•CIDP presents rarely with cranial nerve involvement with ocular signs.•We present a case of bilateral facial nerve palsy as a rare manifestation of CIDP.•CIDP is associated with Crohn’s disease as ...well as anti-TNF α agents.•The case was treated with withdrawal of anti-TNF α with steroid and IVIg.
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) presents uncommonly with cranial nerve involvement with ophthalmological implications.
We report the case of a 37year-old man who developed CIDP which manifested as progressive and relapsing bilateral facial nerve palsy with lagophthalmos and exposure keratopathy, in the setting of treatment of Crohn’s disease with the anti-TNF-alpha agent adalimumab.
Symptoms gradually improved over the course of several months following withdrawal of adalimumab and treatment with intravenous immunoglobulin (IVIg) and oral prednisolone.
Bilateral facial nerve involvement occurs uncommonly as a feature of CIDP in its classic form. The prognosis is good for recovery of facial nerve function with discontinuation of anti-TNF-alpha therapy and concurrent use of steroid and intravenous immunoglobulin in this case.
Abstract Epilepsy encompasses a diverse group of seizure disorders caused by a variety of structural, cellular and molecular alterations of the brain primarily affecting the cerebral cortex, leading ...to recurrent unprovoked epileptic seizures. In this two-part review we examine the mechanisms underlying normal neuronal function and those predisposing to recurrent epileptic seizures starting at the most basic cellular derangements (Part 1) and working up to the highly complex epileptic networks (Part 2). We attempt to show that multiple factors can modify the epileptic process and that different mechanisms underlie different types of epilepsy, and in most situations there is an interplay between multiple genetic and environmental factors.
Aim
To determine whether short‐term intensive group‐based therapy combining modified constraint‐induced movement therapy and bimanual therapy (hybrid‐CIMT) is more effective than an equal total dose ...of distributed individualized occupational therapy (standard care) on upper limb motor and individualized outcomes.
Method
Fifty‐three children with unilateral cerebral palsy (69% males; mean age 7y 10mo, SD 2y 4mo; Manual Ability Classification System level I, n=24; level II, n=23) were randomly allocated, and 44 received either hybrid‐CIMT (n=25) or standard care (n=19). Standard care comprised six weekly occupational therapy sessions and a 12‐week home programme. Outcomes were assessed at baseline, 13 weeks, and 26 weeks after treatment.
Results
Groups were equivalent at baseline. Standard care achieved greater gains on satisfaction with occupational performance after intervention (estimated mean difference −1.2, 95% CI −2.2 to −0.1; p=0.04) and Assisting Hand Assessment at 26 weeks (estimated mean difference 3.1, 95% CI 0.2–6.0; p=0.04). Both groups demonstrated significant improvements in dexterity of the impaired upper limb, and bimanual and occupational performance over time. The differences between groups were not clinically meaningful.
Interpretation
There were no differences between the two models of therapy delivery. Group‐based intensive camps may not be readily available; however, individualized standard care augmented with a home programme may offer an effective alternative but needs to be provided at a sufficient dose.
What this paper adds
Distributed occupational therapy with a home programme was equivalent to an intensive group‐based therapy on unimanual, bimanual, and individualized outcomes.
The target dose of therapy was not achieved for the distributed model of occupational therapy group as home practice was substantially less than anticipated.
The challenges for both researchers and clinicians in reliance on home practice to augment direct therapy provision are highlighted.
Clinically meaningful long‐term changes in upper limb outcomes should be considered in relation to both the amount of therapy and nature of intervention.
This article is commented on by Eliasson on page 498 of this issue.
Population-based stroke incidence studies are the only accurate way to determine the number of strokes that occur in a given society. Because the major stroke subtypes have different patterns of ...incidence and outcome, information on the natural history of stroke subtypes is essential. The purpose of the present study was to determine the incidence and case-fatality rate of the major stroke subtypes in a geographically defined region of Melbourne, Australia.
All suspected strokes that occurred among 133 816 residents of suburbs north and east of Melbourne, Australia, during a 12-month period of 1996 and 1997 were identified and assessed. Multiple overlapping sources were used to ascertain cases, and standard criteria for stroke and case-fatality were used. Stroke subtypes were defined by CT, MRI, and autopsy.
