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Yukio Fukuyama - One of the best experts on this subject based on the ideXlab platform.
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Leigh's Subacute Necrotizing Encephalomyelo‐pathy in a Child with Infantile Spasms and Hypsarrhythmia
Developmental Medicine & Child Neurology, 2008Co-Authors: Shigehiko Kamoshita, Ikuko Mizutani, Yukio FukuyamaAbstract:SUMMARY The pathological examination of the brain of a 7-month-old boy with infantile spasms and Hypsarrhythmia revealed a lesion characteristic of Leigh's subacute necrotizing encephalomyelopathy. The rarity of these two conditions in one patient is indicated in the brief review of the literature. RESUME Encephalomyelopathie necrosante subaigue de Leigh L'examen anatomique du cerveau d'un garcon de 7 mois ayant presente des spasms infantiles et une hypsarythmie a montre des lesions caracteristiques de l'encephalomyelopathie necrosante subaigue de Leigh. La rarete de cette association chez le meme sujet est soulignee par une breve revue de la litterature. ZUSAMMENFASSUNG Leigh's Subakute Nekrotisierende Encephalomyelopathie bei einem Kind mit infantilen Krampfen und Hypsarrhythmie Die pathologische Untersuchung des Gehirns eines 7 Monate alten Jungen mit infantilen Krampfen und Hypsarrhythmie ergab eine fur die Subakute Nekrotisierende Encephalomyelopathie nach Leigh typische Schadigung. Auf die Seltenheit dieser beiden Symptome bei einem Patienten wird in einem kurzen Uberblick uber die Literatur hingewiesen. RESUMEN Encefalomielopatia necrotizante subaguda de Leigh en un nino con espasmos infantiles e hipsarritmia El examen anatomo-patologico de un nino de 7 meses de edad con espasmos infantiles e hipsarritmia revelo una lesion caracteristica de encefalomielopatia necrotizante subaguda de Leigh. La rareza de esta coincidencia en un mismo paciente, se pone de manifiesto en una breve revision de la literatura.
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leigh s subacute necrotizing encephalomyelo pathy in a child with infantile spasms and Hypsarrhythmia
Developmental Medicine & Child Neurology, 2008Co-Authors: Shigehiko Kamoshita, Ikuko Mizutani, Yukio FukuyamaAbstract:SUMMARY The pathological examination of the brain of a 7-month-old boy with infantile spasms and Hypsarrhythmia revealed a lesion characteristic of Leigh's subacute necrotizing encephalomyelopathy. The rarity of these two conditions in one patient is indicated in the brief review of the literature. RESUME Encephalomyelopathie necrosante subaigue de Leigh L'examen anatomique du cerveau d'un garcon de 7 mois ayant presente des spasms infantiles et une hypsarythmie a montre des lesions caracteristiques de l'encephalomyelopathie necrosante subaigue de Leigh. La rarete de cette association chez le meme sujet est soulignee par une breve revue de la litterature. ZUSAMMENFASSUNG Leigh's Subakute Nekrotisierende Encephalomyelopathie bei einem Kind mit infantilen Krampfen und Hypsarrhythmie Die pathologische Untersuchung des Gehirns eines 7 Monate alten Jungen mit infantilen Krampfen und Hypsarrhythmie ergab eine fur die Subakute Nekrotisierende Encephalomyelopathie nach Leigh typische Schadigung. Auf die Seltenheit dieser beiden Symptome bei einem Patienten wird in einem kurzen Uberblick uber die Literatur hingewiesen. RESUMEN Encefalomielopatia necrotizante subaguda de Leigh en un nino con espasmos infantiles e hipsarritmia El examen anatomo-patologico de un nino de 7 meses de edad con espasmos infantiles e hipsarritmia revelo una lesion caracteristica de encefalomielopatia necrotizante subaguda de Leigh. La rareza de esta coincidencia en un mismo paciente, se pone de manifiesto en una breve revision de la literatura.
Shigehiko Kamoshita - One of the best experts on this subject based on the ideXlab platform.
