Effect of valproate on the sleep microstructure of juvenile myoclonic epilepsy patients – a cross-sectional CAP based study

ObjectiveStudies looking at the effect of anti-epileptic medications on sleep microstructure of patients with epilepsy are almost non-existent. The aim of this study was to compare sleep microstructural characteristics of drug-naïve juvenile myoclonic epilepsy (JME) patients with those on valproate (VPA) monotherapy.MethodsThree age- (p = 0.287) and gender- (p = 0.766) matched groups (N = 20 in each group): (1) drug-naïve JME (mean age: 21.2 ± 4.06 years; M : F = 9:11); (2) JME on VPA (mean age: 21.85 ± 4.28 years; M : F = 11:9); (3) healthy controls (mean age: 23.2 ± 3.82 years; M : F = 9:11) underwent overnight polysomnography. Scoring and analysis of arousals American Sleep Disorders Association (ASDA, 2002), cyclic alternating pattern (CAP) (Terzano et al., 2002) parameters were performed. Comparison of arousal and CAP parameters was performed using one-way ANOVA, followed by pairwise comparisons using Fisher's LSD test (p ≤ 0.05).ResultsRapid eye movement (REM) arousal indices were higher in JME patients (Group 1 [p = 0.002] and Group 2 [p <0.001]), whereas the overall and NREM arousal indices were comparable between the three groups. CAP rate was higher in JME patients as compared to controls (p <0.001). Duration of phase A and its subtypes (p <0.001) was reduced in drug-naïve patients as compared to VPA group and controls. Finally, percentage of phase A1 (p = 0.003) was decreased and A3 (p = 0.045) was increased in drug-naïve patients as compared to VPA group and controls.ConclusionsWe found significant alterations in REM arousal indices and several CAP parameters in JME patients. However, many of these alterations were not seen in the valproate group. This might indicate that anti-epileptic medications such as valproate may beneficially modulate arousal instability in JME patients, and hence promote sleep quality and continuity.     

Psychological treatment of sexual problems. Thematic analysis of guidelines and recommendations,based on a systematic literature review 2001–2010

In order to explore the basis for evaluation of quality in psychological treatment of sexual problems, a literature search, confined to publications between 2001 and 2010, was conducted in 2012. Two hundred sixty one articles were found and ordered according to the subject and type of publication. Thematic analysis of 37 articles on guidelines and recommendations for treatment of sexual problems is presented. Guidelines and recommendations published in the period from 2001 up to and including 2010 provides an overview of the discussion among professionals in the first decade of the 21st century. Six themes are analysed: (1) definitions of male and female dysfunctions, (2) prevalence of sexual problems, (3) lack of randomized and controlled trials, (4) need for a bio-psycho-social approach, (5) the subjective perspective, and (6) recommendations. Excerpts from the material pertaining to discussions and/or reflections on these themes are presented and discussed.

The main conclusions are

1. (1) the definition of psychological treatment is imprecise and needs clarification,

2. (2) not every therapist should work with sexual problems,

3. (3) sexual problems cannot be regarded as “dysfunctions” in the traditional biomedical understanding, but must be understood in a bio-psycho-social perspective,

4. (4) psychological treatment is recommended in spite of lack of evidence,

5. (5) the literature reflects a more reserved belief in pharmacological treatment,

6. (6) material that qualifies as scientific evidence includes more than randomized and controlled trials and

7. (7) combination treatment is recommended.

     

Intracranial arachnoid cysts and epilepsy in children: Should this be treated surgically? Our 29-year experience and review of the literature

Introduction and objectiveArachnoid cysts (ACs) are relatively frequent lesions related to different neurological symptoms, being mostly incidentally diagnosed. This study aims to clarify whether AC surgery in epileptic patients is useful in their treatment.Material and methodsThe patients registered in the database of the Neuropediatrics Section from May 1990 to August 2019 are analyzed retrospectively. Patients in whom the diagnosis of ACs and epilepsy coincide are studied. The location, size and number of ACs, neurological development, age at diagnosis, follow-up time, the performance of surgery on the cyst, evolution, anatomical relationship between brain electrical activity and location of AC, and type of epilepsy are analyzed.ResultsAfter analyzing the database, we found 1881 patients diagnosed with epilepsy, of which 25 had at least one intracranial AC. In 9 of the patients, cerebral or genetic pathologies were the cause of epilepsy. Of the other 16, only 2 patients showed that the type of epilepsy and the epileptogenic focus coincided with the location of the AC; one of them was surgically treated without success, and the other one remained asymptomatic without receiving medical or surgical treatment.ConclusionsAlthough it is necessary to design a prospective study to establish causality, the results of our research and the available literature suggest that there is no causal relationship between the presence of ACs and epilepsy. The study and treatment of these patients should be carried out in a multidisciplinary epilepsy surgery unit, without initially assuming that the AC is the cause of epilepsy.     

