Severe transitory encephalopathy with reversible lesions of the claustrum

Sperner, J.; Sander, B.; Lau, Stefanie; Krude, Heiko; Scheffner, D.

doi:10.1007/bf01396197

Cited by 41 publications

(35 citation statements)

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“…Several similar cases had previously been described, including several children with similar neuro-radiologic features, which were attributed to the spectrum of febrile infection-related epilepsy syndrome (FIRES) (13–15). Bilateral claustrum abnormalities were associated with medically intractable SE, focal motor seizures, and myoclonic seizures.…”

Section: Introductionmentioning

confidence: 66%

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New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features

et al. 2017

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New-onset refractory status epilepticus (NORSE) is a rare but challenging condition occurring in a previously healthy patient, often with no identifiable cause. We describe the electro-clinical features and outcomes in a group of patients with NORSE who all demonstrated a typical magnetic resonance imaging (MRI) sign characterized by bilateral lesions of the claustrum. The group includes 31 patients (12 personal and 19 previously published cases; 17 females; mean age of 25 years). Fever preceded status epilepticus (SE) in 28 patients, by a mean of 6 days. SE was refractory/super-refractory in 74% of the patients, requiring third-line agents and a median of 15 days staying in an intensive care unit. Focal motor and tonic–clonic seizures were observed in 90%, complex partial seizures in 14%, and myoclonic seizures in 14% of the cases. All patients showed T2/FLAIR hyperintense foci in bilateral claustrum, appearing on average 10 days after SE onset. Other limbic (hippocampus, insular) alterations were present in 53% of patients. Within the personal cases, extensive search for known autoantibodies was inconclusive, though 7 of 11 patients had cerebrospinal fluid lymphocytic pleocytosis and 3 cases had oligoclonal bands. Two subjects died during the acute phase, one in the chronic phase (probable sudden unexplained death in epilepsy), and one developed a persistent vegetative state. Among survivors, 80% developed drug-resistant epilepsy. Febrile illness-related SE associated with bilateral claustrum hyperintensity on MRI represents a condition with defined clinical features and a presumed but unidentified autoimmune etiology. A better characterization of de novo SE is mandatory for the search of specific etiologies.

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Section: Introductionmentioning

confidence: 66%

“…Nineteen cases were retrieved from previous case-reports and series (13–15, 20–32), and 12 personal cases fulfilled the inclusion criteria. Tables 1 and 2 summarize the main clinical and MRI findings of all 31 patients.…”

Section: Resultsmentioning

confidence: 99%

New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features

et al. 2017

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“…Early neuroscientists often examined the brains of the deceased in order to relate abnormalities with some functional deficit the patient experienced during life. The claustrum cannot be studied in this way, as researchers have yet to induce a lesion affecting the claustrum without also affecting neighboring structures, although some natural lesions have been reported in epileptic subjects (Sperner et al, 1996; Duffau et al, 2007). Even with the advent of histological staining techniques, it is incredibly difficult to localize an injection in the claustrum without some tracer spreading.…”

Section: Methods For Studying the Claustrummentioning

confidence: 99%

“…The case indicates that lesions of the claustrum function more like gray matter disease than white matter disease. Additionally, the case study implies a functional correlation between seizures, behavioral state, and abnormalities in the claustrum (Sperner et al, 1996). …”

Section: Clinical Syndromes Associated With Claustral Damage and Dysfmentioning

confidence: 99%

A case study in connectomics: the history, mapping, and connectivity of the claustrum

Torgerson

Horn

2014

Front. Neuroinform.

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The claustrum seems to have been waiting for the science of connectomics. Due to its tiny size, the structure has remained remarkably difficult to study until modern technological and mathematical advancements like graph theory, connectomics, diffusion tensor imaging, HARDI, and excitotoxic lesioning. That does not mean, however, that early methods allowed researchers to assess micro-connectomics. In fact, the claustrum is such an enigma that the only things known for certain about it are its histology, and that it is extraordinarily well connected. In this literature review, we provide background details on the claustrum and the history of its study in the human and in other animal species. By providing an explanation of the neuroimaging and histology methods have been undertaken to study the claustrum thus far—and the conclusions these studies have drawn—we illustrate this example of how the shift from micro-connectomics to macro-connectomics advances the field of neuroscience and improves our capacity to understand the brain.

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“…In 1996, its involvement in a case of de novo refractory status was reported for the first time. 16 Only in recent years has the scientific community gained a strong interest in the function of the claustrum, mainly with respect to its role in sensory integration and consciousness. [17][18][19][20][21] METHODS Patients and investigations.…”

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confidence: 99%

Claustrum damage and refractory status epilepticus following febrile illness

et al. 2015

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Objective: To characterize the clinical, EEG, and brain imaging findings in an adult case series of patients with de novo refractory status epilepticus (SE) occurring after a febrile illness.Methods: A retrospective study (2010)(2011)(2012)(2013) was undertaken with the following inclusion criteria: (1) previously healthy adults with refractory SE; (2) seizure onset 0-21 days after a febrile illness; (3) lacking evidence of infectious agents in CSF; (4) no history of seizures (febrile or afebrile) or previous or concomitant neurologic disorder.Results: Among 155 refractory SE cases observed in the study period, 6 patients (17-35 years old) fulfilled the inclusion criteria. Confusion and stupor were the most common symptoms at disease onset, followed after a few days by acute repeated seizures that were uncountable in all but one. Seizures consisted of focal motor/myoclonic phenomena with subsequent generalization. Antiepileptic drugs failed in every patient to control seizures, with all participants requiring intensive care unit admission. Barbiturate coma with burst-suppression pattern was applied in 4 out of 6 patients for 5-14 days. One participant died in the acute phase. In each patient, we observed a reversible bilateral claustrum MRI hyperintensity on T2-weighted sequences, without restricted diffusion, time-related with SE. All patients had negative multiple neural antibodies testing. Four out of 5 surviving patients developed chronic epilepsy.Conclusions: This is a hypothesis-generating study of a preliminary nature supporting the role of the claustrum in postfebrile de novo SE; future prospective studies are needed to delineate the specificity of this condition, its pathogenesis, and the etiology. Neurology ® 2015;85:1224-1232 GLOSSARY ADC 5 apparent diffusion coefficient; AED 5 antiepileptic drug; FIRES 5 febrile infection-related epilepsy syndrome; ICU 5 intensive care unit; IVIg 5 IV immunoglobulin; NORSE 5 new-onset refractory status epilepticus; PEX 5 plasma exchange; SE 5 status epilepticus.In the last 2 decades, several authors described a series of syndromes characterized by the development of a difficult to treat status epilepticus (SE) in previously healthy children after a febrile illness.1-7 The condition is characterized by a refractory SE and followed by drug-resistant epilepsy, with often severe neuropsychiatric sequelae or death. These entities have been identified by different acronyms, but febrile infection-related epilepsy syndrome (FIRES) is the one that best underscores the main features of the disorder.8 Cases with a similar clinical picture have been described also in adults and in these case series the most frequently used definition is new-onset refractory SE (NORSE).9-13 Recently, it was pointed out that different terms probably have been used to describe the same condition.14,15 However, there is no consensus among investigators. Adult cases are more heterogeneous, some with clear similarities with FIRES cases (with only the age at onset as the main difference), other...

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Severe transitory encephalopathy with reversible lesions of the claustrum

Cited by 41 publications

References 6 publications

New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features

New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features

A case study in connectomics: the history, mapping, and connectivity of the claustrum

Claustrum damage and refractory status epilepticus following febrile illness

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