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Leigh's Subacute Necrotizing Encephalomyelo‐pathy in a Child with Infantile Spasms and Hypsarrhythmia
Developmental Medicine & Child Neurology, 2008Co-Authors: Shigehiko Kamoshita, Ikuko Mizutani, Yukio FukuyamaAbstract:SUMMARY The pathological examination of the brain of a 7-month-old boy with infantile spasms and Hypsarrhythmia revealed a lesion characteristic of Leigh's subacute necrotizing encephalomyelopathy. The rarity of these two conditions in one patient is indicated in the brief review of the literature. RESUME Encephalomyelopathie necrosante subaigue de Leigh L'examen anatomique du cerveau d'un garcon de 7 mois ayant presente des spasms infantiles et une hypsarythmie a montre des lesions caracteristiques de l'encephalomyelopathie necrosante subaigue de Leigh. La rarete de cette association chez le meme sujet est soulignee par une breve revue de la litterature. ZUSAMMENFASSUNG Leigh's Subakute Nekrotisierende Encephalomyelopathie bei einem Kind mit infantilen Krampfen und Hypsarrhythmie Die pathologische Untersuchung des Gehirns eines 7 Monate alten Jungen mit infantilen Krampfen und Hypsarrhythmie ergab eine fur die Subakute Nekrotisierende Encephalomyelopathie nach Leigh typische Schadigung. Auf die Seltenheit dieser beiden Symptome bei einem Patienten wird in einem kurzen Uberblick uber die Literatur hingewiesen. RESUMEN Encefalomielopatia necrotizante subaguda de Leigh en un nino con espasmos infantiles e hipsarritmia El examen anatomo-patologico de un nino de 7 meses de edad con espasmos infantiles e hipsarritmia revelo una lesion caracteristica de encefalomielopatia necrotizante subaguda de Leigh. La rareza de esta coincidencia en un mismo paciente, se pone de manifiesto en una breve revision de la literatura.
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leigh s subacute necrotizing encephalomyelo pathy in a child with infantile spasms and Hypsarrhythmia
Developmental Medicine & Child Neurology, 2008Co-Authors: Shigehiko Kamoshita, Ikuko Mizutani, Yukio FukuyamaAbstract:SUMMARY The pathological examination of the brain of a 7-month-old boy with infantile spasms and Hypsarrhythmia revealed a lesion characteristic of Leigh's subacute necrotizing encephalomyelopathy. The rarity of these two conditions in one patient is indicated in the brief review of the literature. RESUME Encephalomyelopathie necrosante subaigue de Leigh L'examen anatomique du cerveau d'un garcon de 7 mois ayant presente des spasms infantiles et une hypsarythmie a montre des lesions caracteristiques de l'encephalomyelopathie necrosante subaigue de Leigh. La rarete de cette association chez le meme sujet est soulignee par une breve revue de la litterature. ZUSAMMENFASSUNG Leigh's Subakute Nekrotisierende Encephalomyelopathie bei einem Kind mit infantilen Krampfen und Hypsarrhythmie Die pathologische Untersuchung des Gehirns eines 7 Monate alten Jungen mit infantilen Krampfen und Hypsarrhythmie ergab eine fur die Subakute Nekrotisierende Encephalomyelopathie nach Leigh typische Schadigung. Auf die Seltenheit dieser beiden Symptome bei einem Patienten wird in einem kurzen Uberblick uber die Literatur hingewiesen. RESUMEN Encefalomielopatia necrotizante subaguda de Leigh en un nino con espasmos infantiles e hipsarritmia El examen anatomo-patologico de un nino de 7 meses de edad con espasmos infantiles e hipsarritmia revelo una lesion caracteristica de encefalomielopatia necrotizante subaguda de Leigh. La rareza de esta coincidencia en un mismo paciente, se pone de manifiesto en una breve revision de la literatura.
Shaun A. Hussain - One of the best experts on this subject based on the ideXlab platform.
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Hypsarrhythmia is associated with widespread, asymmetric cerebral hypermetabolism.
Seizure-european Journal of Epilepsy, 2019Co-Authors: Julius Weng, Regina Ahn, Shaun A. HussainAbstract:Abstract Purpose Hypsarrhythmia is the interictal EEG pattern most often associated with infantile spasms. We set out to evaluate the metabolic impact of Hypsarrhythmia among patients with infantile spasms by contrasting regional cerebral metabolic activity among children with and without Hypsarrhythmia. Methods Patients with video-EEG confirmed infantile spasms who underwent simultaneous interictal EEG and FDG-PET as part of a surgical evaluation were retrospectively identified. Pons-normalized relative cerebral metabolic activity (RCA) was ascertained in 18 cortical and 6 subcortical pre-specified regions of interest (ROIs). Results We identified 63 patients with infantile spasms who underwent simultaneous EEG/PET, including children with Hypsarrhythmia (n = 9), high-voltage EEG background (n = 20), and multifocal independent spike discharges (MISD) (n = 34). Among them, a putative epileptogenic zone was identified within the left-hemisphere only (n = 27), right-hemisphere only (n = 20), or assumed to be bilateral (n = 16). After adjustment for age at PET, the presence of Hypsarrhythmia was associated with hypermetabolism in 11 of 18 cortical ROI’s. After adjustment for lateralized epileptogenic zones, the association between Hypsarrhythmia and hypermetabolism was generally stronger within the left hemisphere. Conclusion Hypsarrhythmia is associated with widespread—and curiously left more than right—elevations in pons-normalized RCA, which is not evident on routine clinical review of individual PET studies. This study suggests that Hypsarrhythmia may be a quasi-ictal phenomenon based on widespread and usually bilateral cortical hypermetabolism.
