Child Neurology (Epilepsy and EEG)

Background: Febrile infection-related epilepsy syndrome (FIRES) is a devastating entity characterized by acute onset of refractory epileptic status preceded by a febrile infection, for which no aetiology has been identified so far. Methods: We report the cases of two males presenting with typical FIRES, for whom extensive investigations revealed no specific aetiology. Failure of controlling seizures with multiple anticonvulsants as well as barbiturate coma lead to the decision to try immunotherapy. The first patient received plasma exchange after a negative trial of IVIGs, while the second received plasma exchange in the beginning. Results: Significant improvement in the seizure frequency and intensity was obtained following plasma exchange, and weaning of barbiturate coma was successful in the days following treatment. Both patients remain with significant temporal lobe epilepsy, requiring treatment with 3 anti epileptics. However, cognitive outcome is surprisingly good for both, both exhibiting normal IQs and normal everyday function, with the second patient showing even better recovery, possibly due to earlier treatment with plasma exchange. Conclusion: Our findings of favourable outcome with plasma exchange favours the auto-immune hypothesis often discussed in FIRES. While awaiting further insight onto the aetiology of this syndrome, we suggest a trial of plasma exchange in patients affected.

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Plasma exchange as a source of protein c for acute-onset protein c pathway failure

British Journal of Haematology ◽

10.1046/j.1365-2141.2003.03983_2.x ◽

2002 ◽

Vol 120 (1) ◽

pp. 167-168 ◽

Cited By ~ 3

Author(s):

P. M. Baker ◽

D. M. Keeling ◽

M. Murphy

Keyword(s):

Plasma Exchange ◽

Protein C ◽

Acute Onset

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Corticosteroids versus clobazam in epileptic encephalopathy with ESES: a European multicentre randomised controlled clinical trial (RESCUE ESES*)

Trials ◽

10.1186/s13063-020-04874-2 ◽

2020 ◽

Vol 21 (1) ◽

Author(s):

Bart van den Munckhof ◽

◽

Alexis Arzimanoglou ◽

Emilio Perucca ◽

Heleen C. van Teeseling ◽

...

Keyword(s):

Cognitive Functioning ◽

Treatment Response ◽

Controlled Trial ◽

Epileptic Encephalopathy ◽

Cognitive Outcome ◽

Controlled Study ◽

Controlled Clinical Trial ◽

Epilepsy Syndrome ◽

Eeg Abnormalities ◽

Randomised Controlled

Abstract Background Epileptic encephalopathy with electrical status epilepticus in sleep (ESES) is an epilepsy syndrome occurring almost exclusively in children, usually at an age between 4 and 12 years. It is characterised by abundant sleep-induced epileptic activity in the electroencephalogram (EEG) and by acquired cognitive and behavioural deficits. The goal of treatment is to prevent further decline or even improve cognitive functioning. Based on mostly small and retrospective studies, corticosteroids and clobazam are regarded by many clinicians as the most effective pharmacological treatments. This European multicentre randomised controlled trial is designed to compare the effects of corticosteroids and clobazam on cognitive functioning after 6 months. Secondary outcomes include cognitive functioning after 18 months, EEG abnormalities in sleep, safety and tolerability, and seizure frequency. We also aimed at investigating whether treatment response in epileptic encephalopathy with ESES can be predicted by measurement of inflammatory mediators and autoantibodies in serum. Methods The pragmatic study will be performed in centres with expertise in the treatment of rare paediatric epilepsy syndromes across Europe. A total of 130 patients, 2 to 12 years of age, with epileptic encephalopathy with ESES will be enrolled and randomised in a 1:1 ratio to receive either corticosteroids (monthly intravenous methylprednisolone pulses or daily oral prednisolone) or oral clobazam for 6 months according to an open-label parallel-group design. Follow-up visits with clinical assessment, EEGs, and neuropsychological testing are scheduled for up to 18 months. Blood samples for cytokine and autoantibody testing are obtained before treatment and 8 months after treatment initiation. Discussion The treatment of epileptic encephalopathy with ESES aims at improving cognitive outcome. This randomised controlled study will compare the most frequently used treatments, i.e. corticosteroids and clobazam. If the study proves superiority of one treatment over the other or identifies biomarkers of treatment response, results will guide clinicians in the early treatment of this severe epilepsy syndrome. Trial registration ISRCTN, ISRCTN42686094. Registered on 24 May 2013.

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Acute-onset epilepsy triggered by fever mimicking FIRES (febrile infection-related epilepsy syndrome): The role of protocadherin 19 (PCDH19) gene mutation

Epilepsia ◽

10.1111/j.1528-1167.2011.03193.x ◽

2011 ◽

Vol 52 (11) ◽

pp. e172-e175 ◽

Cited By ~ 22

Author(s):

Nicola Specchio ◽

Lucia Fusco ◽

Federico Vigevano

Keyword(s):

Gene Mutation ◽

Acute Onset ◽

Epilepsy Syndrome

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The Varied Outcomes of Schizophrenia

The Canadian Journal of Psychiatry ◽

10.1177/070674379704200105 ◽

1997 ◽

Vol 42 (1) ◽

pp. 34-43 ◽

Cited By ~ 135

Author(s):

Larry Davidson ◽

Thomas H McGlashan

Keyword(s):

