Long-term use of methylphenidate in a boy with hypothalamic tumor, drug-resistant epilepsy and ADHD.

•We report the successful treatment of a boy with hypothalamic tumor, gelastic seizures, drug-resistant epilepsy and ADHD•The use of methylphenidate significantly reduced symptoms of ADHD while seizure frequency remained unchanged.

Attention deficit/hyperactivity disorder and interictal epileptiform discharges: it is safe to use methylphenidate?

PURPOSE: This study investigated whether interictal epileptiform discharges (IED) on a baseline routine EEG in children with ADHD was associated with the occurrence of epileptic seizures (Sz) or influenced the use of methylphenidate (MPH) during 2 years follow-up. METHODS: A retrospective chart-review of 517 ADHD children with EEG revealed IED in 39 cases. These patients (IED group) were matched on age and gender with 39 patients without IED (non-IED group). We measured at baseline, 1 year and 2 years Sz occurrence, the use of MPH and antiepileptic drug (AED). RESULTS: At baseline, 12 patients in the IED group had active epilepsy and three of them had Sz during the last year. 36 (92.3%) patients were treated with MPH. Initial positive response to MPH was achieved in 83.3% compared with 89.2% in the non-IED group. At 1 and 2 years follow-up, three patients who also had Sz at baseline and difficult to treat epilepsy, had Sz, without changes in seizure frequency. We found no statistically significant differences between the groups with respect to MPH use at 1 year and at 2 years. Ten patients from IED group, who did not have confirmed epilepsy diagnosis, temporarily used AEDs during the first year of follow-up. CONCLUSION: Despite the occurrence of IED, the use of MPH was safe during 2 years follow-up. IED predict the Sz occurrence in children with previous epilepsy, but does not necessarily suggest an increased seizure risk. A caution is warranted in order not to overestimate the significance of temporarily occurrence of IED.

Epilepsy in a large cohort of children diagnosed with attention deficit/hyperactivity disorders (ADHD).

PURPOSE: The knowledge about possible relationships between ADHD and epilepsy is largely based on small samples of ADHD patients and on cohorts with epilepsy. There is insufficient information about the clinical characteristics of epilepsy among children diagnosed with ADHD. The aim of this study was to investigate the prevalence and characteristics of epilepsy in a large, unselected cohort of children with ADHD. METHODS: We conducted a retrospective chart-review of children with ADHD who were evaluated in our clinic between the years 2000 and 2005. We compared age, sex, disorders of psychological development, cognitive level, pharmacological treatment for ADHD, initial response to treatment and ADHD subtype with and without epilepsy. In addition, we compared our data with data from a Norwegian study in a large general pediatric population. RESULTS: Of 607 children with ADHD (age 6-14 years; 82.4% males); 14 (2.3%) had a history of epilepsy, and 13 of these had active epilepsy. This is a higher occurrence than expected in the general pediatric population (0.5%). The majority of our patients had mild (an easily treated) epilepsy and they were more likely to be seizure free (79%) compared to the patients with epilepsy in general pediatric population. The ADHD patients with and without epilepsy did not differ regarding age, gender, disorders of psychological development, IQ level<85 or ADHD subtype. The patients had been diagnosed with epilepsy on average 1.8 years before the ADHD assessment. All patients with epilepsy were treated with methylphenidate (MPH), and initial response to MPH was achieved in 85.7%. CONCLUSION: The epilepsy diagnosis preceded the ADHD diagnosis, and was found in a significantly higher rate than would be expected in the general pediatric population. The majority of patients had mild epilepsy and ADHD-Combined Inattentive/Hyperactive-Impulsive Subtype. All cases with epilepsy and ADHD were treated with MPH, with initial response achieved in 86%.

[Assessment of ADHD with EEG]. / EEG-utredning ved AD/HD.