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Quantitative Characteristics of Hypsarrhythmia in Infantile Spasms
2018 40th Annual International Conference of the IEEE Engineering in Medicine and Biology Society (EMBC), 2018Co-Authors: Rachel J. Smith, Shaun A. Hussain, Daniel W. Shrey, Beth A. LopourAbstract:Infantile spasms is a type of epilepsy characterized by clinical seizures termed “spasms” and often an electroencephalographic (EEG) pattern known as Hypsarrhythmia. Multiple studies have shown that the interrater reliability for human visual recognition of Hypsarrhythmia is poor. Quantitative measurements of this EEG pattern would provide objective basis for identification; however, the basic temporal and spectral characteristics of Hypsarrhythmia have never been assessed. Thus, we measured EEG amplitude and power spectra in 21 infantile spasms patients before and after treatment, as well as 21 control subjects. The Hypsarrhythmia EEG pattern was associated with (1) high broadband amplitude, especially in frontal and central brain regions, (2) high median power in the delta and alpha frequency bands, and (3) low spectral edge frequency. Our results indicate that Hypsarrhythmia can be quantitatively distinguished from data without Hypsarrhythmia. Introduction of these quantitative measures into clinical practice may increase diagnostic accuracy, expediting proper treatment and improving outcomes.
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EMBC - Quantitative Characteristics of Hypsarrhythmia in Infantile Spasms
Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual Inte, 2018Co-Authors: Rachel J. Smith, Shaun A. Hussain, Daniel W. Shrey, Beth A. LopourAbstract:Infantile spasms is a type of epilepsy characterized by clinical seizures termed “spasms” and often an electroencephalographic (EEG) pattern known as Hypsarrhythmia. Multiple studies have shown that the interrater reliability for human visual recognition of Hypsarrhythmia is poor. Quantitative measurements of this EEG pattern would provide objective basis for identification; however, the basic temporal and spectral characteristics of Hypsarrhythmia have never been assessed. Thus, we measured EEG amplitude and power spectra in 21 infantile spasms patients before and after treatment, as well as 21 control subjects. The Hypsarrhythmia EEG pattern was associated with (1) high broadband amplitude, especially in frontal and central brain regions, (2) high median power in the delta and alpha frequency bands, and (3) low spectral edge frequency. Our results indicate that Hypsarrhythmia can be quantitatively distinguished from data without Hypsarrhythmia. Introduction of these quantitative measures into clinical practice may increase diagnostic accuracy, expediting proper treatment and improving outcomes.
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The impact of Hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium
Epilepsia, 2017Co-Authors: Scott Demarest, Shaun A. Hussain, Anup D. Patel, Renée A. Shellhaas, William D. Gaillard, Cynthia Keator, Katherine C. Nickels, Tobias Loddenkemper, Russell P. Saneto, Elaine C. WirrellAbstract:SummaryObjective The multicenter National Infantile Spasms Consortium prospective cohort was used to compare outcomes and phenotypic features of patients with infantile spasms with and without Hypsarrhythmia. Methods Patients aged 2 months to 2 years were enrolled prospectively with new-onset infantile spasms. Treatment choice and categorization of Hypsarrhythmia were determined clinically at each site. Response to therapy was defined as resolution of clinical spasms (and Hypsarrhythmia if present) without relapse 3 months after initiation. Results Eighty-two percent of patients had Hypsarrhythmia, but this was not associated with gender, mean age, preexisting developmental delay or epilepsy, etiology, or response to first-line therapy. Infants with Hypsarrhythmia were more likely to receive standard treatment (adrenocorticotropic hormone, prednisolone, or vigabatrin [odds ratio (OR) 2.6, 95% confidence interval (CI) 1.4–4.7] and preexisting epilepsy reduced the likelihood of standard treatment (OR 3.2, 95% CI 1.9–5.4). Hypsarrhythmia was not a determinant of response to treatment. A logistic regression model demonstrated that later age of onset (OR 1.09 per month, 95% CI 1.03–1.15) and absence of preexisting epilepsy (OR 1.7, 95% CI 1.06–2.81) had a small impact on the likelihood of responding to the first-line treatment. However, receiving standard first-line treatment increased the likelihood of responding dramatically: vigabatrin (OR 5.2 ,95% CI 2–13.7), prednisolone (OR 8, 95% CI 3.1–20.6), and adrenocorticotropic hormone (ACTH; OR 10.2, 95% CI 4.1–25.8) . Significance First-line treatment with standard therapy was by far the most important variable in determining likelihood of response to treatment of infantile spasms with or without Hypsarrhythmia.
