Incidence of acquired demyelination of the CNS in Canadian children.

BACKGROUND: The incidence of acquired demyelination of the CNS (acquired demyelinating syndromes [ADS]) in children is unknown. It is important that physicians recognize the features of ADS to facilitate care and to appreciate the future risk of multiple sclerosis (MS). OBJECTIVE: To determine the incidence, clinical features, familial autoimmune history, and acute management of Canadian children with ADS. METHODS: Incidence and case-specific data were obtained through the Canadian Pediatric Surveillance Program from April 1, 2004, to March 31, 2007. Before study initiation, a survey was sent to all pediatric health care providers to determine awareness of MS as a potential outcome of ADS in children. RESULTS: Two hundred nineteen children with ADS (mean age 10.5 years, range 0.66-18.0 years; female to male ratio 1.09:1) were reported. The most common presentations were optic neuritis (ON; n = 51, 23%), acute disseminated encephalomyelitis (ADEM; n = 49, 22%), and transverse myelitis (TM; n = 48, 22%). Children with ADEM were more likely to be younger than 10 years, whereas children with monolesional ADS (ON, TM, other) were more likely to be older than 10 years (p < 0.001). There were 73 incident cases per year, leading to an annual incidence of 0.9 per 100,000 Canadian children. A family history of MS was reported in 8%. Before study initiation, 65% of physicians indicated that they considered MS as a possible outcome of ADS in children. This increased to 74% in year 1, 81% in year 2, and 87% in year 3. CONCLUSION: The incidence of pediatric acquired demyelinating syndromes (ADS) is 0.9 per 100,000 Canadian children. ADS presentations are influenced by age.

Electroclinical features of absence seizures in childhood absence epilepsy.

OBJECTIVE: To accurately define the electroclinical features of absence seizures in children with newly diagnosed, untreated childhood absence epilepsy (CAE). METHODS: The authors searched an EEG database for absence seizures in normal children with new onset untreated absence epilepsy. Seventy consecutive children were classified into IGE syndromes. The clinical and EEG features of the seizures in the children with CAE were analyzed using video-EEG recordings. RESULTS: The authors analyzed 339 absence seizures in 47 children with CAE. The average seizure duration was 9.4 seconds and clinical features consisted of arrest of activity, loss of awareness, staring, and 3-Hz eyelid movements, but there was individual variation. Ictal EEG predominantly showed regular 3-Hz generalized spike and wave (GSW) with one or two spikes per wave; however, disorganization of discharges was common and three or more spikes per wave occurred rarely. Postictal slowing was frequent. Interictal abnormalities included fragments of GSW, posterior bilateral delta activity, and focal discharges. Although all 47 children met the current criteria for CAE, only 5 fulfilled the recently proposed criteria for CAE. CONCLUSION: The heterogeneous nature of each clinical and EEG feature of untreated absence seizures is of critical importance when determining criteria for childhood absence epilepsy.

Spasms in children with definite and probable mitochondrial disease.

The diagnosis of mitochondrial encephalomyopathies is complex and a system for classification of the diagnosis as definite, probable, and possible has been proposed. The objective of this study was to explore the spectrum of epileptic disorders associated with probable and definite mitochondrial disease in children using this classification system. The patient population with mitochondrial disease and epilepsy was selected from a tertiary care children's hospital. Interictal electroencephalograms and video-EEG recordings were used to characterize seizure types. Ten children fulfilled the criteria for probable or definite mitochondrial disease and had epilepsy. Four had siblings with a similar clinical phenotype. Spasms were the most common seizure type and were the initial seizure type in seven patients and two siblings. Four patients had only partial seizures, with or without generalization, and one patient had seizures that were difficult to classify. Blood lactate concentrations were elevated consistently in patients with partial seizures alone but were occasionally normal in children with spasms. Spasms were the most common presenting seizure type in children with probable and definite mitochondrial disease.

Postvaricella angiopathy: report of a case with pathologic correlation.