BACKGROUND: Many children with ADHD develop epilepsy, and approximately 20% of children with epilepsy also have ADHD. In this article we discuss the use of EEG in connection with ADHD in children, with emphasis on the diagnosis of comorbid epilepsy. METHOD: The article is based on a literature search in PubMed, personal literature archives and the authors' own experience with the use of EEG, treatment of epilepsy and the diagnosis and treatment of ADHD in children and adolescents. RESULTS: A moderately elevated prevalence of epileptiform EEG activity is described in children with ADHD without epilepsy compared with healthy children, during both wakefulness and sleep. Selected material and a lack of controlled blinded studies probably explain much of this difference. The significance of epileptiform EEG activity in children with ADHD without seizures is uncertain. Evaluating the extent to which EEG findings may explain the symptoms and whether anti-epileptic drugs should be tried is a specialist task. In many studies, spectral analysis of the frequency content of the EEG (QEEG) has shown higher slow theta activity and a higher theta/beta ratio in children with ADHD. INTERPRETATION: Seizure symptoms, disturbed sleep quality, significant changes in behaviour or regression of cognitive ability in children with ADHD should lead to paediatric neurological assessment with EEG and possibly a 24-hour EEG. In our view, the QEEG variables are artifact-prone and biologically unspecific. We therefore do not recommend the use of QEEG as a stand-alone diagnostic marker.

[EEG--when and how?]. / EEG--når og hvordan?

BACKGROUND: Electroencephalography (EEG) is used for studying functional disorders of the brain. We provide an overview of the areas where EEG is most strongly indicated and the clinical benefit of the examination. METHOD: This article is based on articles from personal literature archives and literature searches in PubMed, textbook on methods in clinical neurophysiology, the work of one of the authors on a new European standard for EEG and the authors' clinical experience. RESULTS: EEG is primarily indicated for assessing suspected epileptic seizures and encephalopathies. Standard EEG, sleep-deprived EEG and long-term EEG examinations are used in diagnosing epilepsy. EEG will identify epileptiform activity in about 90% of patients with epilepsy. Epileptiform EEG activity in these patients will provide support for the diagnosis of epilepsy, while the absence of such activity will not exclude the diagnosis. EEG is also important for classifying the type of epileptic syndrome. In cases of encephalopathy, pathology will take the form of an increase in slow activity on the EEG and will support the diagnosis, but as a rule the EEG finding is non-specific. Findings in some cases may point to the underlying aetiology. INTERPRETATION: Electroencephalograms must be interpreted by an experienced clinical neurophysiologist or neurologist with specialist expertise in EEG interpretation, and must always be evaluated in the light of the clinical picture.

Migraine is frequent in patients with systemic lupus erythematosus: a case-control study.

INTRODUCTION: The objective of this study was to compare the prevalence of primary headaches in systemic lupus erythematosus (SLE) versus healthy subjects, and to determine whether headaches in SLE are associated with MRI- or cerebrospinal fluid (CSF) abnormalities. PATIENTS AND METHODS: The case-control study included MRI- and CSF investigations. Headache was classified according to the International Classification of Headache Disorders. Depression and fatigue were measured with Beck Depression Inventory (BDI) and Fatigue Severity Scale (FSS) respectively. RESULTS: Twenty-four out of 67 SLE patients and 13 out of 67 age- and gender matched healthy subjects had migraine (36% vs 19%, P = 0.03). Nine (13%) SLE patients had migraine with aura vs 4 (6%) in healthy subjects, P = 0.14. The prevalence of tension type headache was equal (60% in patients vs 58% in controls). There was no association between migraine and SLE disease activity, biochemical or immunological markers, cerebral white matter hyperintensities, interleukin-6 in CSF, impairment of the blood-brain barrier, or intrathecal immunoglobulin production. SLE patients had higher BDI- and FSS scores compared with healthy control subjects, and SLE patients with migraine had higher BDI scores than lupus patients without migraine. CONCLUSIONS: Migraine is more prevalent in SLE patients, associated with depression like in the general population, but not associated with disease activity or abnormalities detected on cerebral MRI, in CSF, or any SLE characteristics except from SLE photosensitivity. The inclusion of the migraine item in SLE disease activity instruments remains questionable.