Clinical characteristics and outcomes of patients with Guillain–Barré and acquired CNS demyelinating overlap syndrome: a cohort study based on a literature review

Abstract

Background:

Some patients with Guillain–Barré syndrome (GBS) also have acquired demyelination of the central nervous system (CNS) (i.e. acquired demyelinating syndrome, ADS). Often, the overlap of GBS and ADS is overlooked. Therefore, we evaluated case reports of GBS/ADS overlap syndrome.

Methods:

We mainly performed website-based research based on articles in cases presented with GBS/ADS overlap syndrome. A total of 66 cases were included. Clinical and prognosis data were analyzed.

Results:

A total of 85% of patients with simultaneous or consecutive occurrence of GBS and ADS were identified within 4 weeks of the initial diagnosis. Transverse myelitis (TM) (32%) was the most common ADS found in GBS/ADS. Patients with Miller Fisher syndrome (MFS)/ADS overlap syndrome had greater female predominance, mean age, frequency of onset at the same time period, or within a short period, and percentage of sole involvement of the subtentorial region. The outcome was favorable based on the functional status in 74% of patients. The sensory level (OR = 0·182, 95% CI = 0·055–0·598; P = 0·005) was the best predictor of a poor outcome, while visual deficit (OR = 4·667, 95% CI = 1·187–18·352; P = 0·027) predicted a favorable outcome.

Conclusion:

The ADS in GBS are diverse, CNS demyelinating may occur at any time, but early in the GBS course (and vice versa). MFS/ADS overlap syndromes is more common. The prognosis is generally good, but patients with sensory level deficit are likely to have a poor prognosis. The features of MFS/other CIS may better reflect involvement of the brainstem in MFS itself, rather than ADS in autoimmune peripheral neuropathies.     

DBS in tremor with dystonia: VIM,GPi or both? A review of the literature and considerations from a single-center experience

Journal of Neurology - Deep brain stimulation (DBS) is an established treatment for dystonia and tremor. However, there is no consensus about the best surgical targeting strategy in patients with...     

Parkinsonism associated with Sj?gren's syndrome: three cases and a review of the literature.

Sj?gren's syndrome (SS) is a common multisystem autoimmune disorder. As with other autoimmune disorders such as systemic lupus erythematosus (SLE), SS has been associated with a wide range of neurologic abnormalities. Parkinsonism has been reported previously in five SS patients. We present three additional cases of SS with parkinsonism.     

Medulloepithelioma of the optic nerve with intradural extension – report of two cases and a review of the literature

Medulloepithelioma is an uncommon tumour. Among the various sites of origin the optic nerve is rare. We report here two cases of medulloepithelioma of the optic nerve that had extended intradurally into the cranial cavity. A review of the published cases is also given. Received: 30 April 1999 Revised: 17 June 1999     

Acute psychosis during the postictal period in a patient with idiopathic generalized epilepsy: postictal psychosis or aggravation of schizophrenia? A case report and review of the literature

In a cross-sectional study, we evaluated the impact of the chronic use of benzodiazepines (BDZ) prescribed for seizure control on the anxiety levels of patients with temporal lobe epilepsy. We assessed the anxiety level of 99 patients with temporal lobe epilepsy with (n=15) or without (n=84) BDZ for seizure control, using the Beck Anxiety Inventory (BAI) or the Hamilton Anxiety Scale (HAMA). Independent risk factors for high anxiety levels were being a female patient (O.R.=2.93; 95% C.I.=1.05-8.16; p=0.039), having uncontrolled seizures (O.R.=4.49; 95% C.I.=1.66-12.11; p=0.003) and having a history of a psychiatric disorder (O.R.=4.46; 95% C.I.=1.63-12.21; p=0.004). However, there were no statistically significant differences in anxiety levels between patients utilizing or not utilizing BDZ prescribed exclusively for seizure control. We concluded that in our study, patients with chronic use of BDZ prescribed exclusively for seizure control showed similar anxiety levels than patients who were not using this class of drug. Additional studies are needed to define better strategies for the treatment of anxiety disorders in epilepsy.     

Chemical priming for spinal cord injury: a review of the literature part II��potential therapeutics

Introduction  

Spinal cord injury is a complex cascade of reactions secondary to the initial mechanical trauma that puts into action the innate properties of the injured cells, the circulatory, inflammatory, and chemical status around them, into a non-permissive and destructive environment for neuronal function and regeneration. Priming means putting a cell, in a state of “arousal” towards better function. Priming can be mechanical as trauma is known to enhance activity in cells.     

