continues to have significant gait impairment. An epidemiological investigation identified well water as the potential source of manganese exposure for our patient, but to date, we have been unable to identify the nature of her neurotoxic susceptibility.

Epilepsy and activity—A population‐based study

Purpose:  To compare the activity profiles of a nationally representative sample of individuals with epilepsy compared to the general population.

Alice in Wonderland and Other Migraine Associated Phenomena—Evolution Over 30 Years After Headache Diagnosis

BACKGROUND The International Classification of Headache Disorders-III beta includes a number of episodic syndromes associated with migraine. Those who treat pediatric headaches are aware of a number of other phenomena (such as the Alice in Wonderland syndrome) which are thought to occur as precursors of migraine. There is no available data on the course of these phenomena over the decades following childhood headache diagnosis. METHODS Patients who were observed by one of the authors in 1983 were contacted by telephone in 1993, 2003, and 2013. Details were gathered regarding the presence and characteristics of ongoing headaches and about the presence of sleepwalking, motion sickness, and distortions of either time or space perceptions. RESULTS Twenty-eight patients were monitored in 1993, 2003, and 2013. Ongoing headaches were reported by 71%. Sleepwalking was only present in one patient in 2013. More than a third still complained of motion sickness, and more than one quarter still experienced distortions of time. Distortions of space were still reported by nearly 20%. Reporting any of these phenomena was not consistent over time, with some patients reporting distortions for the first time in adulthood. There was no clear correlation with migraine, and patients with tension-type headaches also reported the phenomena. CONCLUSIONS Motion sickness and distortions of both space and time persist into the fifth decade for many patients initially observed with headaches in childhood. The correlation with migraine is less clear than previously thought.

The diagnostic utility of intracranial EEG monitoring for epilepsy surgery in children

There are limited data on the indications for the use of chronic invasive electroencephalography (EEG) monitoring (IEM) for pediatric epilepsy surgery.

The Prognosis of Pediatric Headaches—A 30-Year Follow-up Study

BACKGROUND Although headaches in childhood are common, there are few data available on their long-term prognosis. We have monitored a group of patients since diagnosis in 1983. METHODS Patients who were part of the 20-year follow-up study in 2003 were contacted, and data were collected using a standardized telephone interview. Details of headache characteristics and identified precipitants and alleviating factors were gathered. The most effective means of controlling the headaches were also recorded. RESULTS Follow-up was achieved for 28 of 60 patients (47%). Over the 30 years since diagnosis, eight patients (29%) reported a complete resolution of headaches, including three whose headaches resolved between the 20- and 30-year follow-up studies. The type of headache varied over the 30-year time interval with only three patients maintaining the same headache type at all four time periods of 1983, 1993, 2003, and 2013. Only one patient used prescription medication as the primary method for controlling headaches. The most commonly used intervention was nonprescription analgesia, self-relaxation and/or hypnosis, and precipitant avoidance. CONCLUSIONS Headaches persist in approximately 70% of children 30 years after diagnosis. Encouraging children to manage their headaches with simple analgesia and precipitant avoidance appears to have long-term benefits.

Which characteristics of children with a febrile seizure are associated with subsequent physician visits

Objective. To reanalyze an existing data set to determine which children with an initial febrile seizure have excessive subsequent physician visits. Methods. Individual data from a regional cohort of 75 children with a first febrile seizure and 150 febrile and 150 afebrile control subjects were linked to a comprehensive physician services database. The impact of study variables on subsequent physician utilization over the following 6 years was modeled using analysis of variance. Results. Children with a known family history of febrile seizures at the time of study entry had 24% fewer physician visits. Control children with a known family history of afebrile seizures had 7% fewer visits than those with negative family histories. Children with an initial febrile seizure had 45% more physician visits when they knew of a relative with afebrile seizures than those with negative family histories. Conclusions. Knowing the family history of seizures is probably a marker of reduced physician utilization. At the time of an initial febrile seizure, knowing the family history of afebrile seizures defines a group of patients with excessive subsequent physician visits.

What Do Patients and Families Want From a Child Neurology Consultation

Understanding what patients and their parents want is essential to plan appropriate patient-centered care. Questionnaires were distributed to 500 consecutive children and parents seen for their first pediatric neurology consultation. Both patients and their families answered questions about their expectations of the consultation, their level of worry, and the Penn State Worry Questionnaire. The 5 most important issues for the parents were to get information, to work with the doctor to manage the problem, to have questions answered, to find out what was wrong, and to discuss the impact on the child’s life. The children had very similar priorities. The 5 least important concerns for parents were to get a prescription, blood tests, to talk to others with similar problems, to get a radiograph/computed tomography/magnetic resonance imaging (MRI) and to be told nothing is wrong. The pediatric neurologists did well in anticipating these priorities but had more difficulty appreciating parent and patient level of worry.

Perceived need for restrictions on activity for children with epilepsy

BACKGROUND Children and youth with epilepsy have long been subjected to excessive restrictions on extracurricular activities due to concerns over risk of injury. Over time physicians and medical regulatory associations have liberalized the advice given for people with epilepsy to promote independence, self-esteem and general health benefits of physical activity. Current evidence suggests that few restrictions are needed for children with epilepsy beyond water-related precautions and avoidance of very high-risk activities. However, more stringent restrictions on daily activities may be imposed by caregivers. This study was aimed at exploring current perceptions of parents regarding restrictions on activity for children with epilepsy and the childs perspective on restrictions related to the diagnosis. METHODS A self-administered questionnaire was offered to a sample of parent-child dyads of children/youth with epilepsy attending summer camp for children with epilepsy age 8-18years. A 10-item validated HARCES Parent Scale of Childhood Epilepsy was completed by the parent/guardian and a modified-HARCES completed by the child. The primary objective was to assess the degree of restrictions placed on children with epilepsy from the perspective of child and parent assessed independently. Agreement of perceived restrictions between parent-child dyads was also determined. RESULTS 21 parent/guardian-child pairs were recruited with mean age of children/youth 12.7years (range 9-16years). Total HARCES scores for parents and guardians ranged from 11-26 (x=16.5; SD 4.9) while total scores for children with epilepsy similarly ranged from 10-25 (x=15.2; SD 4.9). There were no differences in total parent scores when analyzed by childs age (<13 or >13years), gender, age of seizure onset, seizure frequency or seizure type. Total HARCES scores showed no agreement between parent and child pairs with correlation of 0.2798 (95% CI -0.173-0.635). CONCLUSIONS Children and youth with epilepsy often face activity restrictions based on fear of perceived risk of injury. This small sample shows evidence that even more permissive parents and his/her children still feel limited by such restrictions. Parents and children do not perceive these restrictions in the same way despite similar education by physicians highlighting an important secondary role of epilepsy camps in targeting misperceptions and educating families on appropriate precautions.