Varicella is a common childhood illness, and central nervous system complications occur frequently. Delayed angiopathy has been described, although there are few reports of clinicopathologic correlation. A previously well 4-year-old male is presented. He suffered varicella 2 months before presentation with extensive right middle cerebral artery (MCA) territory infarction. Cerebral angiography demonstrated an isolated 89% stenosis of the right proximal MCA. He developed cerebral edema refractory to medical treatment and progressed to transtentorial herniation. Right frontal temporoparietal craniotomies were performed with evacuation of infarcted brain tissue. Pathologic studies revealed small vessel vasculitis with lymphocytic infiltration of the vessel wall. Areas of demyelination were present within the white matter. Polymerase chain reaction for varicella was negative on brain tissue. Postvaricella angiopathy, although an uncommon complication, may affect both small and large blood vessels, with catastrophic results.

Early improvement during manual-guided cognitive and dynamic psychotherapies predicts 16-week remission status.

This study examined the extent to which improvement from baseline to weeks 2, 3, and 4 on the Beck Depression Inventory and Beck Anxiety Inventory predict week 16 clinical remission for patients with major depressive disorder, generalized anxiety disorder, and/or obsessive-compulsive or avoidant personality disorders who were receiving manual-based psychotherapies. Logistic regression and receiver-operator characteristic analyses revealed relatively accurate identification of remitters and nonremitters based on improvement from baseline to sessions 2 to 4 in both original and cross-validation samples. Predictive success did not vary as a function of diagnosis, treatment type (cognitive or dynamic), or treatment status (short-term or long-term). The clinical implications of the results are discussed.

Supportive techniques: are they found in different therapies?

Therapists of different persuasions use various techniques. Although many of these techniques are specific to their theory of treatment, others are practiced in common among different forms of psychotherapy. Many of these common techniques have been previously described, but supportive techniques have been largely ignored. The authors distinguish between the use of supportive techniques and the therapeutic alliance. Using Luborsky's definition of supportive techniques, they examine the empirical literature on the use of these supportive techniques in various therapies. They conclude that supportive techniques are often used in different forms of psychotherapy or counseling.

Therapeutic alliance as a predictor of outcome and retention in the National Institute on Drug Abuse Collaborative Cocaine Treatment Study.

The authors examined the relation between therapeutic alliance, retention, and outcome for 308 cocaine-dependent outpatients participating in the National Institute on Drug Abuse Collaborative Cocaine Treatment Study. High levels of alliance were observed in supportive-expressive therapy (SE), cognitive therapy (CT), and individual drug counseling (IDC), and alliance levels increased slightly but significantly from Session 2 to Session 5 in all groups. In contrast to other studies, alliance was not a significant predictor of drug outcome. However, alliance did predict patient retention differentially across the 3 treatments. In SE and IDC, either higher levels of alliance were associated with increased retention or no relationship between alliance and retention was found, depending on the time alliance was measured. In CT, higher levels of alliance were associated with decreased retention.

Factors associated with behavioral and cognitive abnormalities in children receiving topiramate.

The objective of this study was to examine the factors associated with the occurrence of behavioral and cognitive abnormalities in children treated with topiramate. A retrospective chart review of patients up to 18 years of age who had been treated with topiramate at a tertiary epilepsy center was performed. Behavioral or cognitive abnormalities were observed in 11 (14.6%) of 75 children between 2 weeks and 4 months after initiation of therapy. The mean dosage (4.6 mg/kg daily) at which these abnormalities were observed was similar to the mean final dose (5.8 mg/kg daily) in children without abnormalities. The mean rate of dosage increase was 0.72 mg/kg weekly and 0.7 mg/kg weekly in those with and without abnormalities, respectively. Five of the 11 children with behavioral or cognitive abnormalities had a previous history of behavioral or cognitive abnormalities, but only nine of the 64 children without abnormalities had a previous history of behavioral or cognitive abnormalities (P = 0.03). Lamotrigine was used concurrently in four of the 11 children with behavioral or cognitive abnormalities but in only seven of the 64 children without abnormalities (P = 0.05). Behavioral and cognitive abnormalities in children treated with topiramate do not appear to be related to the rate of dosage increase. A previous history of behavioral problems and the concurrent use of lamotrigine may be predisposing factors.