Myasthenia gravis and Waldenström's macroglobulinemia: a case report and review of the literature

An elderly man with ocular type myasthenia gravis (MG) was found to have monoclonal gammopathy (IgM). Waldenström's macroglobulinemia (WM) was proven later. This is the second case report of MG associated with WM. However, this case is unique due to the presence of anti‐acetylcholine receptor (anti‐AChR) antibody (IgG). MG is considered to be an autoimmune disease and WM an immunoproliferative disorder. The association of MG and WM is not clear.     

Neuropsychiatric debut as a presentation of Guillain-Barré Syndrome: An atypical clinical case and literature review

IntroductionGuillain Barré Syndrome (GBS) is an acute immune-mediated polyneuropathy most frequently presenting two to four weeks after an acute mild-moderately severe infection as progressive muscular weakness of the lower limbs extending proximally with dysreflexia and autonomic dysfunction. While GBS is typically believed to be isolated to the Peripheral Nervous System, Central Nervous System (CNS) and psychiatric manifestations as a sequela of the disease have been described in different imaging and clinical studies. Many variants of presentation of GBS have been recognized, however a case presenting with primarily psychiatric and autonomic dysfunction preceding muscle weakness has not been cited in the literatures to date.Case presentationWe describe a 24-year-old previously healthy male presenting with behavioral symptoms including depression, anxiety, and amnesia, and autonomic dysfunction which preceded muscle weakness by two weeks. CNS imaging and blood work results were unremarkable. GBS was confirmed upon cerebral spinal fluid analysis remarkable for an important cytoalbuminologic dissociation and markedly elevated protein concentration. The patient responded well to five cycles of inpatient plasmapheresis and short-term selective serotonin reuptake inhibitor treatment with complete recovery of both neurological and behavioral symptoms.ConclusionThough GBS is typically considered a peripheral neuropathy, evidence for CNS involvement exists; GBS should be considered within the differential diagnosis, and neurological features should be monitored, in a patient with new onset unclear psychiatric and CNS symptoms.     

Marijuana: an effective antiepileptic treatment in partial epilepsy? A case report and review of the literature

Although more data are needed, animal studies and clinical experience suggest that marijuana or its active constituents may have a place in the treatment of partial epilepsy. Here we present the case of a 45-year-old man with cerebral palsy and epilepsy who showed marked improvement with the use of marijuana. This case supports other anecdotal data suggesting that marijuana use may be a beneficial adjunctive treatment in some patients with epilepsy. Although challenging because of current federal regulations, further studies are needed to examine the role of marijuana in the treatment of this disorder.     

Langerhans cell histiocytosis of the parietal bone with epidural and extracranial expansion – case report and a review of the literature

Langerhans cell histiocytosis is a rare neoplasm that belongs to the histiocytic and dendritic cell neoplasm group according to the 2008 WHO classification. It has been defined as neoplastic proliferation of Langerhans cells that express CD1a and S-100 proteins and have Birbeck granules on the ultrastructural examination. Clinical presentation and behaviour are heterogeneous and can range from a solitary lytic bone lesion with a favourable course to a fatal disseminated leukaemia-like form, with a wide spectrum of intermediate clinical presentations between these two extremes. Here, we present a case report of a solitary calvarial lesion in an adolescent boy along with a review of the literature. Presenting features, initial diagnostic evaluation and treatment protocol of a unifocal monosystemic calvarial location of LCH are presented.     

The moral brain and moral behaviour in patients with Parkinson’s disease: a review of the literature

Morality is a complex and versatile concept that necessitates the integrated activity of multiple interacting networks in the brain. Numerous cortical and subcortical areas, many of which are implicated in either emotional and cognitive control or Theory of Mind, are involved in the processing of moral behaviour. Different methods have been used to investigate various aspects of morality, which has lead to confusing and sometimes opposing results. Emotional, cognitive and personality changes have long been recognized in Parkinson’s disease (PD) patients, suggesting a potential impact on moral aspects of behaviour in daily living situations. Alterations in social cognition have been described in all stages of PD but these are rather directly related to PD pathology and not to dopaminergic or DBS treatment. There are no convincing data supporting the hypothesis that dopaminergic treatment or deep brain stimulation of the STN per se interfere with morality in PD patients, although subgroups of patients may display socially unacceptable behaviour. Research in social cognition in PD patients is a fascinating topic that needs further attention in view of the impact on quality of life for PD patients and their caregivers.