Epileptiform abnormalities in children diagnosed with attention deficit/hyperactivity disorder.

The frequency of epileptiform abnormalities (EAs) and correlates of EAs in children with attention deficit/hyperactivity disorder (ADHD) was investigated in a retrospective study. The total sample consisted of 607 children with ADHD from the only center diagnosing and treating ADHD in the region. EEGs were obtained in 517 cases, and EAs were found in 39 (7.5%) children. Of these, 21(53.9%) showed generalized EAs, 16 (41%) had focal EAs, and 2 (5.1%) had both. Rolandic spikes were observed in 9 cases (1.7% of the total group). A previous history of epileptic seizures was reported in 14 (2.5%) children, and was more common in those with EAs than in those without. The group with ADHD and EAs had a larger proportion of girls and the ADHD inattentive subtype, independent of a history of epilepsy. Future research should focus on the clinical consequences of EAs in children with ADHD.

Peripheral neuropathy in primary sjogren syndrome: a population-based study.

BACKGROUND: Neurological manifestations appear to be frequently involved in patients with primary Sjögren syndrome (PSS). OBJECTIVE: To investigate the involvement of the peripheral nervous system, including small-diameter nerve fibers, in an unselected cohort of patients who fulfilled the new international criteria for PSS. DESIGN: Cross-sectional study. SETTING: Stavanger University Hospital. Patients Sixty-two patients with PSS (mean +/- SD age, 57.1 +/- 14.6 years). INTERVENTIONS: Clinical neurologic examinations, conventional nerve conduction studies, and skin punch biopsies. MAIN OUTCOME MEASURES: Signs of large-diameter and small-diameter peripheral nerve fiber neuropathy as determined by clinical examination, nerve conduction studies, and densities of intraepidermal nerve fibers in skin punch biopsy specimens. RESULTS: Seventeen patients (27%) were diagnosed as having neuropathy after clinical examination. The results of nerve conduction studies were abnormal in 34 patients (55%): 19 patients (31%) had motor neuropathy, 8 (13%) had sensory neuropathy, and 7 (11%) had sensorimotor neuropathy. Two patients had intraepidermal nerve fiber densities less than 3.4 fibers per millimeter, fitting the morphologic criteria for small-diameter nerve fiber neuropathy. CONCLUSIONS: Peripheral neuropathy occurs in a large proportion of patients with PSS, in most cases as a subclinical demyelinating neuropathy. Small-diameter nerve fiber neuropathy is not a frequent finding in these patients.

Small-diameter nerve fiber neuropathy in systemic lupus erythematosus.

BACKGROUND: Systemic lupus erythematosus (SLE) is an inflammatory, autoimmune, multiorgan disease often involving the central and peripheral nervous systems. OBJECTIVE: To determine whether there is a selective small-diameter nerve fiber neuropathy in patients with SLE. DESIGN: Cross-sectional study. SETTING: Stavanger University Hospital, Stavanger, Norway. Patients Sixty patients with SLE, aged 43.2 +/- 13.5 years (mean +/- SD). INTERVENTIONS: Skin biopsies, nerve conduction studies, and clinical neurologic examinations. MAIN OUTCOME MEASURES: Density of intraepidermal small-diameter nerve fibers in skin biopsy specimens and large-diameter nerve fiber function as determined by nerve conduction studies and clinical examinations. RESULTS: The mean density of intraepidermal small-diameter nerve fibers in patients with SLE was 7.5 +/- 3.8/mm. Eight patients (13%) had densities below reference values, consistent with small-diameter nerve fiber neuropathy, and results of nerve conduction studies were normal in 6 of them. Eleven patients (18%) had abnormal results of nerve conduction studies, reflecting large-diameter nerve fiber neuropathy, and 4 patients (7%) were classified by an experienced neurologist as having polyneuropathy after the clinical examination. CONCLUSIONS: An abnormal reduction in intraepidermal small-diameter nerve fiber densities is evident in some patients despite normal function of their larger nerve fibers. This adds further support to the theory that a pure small-diameter nerve fiber neuropathy may occur in SLE.