Study of Regional Cerebral Blood Flow in West Syndrome

Chiron, Catherine; Dulac, Olivier; Bulteau, Christine; Nuttin, C.; Depas, Gisèle; Raynaud, Claude; Syrota, A.

doi:10.1111/j.1528-1157.1993.tb00450.x

Cited by 75 publications

(41 citation statements)

References 28 publications

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“…Ictal SPECT had been used as a new method of slow dye infusion according to a previous study [9]. Regardless of the difficulty of ictal SPECT in IS, we experienced high concordance (71.4%) with EEG foci, and our findings are similar to the results of previous studies [9,19,20]. Also, localized cerebral hypoperfusion on interictal SPECT might often be associated with IS, even when the MRI is normal, while focal hypoactivity in SPECT could reflect a cortical lesion corresponding to the EEG foci [19,20,21,22].…”

Section: Discussionsupporting

confidence: 82%

Electroencephalography Features of Primary Epileptogenic Regions in Surgically Treated MRI-Negative Infantile Spasms

et al. 2010

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Objective: To evaluate the surgical outcome for intractable, MRI-negative infantile spasms (IS), and to identify diagnostic targets in the focal epileptogenic area by methods other than MRI. Methods: We retrospectively studied 9 patients who had had surgery for intractable IS, and whose lesions did not appear on MRI. We analyzed video/electroencephalography (EEG), single photon emission computed tomography (SPECT) and positron emission tomography (PET) findings and their surgical outcomes. In 7 patients who were seizure free after surgery, we analyzed the EEG parameters for characteristics expected in the primary epileptogenic region. Results: All patients underwent resective surgery including frontal lobectomy and multilobar resection. Seven patients showed an Engel class I outcome, and 2 patients showed a class III outcome. Interictal SPECT results showed 66.7% concordance for the hemisphere affected (lateralization), and 55.6% for lesion location (localization). Ictal SPECT showed 71.4% concordance for lateralization and localization. PET showed 66.7% concordance for lateralization, and 55.6% for localization. EEG parameters, including localized paroxysmal fast activities, spindle-shaped fast activities, repetitive or rhythmic sharp/spike wave discharges, and subclinical seizures showed highly localized specificity, and may serve to identify the epileptogenic lesion. Conclusion: Surgical treatment of MRI-negative IS should be justified using a combination of diagnostic methods.

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Section: Discussionsupporting

confidence: 82%

Electroencephalography Features of Primary Epileptogenic Regions in Surgically Treated MRI-Negative Infantile Spasms

et al. 2010

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“…15,16 In adults, partial status epilepticus with reversible DWI images 4,17 involve the cortical region of seizure origin. ADC is decreased by 6 to 28% or increased by 46%.…”

Section: Discussionmentioning

confidence: 99%

Transient magnetic resonance diffusion abnormalities in West syndrome: the radiological expression of non‐convulsive status epilepticus?