Three hundred eighty-one strokes occurred among 353 persons during the study period, with 276 (72%) being first-ever-in-a-lifetime strokes. Of these, 72.5% (95% CI 67.2% to 77.7%) were cerebral infarction, 14.5% (95% CI 10.3% to 18.6%) were intracerebral hemorrhage, 4.3% (95% CI 1.9% to 6.8%) were subarachnoid hemorrhage, and 8.7% (95% CI 5.4% to 12.0%) were stroke of undetermined type. The 28-day case-fatality rate was 12% (95% CI 7% to 16%) for cerebral infarction, 45% (95% CI 30% to 60%) for intracerebral hemorrhage, 50% (95% CI 22% to 78%) for subarachnoid hemorrhage, and 38% (95% CI 18% to 57%) for stroke of undetermined type.
The overall distribution of stroke subtypes and 28-day case-fatality rates are not significantly different from those of most European countries or the United States. There may, however, be some differences in the incidence of subtypes within Australia.
Summary
Purpose: We used transcranial magnetic stimulation (TMS) to investigate whether there were any characteristic cortical excitability changes in progressive myoclonic epilepsy (PME) compared ...to juvenile myoclonic epilepsy (JME).
Methods: Six patients with PME were studied. Motor threshold (MT) at rest and recovery curve analysis using paired‐pulse stimulation at a number of interstimulus intervals (ISIs) was determined. Results were compared to those of 9 patients with chronic refractory JME and 10 with chronic well‐controlled JME.
Results: PME showed a marked increase in cortical excitability at all the long ISIs (p < 0.01), compared to refractory JME (effect sizes ranging from 1.4 to 1.9) and well‐controlled JME (effect sizes ranging from 2.0 to 2.4). Significant differences at the short ISIs 2–5 ms were seen only on comparison with the well‐controlled group (p < 0.05, effect size 0.6, 0.7). There were no significant differences in MTs of PME compared to either JME groups.
Conclusion: Our findings demonstrate specific differences in cortical excitability using TMS between PME and those with JME, particularly at long latencies in the paired‐pulse paradigm, implicating a role for γ‐aminobutyric acid (GABA)B‐mediated networks.
Objectives. To determine retention of treatment outcomes at 52 weeks following a matched-pairs randomized comparison trial of constraint-induced movement therapy (CIMT) and bimanual training (BIM). ...Methods. Sixty-four children (mean age = 10.2 ± 2.7 years, 52% male) were included. The Melbourne Assessment of Unilateral Upper Limb Function (MUUL), Assisting Hand Assessment (AHA), and Canadian Occupational Performance Measure (COPM) were the primary outcome measures. Evaluations were at baseline and at 26 and 52 weeks. Results. There were no baseline differences between groups on any measure. No significant differences were found between groups on primary outcomes at 52 weeks. Both groups retained the significant gains made from baseline to 26 weeks at the 1-year follow-up assessment for unimanual capacity on the MUUL, for bimanual performance on the AHA, and on the COPM. Conclusion. Intensive unimanual and bimanual training can both lead to long-term significant improvements in unimanual capacity, bimanual performance, and individualized outcomes. Gains established at 26 weeks were maintained at 12 months postintervention despite most children receiving no direct therapy during that time.
To investigate pregnancy-related disease activity in a contemporary multiple sclerosis (MS) cohort.
Using data from the MSBase Registry, we included pregnancies conceived after 31 Dec 2010 from women ...with relapsing-remitting MS or clinically isolated syndrome. Predictors of intrapartum relapse, and postpartum relapse and disability progression were determined by clustered logistic regression or Cox regression analyses.
We included 1998 pregnancies from 1619 women with MS. Preconception annualized relapse rate (ARR) was 0.29 (95% CI 0.27-0.32), fell to 0.19 (0.14-0.24) in third trimester, and increased to 0.59 (0.51-0.67) in early postpartum. Among women who used fingolimod or natalizumab, ARR before pregnancy was 0.37 (0.28-0.49) and 0.29 (0.22-0.37), respectively, and increased during pregnancy. Intrapartum ARR decreased with preconception dimethyl fumarate use. ARR spiked after delivery across all DMT groups. Natalizumab continuation into pregnancy reduced the odds of relapse during pregnancy (OR 0.76 per month 0.60-0.95, p=0.017). DMT re-initiation with natalizumab protected against postpartum relapse (HR 0.11 0.04-0.32, p<0.0001). Breastfeeding women were less likely to relapse (HR 0.61 0.41-0.91, p=0.016). 5.6% of pregnancies were followed by confirmed disability progression, predicted by higher relapse activity in pregnancy and postpartum.