Alliance predicts patients' outcome beyond in-treatment change in symptoms.

The authors examined the relations among therapeutic alliance, outcome, and early-in-treatment symptomatic improvement in a group of 86 patients with generalized anxiety disorders, chronic depression, or avoidant or obsessive-compulsive personality disorder who received supportive-expressive dynamic psychotherapy. Although alliance at Sessions 5 and 10, but not at Session 2, was associated with prior change in depression, alliance at all sessions significantly predicted subsequent change in depression when prior change in depression was partialed out. The results are discussed in terms of the causal role of the alliance in therapeutic outcome.

Alliance and technique in short-term dynamic therapy.

We review existing studies on the extent to which the alliance and techniques predict the outcome of short-term dynamic psychotherapy (STDP). Although the alliance has been found to relate to outcome in diverse psychotherapies, evidence for its role in STDP is more inconsistent. Studies of technique factors have provided support for the relation of competence with exploratory interventions to outcome. Less support for the role of specific transference interpretations was found. Little empirical support exists for the notion that the technical factors are most potent in the context of a positive alliance, although adequate studies of this interaction are scarce. Similarly, adequate studies that attempt to tease apart the extent to which technical factors influence the alliance, and vice versa, are also rare. Despite the methodological limitations of research in this area, the fact that there are some consistent findings relating techniques to outcome suggests that nonspecific relationship factors do not by themselves account for the changes found over the course of STDP. Moreover, such findings encourage additional efficacy and process research on STDP. Some suggestions for future research are presented.

Melatonin treatment of non-epileptic myoclonus in children.

Oral melatonin (MLT) has been used by our Vancouver research group in the treatment of paediatric sleep disorders since 1991; slightly over 200 children, mainly with multiple disabilities, who frequently had seizures, have been treated. Three children with markedly delayed sleep onset due to recurring myoclonus were also referred for MLT treatment: two had non-epileptic, and one had epileptic and non-epileptic myoclonus. Low doses of oral MLT (3 to 5 mg) unexpectedly abolished their myoclonus and allowed them to sleep. There were no adverse effects. It appears that certain types of myoclonus, which might be resistant to conventional anticonvulsant medications, may respond to MLT but the mechanism of action is unclear. Further research on this novel treatment is urgently needed.

Reliability and base rates of interpersonal themes in narratives from psychotherapy sessions.

We present an analysis of the reliability and base rates of the interpersonal contents of narratives told by patients in psychotherapy. Trained judges rated two samples, including 60 opiate-dependent patients in cognitive or psychodynamic therapy and 72 depressed patients in cognitive or interpersonal therapy. Using a comprehensive system based upon a circumplex model and involving 104 separate categories, we found that most categories of interpersonal behavior could be rated reliably. Potential problem categories were identified and strategies for increasing reliability are discussed. In particular, categories related to the concept of the introject (what the self does to the self) had low reliability. An analysis of the base rates of interpersonal themes revealed that issues related to autonomy/ assertion were most prevalent, although some differences between the two samples were evident. The implications of the results for research on narratives and models of psychotherapy are discussed.

Therapist interventions in early sessions of brief supportive-expressive psychotherapy for depression.

Although psychotherapy manuals provide treatment guidelines, detailed descriptions of therapist interventions in manual-guided therapies are lacking. The purpose of the present investigation was to evaluate the types of therapist interventions in Supportive-Expressive (SE) psychotherapy for depression by using a molecular method of assessment and then to compare the results with those attained with a molar method. Four percent of therapist statements per session early in treatment were interpretations, which most often focused on the patient's parents, significant others, and self in the present time frame. This molecular method for assessing therapist interventions did converge with the molar adherence/competence method.

Acquired brachial-plexus neuropathy in the neonate: a rare presentation of late-onset group-B streptococcal osteomyelitis.