Desguerre

Marti

Valayannopoulos

et al. 2008

Develop Med Child Neuro

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The purpose of this study was to report patients with pharmacoresistant West syndrome of unknown cause whose magnetic resonance imaging (MRI) with diffusion weighted imaging (DWI) showed a transient decrease of diffusion in subcortical structures. Of 20 patients investigated over a 2-year period, three males and three females constitute the present series. They had daily clusters of infantile spasms with hypsarrhythmia for 4 to 24 months before the first investigation. Four were severely hypotonic. All aetiological studies involving intermediary metabolism, peroxysomes, mitochondria, and neurotransmitters in cerebrospinal fluid were negative. Patients underwent DWI when first examined at the mean age of 13 months, and on follow-up examination 6 to 18 months later. Diffusion was decreased in the pallidi and posterior brainstem. It was also decreased for five patients in thalami and for three in dentate nuclei. Repeat MRI, performed when spasms were still present but hypsarrhythmia had ceased, did not show the same abnormalities. Because of recruitment bias, this series probably overestimates the true incidence of such DWI abnormalities. The eventuality of toxic lesions, including some inborn error of metabolism or drug toxicity, is considered unlikely although it could not be excluded. The contribution of the epileptic encephalopathy itself appears the most likely course.West syndrome (WS), the most frequent epileptic encephalopathy, comprises epileptic spasms, psychomotor regression, and hypsarrhythmia. 1 In symptomatic cases, lesions may involve the cortex, the white matter, and the subcortical grey matter. Although conventional magnetic resonance imaging (MRI) discloses aetiology in 90% of cases, 2 a sizeable proportion remain unexplained, including those with poor outcome. In addition, the link between abnormalities disclosed on MRI and clinical symptomatology remains unclear in most instances.All abnormalities disclosed by neuroimaging are not necessarily related to the aetiology of WS. Indeed, steroid therapy produces apparent brain atrophy. 3 Diffusion weighted imaging (DWI) is sensitive to the molecular motion of water and enables cytotoxic oedema to be distinguished from vasogenic oedema. It is widely performed to evaluate ischaemic stroke and is increasingly used in other diseases such as neoplasms, inflammatory diseases, and traumatic injury. In the context of epilepsy, partial status epilepticus with reversible DWI images has been reported. 4 We now report a series of patients in which diffusion weighted magnetic resonance showed abnormalities in the subcortical grey matter in which it is not clear whether they result from the aetiology, from the epileptic encephalopathy itself, or from its treatment. MethodPatients with WS referred to our department without identified aetiology undergo axial and coronal, T 1 , T 2 , and fluid-attenuated inversion recovery (FLAIR) MRI, 2 to 4 months after onset of the disease, repeated after the age of 18 months in case of pharmacoresistance. In 2003, a first patient ...

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“…Although spasms can be ascribed to a variety of etiologies (such as perinatal events, brain malformations, and genetic or chromosomal abnormalities), the clinical and electroencephalographic phenotypes are stereotypical - suggesting the involvement of highly specific, age-dependent processes that involve both cortical and subcortical structures (Lado and Moshé, 2002). In positron emission tomography (PET) and single-photon emission computed tomography (SPECT), hemodynamic and metabolic changes have been described within cortical malformations (Hrachovy and Frost, 1989, Chiron et al, 1993, Chugani et al, 1990, Haginoya et al, 2013), but also in remote cortical and subcortical structures, including the brainstem (Hrachovy and Frost, 1989, Chiron et al, 1993, Chugani et al, 1990, Chugani et al, 1992, Haginoya et al, 1998, Haginoya et al, 1999, Haginoya et al, 2013). Some authors suggested that the involvement of the cortex is restricted to the cortical malformations (i.e.…”

Section: Introductionmentioning

confidence: 99%

Non-invasive, multimodal analysis of cortical activity, blood volume and neurovascular coupling in infantile spasms using EEG-fNIRS monitoring

Bourel-Ponchel

Mahmoudzadeh

Delignières

et al. 2017

NeuroImage: Clinical

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Although infantile spasms can be caused by a variety of etiologies, the clinical features are stereotypical. The neuronal and vascular mechanisms that contribute to the emergence of infantile spasms are not well understood. We performed a multimodal study by simultaneously recording electroencephalogram and functional Near-infrared spectroscopy in an intentionally heterogeneous population of six children with spasms in clusters. Regardless of the etiology, spasms were accompanied by two phases of hemodynamic changes; an initial change in the cerebral blood volume (simultaneously with each spasm) followed by a neurovascular coupling in all children except for the one with a large porencephalic cyst. Changes in cerebral blood volume, like the neurovascular coupling, occurred over frontal areas in all patients regardless of any brain damage suggesting a diffuse hemodynamic cortical response. The simultaneous motor activation and changes in cerebral blood volume might result from the involvement of the brainstem. The inconstant neurovascular coupling phase suggests a diffuse activation of the brain likely resulting too from the brainstem involvement that might trigger diffuse changes in cortical excitability.

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Study of Regional Cerebral Blood Flow in West Syndrome

Cited by 75 publications

References 28 publications

Electroencephalography Features of Primary Epileptogenic Regions in Surgically Treated MRI-Negative Infantile Spasms

Electroencephalography Features of Primary Epileptogenic Regions in Surgically Treated MRI-Negative Infantile Spasms

Transient magnetic resonance diffusion abnormalities in West syndrome: the radiological expression of non‐convulsive status epilepticus?

Non-invasive, multimodal analysis of cortical activity, blood volume and neurovascular coupling in infantile spasms using EEG-fNIRS monitoring

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