Infantile spasms: I. PET identifies focal cortical dysgenesis in cryptogenic cases for surgical treatment

Chugani, Harry T.; Shields, W. Donald; Shewmon, D. Alan; Olson, Donald M.; Phelps, Michael E.; Peacock, Warwick J.

doi:10.1002/ana.410270408

Cited by 576 publications

(273 citation statements)

References 21 publications

(1 reference statement)

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“…According to this model, spasms would result from projections of the activated brainstem regions to the spinal cord neurons as well as from the lenticular activation. This model provides an explanation for the observation that patients with infantile spasms with lesions confined to the cerebral cortex can have their spasms abolished and their EEGs improved after resection of cortical lesions (Chugani et al, 1990, Uthman et al, 1991, Wyllie et al, 1996b, Hrachovy and Frost, 2003. It is still unclear, however, which structures in the described network are primarily responsible for hypsarrhythmia.…”

Section: Pathogenesismentioning

confidence: 89%

“…Studies on functional neuroimaging mostly with PET technology in West syndrome have revealed putamen, brainstem and various cortical regions playing an important role in the pathogenesis of hypsarrhythmia (Chugani et al, 1990, Haginoya et al, 2000, Hrachovy et al, 1981, Morimatsu et al, 1972, Metsahonkala et al, 2002, Neville, 1972, Satoh et al, 1986, Hrachovy and Frost, 2003. Using simultaneous EEG-fMRI recordings, Siniatchkin et al (2007) have demonstrated recently, that the high-amplitude slow cortical activity in hypsarrhythmia is associated with positive BOLD effects in putamen and brainstem, and that multifocal epileptiform discharges are correlated with positive BOLD effects in occipital cortex and various other cortical areas.…”

Section: Pathogenesismentioning

confidence: 99%

See 1 more Smart Citation

Neuronal Networks in West Syndrome as Revealed by Source Analysis and Renormalized Partial Directed Coherence

et al. 2012

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Section: Pathogenesismentioning

confidence: 89%

Section: Pathogenesismentioning

confidence: 99%

Neuronal Networks in West Syndrome as Revealed by Source Analysis and Renormalized Partial Directed Coherence

et al. 2012

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“…Moreover, infantile spasms have been suppressed by surgical removal of focal cortical lesions where focal hypometabolism was detected by positron emission tomography (15). Regarding the relation between cortical and sub- gested that the cortical epileptogenic region involving the primary sensorimotor area could function as the primary generator for infantile spasms because focal pathologic lesions were detected in those areas by MRI and positron-emission tomography (PET) scans, and asymmetric and asynchronous spasms were significantly associated with contralateral ictal EEG discharges.…”

Section: Discussionmentioning

confidence: 99%

Analysis of Ictal EEGs of Epilepsy Associated with Tuberous Sclerosis

Ohmori¹,

Ohtsuka²,

Ohno³

et al. 1998

Epilepsia

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Summary: Purpose:To investigate the significance of cortical pathology of tonic spasms in patients with tuberous sclerosis.Methods: The subjects were 38 patients with epilepsy associated with tuberous sclerosis. We analyzed ictal EEGs of tonic spasms and partial seizures by means of video-EEG monitoring for a total of 763 tonic spasms in 20 patients and 107 partial seizures in 15 patients. We also investigated the relation between partial seizures and magnetic resonance imaging (MRI) findings of these patients.Resulrs: Ictal EEG patterns of tonic spasms were divided into generalized and focal patterns. Thirteen patients had only generalized patterns, whereas seven had both patterns. In five patients who had focal ictal patterns of tonic spasms and partial Tuberous sclerosis is one of the main causes of intractable generalized epilepsies such as West syndrome (WS) and Lennox-Gastaut syndrome (LGS) (1-3). Moreover, frontal lobe epilepsies with tuberous sclerosis frequently exhibit the Lennox-like EEG patterns (4). In a preceding article (3, we described how the nature of the epilepsy changed between generalized and localizationrelated epilepsies during the clinical course in a significant number of patients with tuberous sclerosis. Seizure patterns also changed between generalized and partial seizures in an individual patient. In many patients with WS whose epilepsy is associated with tuberous sclerosis, tonic spasms were preceded by partial seizures, or partial seizures appeared after tonic spasms had been suppressed. These findings suggest that cortical pathology plays a significant role in generalized epilepsies associated with tuberous sclerosis.Further to investigate this problem, we carried out a detailed analysis of ictal EEGs of patients with epilepsy with tuberous sclerosis. We also investigated the relation

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“…For these refractory cases, therapy with novel AEDs are definitely necessary (14) especially in Japan, since many AEDs used in other countries have never been available in Japan. Alternatively, some of these cases can be successfully treated by epilepsy surgery, especially cases in Group PE (15,16). We must search extensively for candidates for epilepsy surgery as early in the patients' lives as possible The other group that comprises patients with rare seizures and those in remission but still on AEDs has received insufficient attention to date because of a lack of long-term follow-up studies throughout their lives (13).…”

Section: Discussionmentioning

confidence: 99%

Management of Childhood-onset Epilepsy Evaluated with a Long-term Follow-up Study

Ohtsuka

Kobayashi

Namba

et al. 2008

E&S

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Purpose: While many patients with childhood-onset epilepsy go into remission before reaching adulthood, a significant number of patients continue to suffer from refractory epilepsy. The purpose of this study was to clarify the long-term outcome of childhood-onset epilepsies. Subjects and Methods: We retrospectively studied 445 adult patients with childhoodonset epilepsies who were still being treated at the Department of Child Neurology, Okayama University Hospital. Results: Age at onset of epilepsy was < 7 years in 66% of the patients; 7 to 14 years in 30%; and 15 to 19 years in 5%. We classified the subjects into three groups: Group PE (289 patients) with partial epilepsy; Group GE (60 patients) with generalized epilepsy; Group RE (70 patients) consisting of patients with a history of West syndrome, Lennox-Gastaut syndrome, Doose syndrome and related epileptic syndromes; and 26 other unclassified cases. At follow-up, frequent seizures (≥1 per month) were observed in 26%, 13% and 63% of patients in Groups PE, GE and RE, respectively. Of 168 patients in remission across these three groups, AEDs were discontinued or being reduced in 21% each, while 36% had experienced relapse of seizures, mainly caused by AED withdrawal.

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Infantile spasms: I. PET identifies focal cortical dysgenesis in cryptogenic cases for surgical treatment

Cited by 576 publications

References 21 publications

Neuronal Networks in West Syndrome as Revealed by Source Analysis and Renormalized Partial Directed Coherence

Neuronal Networks in West Syndrome as Revealed by Source Analysis and Renormalized Partial Directed Coherence

Analysis of Ictal EEGs of Epilepsy Associated with Tuberous Sclerosis

Management of Childhood-onset Epilepsy Evaluated with a Long-term Follow-up Study

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