Intrapartum and postpartum relapse probabilities increased among women with MS after natalizumab or fingolimod cessation. In women considered to be at high relapse risk, use of natalizumab before pregnancy and continued up to 34 weeks gestation, with early re-initiation after delivery is an effective option to minimize relapse risks. Strategies of DMT use have to be balanced against potential fetal/neonatal complications.
Objective
Different pathophysiological mechanisms related to the balance of cortical excitatory and inhibitory influences may underlie focal and generalized epilepsies. We used transcranial magnetic ...stimulation to search for interictal excitability differences between patients with idiopathic generalized epilepsy (IGE) and focal epilepsy.
Methods
Sixty‐two drug‐naive patients with newly diagnosed epilepsy (35 IGE, 27 focal epilepsy) were studied. In the latter group, the seizure focus was not located in the motor cortex. Motor threshold at rest, cortical silent period threshold, recovery curve analysis using paired‐pulse stimulation at a number of interstimulus intervals), and cortical silent period were determined. Results were compared with those of 29 control subjects.
Results
Hyperexcitability was noted in the recovery curves at a number of interstimulus intervals in both hemispheres in patients with IGE and in the hemisphere ipsilateral to the seizure focus in those with focal epilepsy compared with control subjects and the contralateral hemisphere in focal epilepsy. Motor threshold and cortical silent period threshold were higher in the ipsilateral hemisphere in focal epilepsy compared with the contralateral hemisphere. No other intragroup or intergroup differences were found in the other measures.
Interpretation
The disturbance of cortical excitatory/inhibitory function was found to be bilateral in IGE, whereas in focal epilepsy it spread beyond the epileptic focus but remained lateralized. This finding confirms that there are differences in cortical pathophysiology comparing the two major types of epilepsy. Ann Neurol 2007
Although much is known about the long-term outcome of stroke patients in terms of mortality and disability, there has been little research on the patient-centered outcome of health-related quality of ...life (HRQoL). There are limited natural history data on HRQoL beyond 2 years after stroke and no data on those factors present at stroke onset that predict HRQoL beyond 2 years after stroke. For these reasons, we aimed to examine these aspects of HRQoL in an unselected population of stroke patients.
All cases of first-ever stroke from a prospective community-based stroke incidence study (excluding subarachnoid hemorrhage) were assessed 5 years after stroke. HRQoL was measured with the assessment of quality of life instrument. ANOVA was used to determine baseline predictors of HRQoL.
In total, 978 cases were recruited, 45% were male, and the mean age (+/-SD) was 75.5+/-13.8 years. Five years after stroke, 441 (45.1%) were alive and 356 were assessed (80.7%). Those assessed were more often born in Australia and older in age (both P<0.05). Seventy-one survivors (20%) had a very low HRQoL (score < or =0.1). The independent baseline predictors of low HRQoL at 5 years after stroke were increasing age, lower socioeconomic status, and markers of stroke severity.
At 5 years after stroke, we found that a substantial proportion of survivors were suffering from poor HRQoL. As our population ages, the number of strokes and, thus, stroke survivors with poor HRQoL is likely to increase. Therefore, strategies to improve HRQoL should be vigorously pursued.
Summary
Purpose: We used transcranial magnetic stimulation (TMS) to investigate cortical excitability changes in Lennox‐Gastaut syndrome (LGS), anticipating we would find a marked increase in ...excitability compared to other patients with refractory epilepsies.
Methods: Eighteen patients with LGS were studied. Motor threshold (MT), short intracortical inhibition (paired pulse TMS at 2 and 5 msec interstimulus intervals ISIs), intracortical facilitation (10 and 15 msec ISIs), and long intracortical inhibition (100–300 msec ISIs) were measured. Results were compared to those of 20 patients with chronic refractory idiopathic generalized epilepsy (IGE), 20 patients with chronic refractory focal epilepsy, and 20 healthy nonepilepsy controls.
Key Findings: A significant decrease in cortical excitability was observed in LGS compared to the other two groups with refractory epilepsy as evidenced by increased MT and intracortical inhibition at both short (2, 5 msec ISIs), and long (100–300 msec ISIs) as well as decreased intracortical facilitation (10, 15 msec ISIs), (p < 0.01; effect sizes ranging from 0.3 to 1.8). Cortical excitability was also lower in LGS compared to nonepilepsy controls (increased MT and decreased intracortical facilitation; p < 0.05; effect sizes ranging from 0.5 to 0.9).
Significance: Interictal cortical excitability is decreased in LGS; a feature that distinguishes it from other refractory epilepsy syndromes. This decrease may be an important mechanism for the neurobehavioral comorbidities associated with LGS.
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