Acquired brachial-plexus neuropathy outside the immediate neonatal period is uncommon. Pseudopalsy of a limb, associated with osteomyelitis, is well recognized. Acquired brachial-plexus neuropathy as the initial presentation of osteomyelitis of the humerus in the neonatal period is described. Three infants presented at 3, 15, and 21 days respectively, with acute monoplegia consistent with brachial-plexus neuropathy. The infants were afebrile and generally well. Initial radiographs of the humerus were normal and blood cultures grew group-B streptococcus in all infants. Nerve conduction studies were consistent with brachial-plexus neuropathy. Following intravenous antibiotics, there was complete recovery in all infants. Osteomyelitis of the humerus should be considered in infants in whom there are no overt signs of sepsis and who present with brachial-plexus neuropathy. Early diagnosis and appropriate treatment should result in a complete neurological recovery.

Encephalopathy complicating Henoch-Schönlein purpura: reversible MRI changes.

A 10-year-old boy with Henoch-Schönlein purpura complicated by encephalopathy, transient cortical blindness, and a secondary generalized seizure is reported. Reversible changes in the posterior white and gray matter were seen on magnetic resonance imaging. Our patient illustrates uncommon neurologic manifestations of Henoch-Schönlein purpura. The nature and location of the lesions and the normalization of the patient's magnetic resonance imaging is consistent with a posterior predominant parieto-occipital encephalopathy and suggests that cerebral edema from blood-brain barrier breakdown may play a central role in the pathophysiology of the central nervous system symptomatology in some patients.

Prospective, open-label, add-on study of lamotrigine in 56 children with intractable generalized epilepsy.

The role of lamotrigine (LTG) in childhood epilepsy is emerging. We evaluated the efficacy and adverse effects of LTG in an open, prospective study of 56 children with generalized epilepsies. Six (11%) children became seizure-free, and 24 (43%) had greater than 50% reduction in seizure frequency. LTG was effective against a broad range of generalized seizure types. Three of 15 patients with Lennox-Gastaut syndrome achieved complete seizure control and eight demonstrated 50 to 99% improvement in seizure control. Increase in seizures (7) and rash (5) were the most common side effects. After valproate was discontinued, LTG therapy was resumed, with no recurrence of rash in any patient. This study suggests that LTG may be a useful drug in the treatment of generalized epilepsies in children.

Varieties of transference patterns in psychotherapy.

This investigation explored the nature of transference of interpersonal patterns in patients' psychotherapy narratives. The relation between interpersonal patterns with significant others in a patient's life and the pattern with the therapist early in treatment was examined. Cluster analysis was used to categorize similar relationships for each of 35 patients. Many patients revealed multiple interpersonal themes in their relationship narratives. Furthermore, these interpersonal themes correlated significantly with the interpersonal pattern extracted from narratives told about the therapist for many of the patients who discussed the therapeutic relationship during therapy. However, the interpersonal pattern evident in the relationship with the therapist was not necessarily the most pervasive pattern exhibited in the narratives about significant others.

Seizures involving the supplementary sensorimotor area in children: a video-EEG analysis.

Most reports of supplementary sensorimotor seizures have been of adults with medically refractory epilepsy. Typically, supplementary sensorimotor seizures have onset in childhood. We describe the electroclinical features in 12 children. Cases were selected from an EEG laboratory population in whom video-EEG was performed routinely on all children. Supplementary sensorimotor seizures were diagnosed when there was bilateral tonic posturing of the upper or lower extremities, preserved consciousness, and lack of postictal confusion. Sensory auras were reported by 8 of 9 children aged > 3 years who had daytime seizures. Speech arrest occurred in all patients in whom it could be assessed, and abnormal vocalization was observed in 7 children. Interictal EEGs were often normal (49% of recordings), and ictal EEG changes could be subtle. An etiology was demonstrated in only 2 children, and brain imaging studies were normal in the other 10 patients. Seizures were controlled with antiepileptic drugs (AEDs) in 6 of the 12 children. The clinical manifestations of supplementary sensorimotor seizures in children are similar to those reported in adults; misdiagnosis is common at all ages.