Negative Symptoms ◽

Cognitive Impairments ◽

Acute Onset ◽

Favourable Outcome ◽

Research Literature ◽

Cross Cultural ◽

Poor Outcome ◽

Affective Symptoms ◽

Insidious Onset ◽

Over Time

Objective: To review variations in outcomes in schizophrenia across individual, historical, and cross-cultural boundaries, as well as within specific domains of functioning. Method: Research literature on the outcomes of schizophrenia appearing within the last 8 years was reviewed. Results: First, a review of follow-up studies published in the developed world suggests that heterogeneity in outcome across individuals with schizophrenia remains the rule, with affective symptoms, later and acute onset, and responsiveness to biological treatments predictive of good outcome. Negative symptoms are associated with poor outcome, cognitive impairments, and incapacity in social and work domains. Deterioration appears to occur within the first few months of onset if not already in the prodrome, with recent early-course studies finding longer duration of untreated psychosis associated with insidious onset, negative symptoms, social and work incapacity, and poor outcome. Second, a review of recent cross-cultural and historical studies provides evidence that outcome varies across time and place, schizophrenia having a more favourable outcome in the developing world and becoming a more benign disorder over the course of this century. Third, a review of studies of the domains of functioning within individuals identifies 4 relatively independent dimensions of depression and negative, psychotic, and disorganized symptoms. Cognitive deficits, which are associated with negative symptoms, also constitute a relatively stable dimension over time, showing neither marked deterioration nor improvement once established early in the course of disorder. Conclusions: The early appearance and stability over time of negative symptoms and cognitive impairments call for assertive intervention efforts early in the course of disorder to prevent chronicity and prolonged disability.

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Acute and transient psychotic disorders: precursors, epidemiology, course and outcome

The British Journal of Psychiatry ◽

10.1192/bjp.185.6.452 ◽

2004 ◽

Vol 185 (6) ◽

pp. 452-459 ◽

Cited By ~ 80

Author(s):

Swaran P. Singh ◽

Tom Burns ◽

Shazad Amin ◽

Peter B. Jones ◽

Glynn Harrison

Keyword(s):

Psychotic Disorders ◽

First Episode Psychosis ◽

Acute Onset ◽

Favourable Outcome ◽

First Episode ◽

Affective Psychosis ◽

Episode Psychosis ◽

Icd 10 ◽

Premorbid Functioning ◽

Better Than

BackgroundICD–10 has introduced the diagnostic group acute and transient psychotic disorders (ATPDs; F23). Aims To validate the nosological distinctiveness of ICD–10 ATPDs by following up an inception cohort with first-episode psychosis. Method All patients with first-episode psychosis identified in Nottingham between 1992 and 1994 and diagnosed using ICD–10 criteria were reassessed 3 years later. ATPD outcomes were compared with schizophrenia and affective psychosis. Multivariate analyses were conducted to determine whether acute onset and early remission predicted favourable 3-year outcome in first-episode psychosis. Results Of 168 cases of first-episode psychosis, 32 (19%) received an intake diagnosis of ATPD. The diagnosis of ATPD was stable in women over 3 years, but not in men. Outcomes in ATPD were better than in schizophrenia and similar to affective psychosis. In non-affective psychoses, favourable outcomes were a function of gender and premorbid functioning rather than acute onset and early remission. Conclusions The ICD–10 criteria for ATPDs identify a diagnostically unstable group of disorders. Acute onset and early remission do not independently predict favourable outcome over 3 years in first-episode psychosis.

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The right eye abducens nerve palsy as a cranial neuropathy of dengue fever: The benefit of corticosteroids in an unusual dengue sequela

Malaysian Journal of Ophthalmology ◽

10.35119/myjo.v1i2.25 ◽

2019 ◽

Vol 1 (2) ◽

pp. 145-151

Author(s):

Mohd Khairul Bin Abd Majid

Keyword(s):

Dengue Fever ◽

Nerve Palsy ◽

Abducens Nerve ◽

Acute Onset ◽

Favourable Outcome ◽

Abducens Nerve Palsy ◽

Oral Steroid ◽

Cranial Neuropathy ◽

Systemic Corticosteroids ◽

The Right

Dengue fever is very common in tropical climate countries and the number of reported cases in Malaysia shows an increasing trend recently, according to the Malaysian Clinical Practice Guidelines. Although dengue fever is common, cranial nerve mononeuropathy is a very rare manifestation in relation to other neurological-associated syndromes. We report a rare case of cranial mononeuropathy of dengue fever in Malaysia and highlight the option of steroid usage as an alternative treatment to hasten the neurological recovery. The patient, a 25-year-old healthy policeman, presented with symptomatic viral fever, which was serologically confirmed as dengue fever. He developed acute-onset binocular diplopia, which was secondary to right eye isolated abducens nerve palsy during the critical phase of dengue fever. His visual acuity was 6/6 in both eyes with slightly restricted abduction of the right eye, consistent with right abducens nerve palsy, which was confirmed with a Hess test. There was corresponding diplopia over the right paracentral visual field. Urgent contrasted brain imaging was done, which ruled out the life-threatening intracranial pathology; therefore, a diagnosis of possible subclinical inflammatory changes causing sixth nerve palsy was made. Subsequently, he was treated with intravenous methylprednisolone 500 mg daily for 3 days and regained full extraocular muscle movement after 1 week. Oral steroid was not initiated. In conclusion, although the isolated unilateral cranial mononeuropathy may improve spontaneously within a certain period of time, a short course of systemic corticosteroids may be considered to hasten the recovery, as it has a favourable outcome.

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