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Long-Range Temporal Correlations Reflect Treatment Response in the Electroencephalogram of Patients with Infantile Spasms.
Brain Topography, 2017Co-Authors: Rachel J. Smith, Shaun A. Hussain, Daniel W. Shrey, Amanda Sugijoto, Neggy Rismanchi, Beth A. LopourAbstract:Infantile spasms syndrome is an epileptic encephalopathy in which prompt diagnosis and treatment initiation are critical to therapeutic response. Diagnosis of the disease heavily depends on the identification of characteristic electroencephalographic (EEG) patterns, including Hypsarrhythmia. However, visual assessment of the presence and characteristics of Hypsarrhythmia is challenging because multiple variants of the pattern exist, leading to poor inter-rater reliability. We investigated whether a quantitative measurement of the control of neural synchrony in the EEGs of infantile spasms patients could be used to reliably distinguish the presence of Hypsarrhythmia and indicate successful treatment outcomes. We used autocorrelation and Detrended Fluctuation Analysis (DFA) to measure the strength of long-range temporal correlations in 21 infantile spasms patients before and after treatment and 21 control subjects. The strength of long-range temporal correlations was significantly lower in patients with Hypsarrhythmia than control patients, indicating decreased control of neural synchrony. There was no difference between patients without Hypsarrhythmia and control patients. Further, the presence of Hypsarrhythmia could be classified based on the DFA exponent and intercept with 92% accuracy using a support vector machine. Successful treatment was marked by a larger increase in the DFA exponent compared to those in which spasms persisted. These results suggest that the strength of long-range temporal correlations is a marker of pathological cortical activity that correlates with treatment response. Combined with current clinical measures, this quantitative tool has the potential to aid objective identification of Hypsarrhythmia and assessment of treatment efficacy to inform clinical decision-making.
John R. Mytinger - One of the best experts on this subject based on the ideXlab platform.
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Improving the inter-rater agreement of Hypsarrhythmia using a simplified EEG grading scale for children with infantile spasms.
Epilepsy Research, 2015Co-Authors: John R. Mytinger, John Millichap, Shaun A. Hussain, Monica P. Islam, Anup D. Patel, Nicole Ryan, Jaime Dawn E. Twanow, Geoffrey L. HeyerAbstract:Abstract Background There is poor inter-rater agreement in determining the presence or absence of Hypsarrhythmia among patients with infantile spasms. Yet, remission of Hypsarrhythmia has been used as a clinical and research outcome measure. Two important features of Hypsarrhythmia are the burden of epileptiform discharges and the amplitudes of background slow waves. We hypothesized that an electroencephalogram (EEG) grading scale emphasizing epileptiform discharge burden and the amplitudes of background slow waves would improve inter-rater agreement in interpreting Hypsarrhythmia. Our aim was to assess inter-rater agreement of Hypsarrhythmia using a novel and simplified EEG grading scale called the ‘BASED' (Burden of Amplitudes and Epileptiform Discharges) score and compare this to the traditional method of EEG analysis. Methods Twenty patients with infantile spasms were prospectively evaluated and electroclinical outcomes were determined. Forty EEG clips (20 pre-treatment and 20 post-treatment), representing the most severely abnormal five minute sleep epoch of each study, were assessed by three reviewers blinded to treatment and clinical outcome. Fleiss' kappa ( К ) was used to assess the inter-rater agreement in the interpretation of Hypsarrhythmia when using the BASED score compared to the traditional method of EEG analysis. Results Reviewers had favorable inter-rater agreement using the BASED score in interpreting Hypsarrhythmia ( К : 0.87) compared to when using the traditional method of EEG analysis to interpret Hypsarrhythmia ( К : 0.09). The three reviewers all agreed on the presence or absence of Hypsarrhythmia in 37/40 (93%) epochs using the BASED score but in only 15/40 (38%) epochs using the traditional method of EEG analysis, p = Conclusion When compared to the traditional method of EEG analysis, the BASED score allowed for better inter-rater agreement in the interpretation of Hypsarrhythmia. Future infantile spasms clinical trials must better define criteria for Hypsarrhythmia.
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Hypsarrhythmia assessment exhibits poor interrater reliability a threat to clinical trial validity
Epilepsia, 2015Co-Authors: Shaun A. Hussain, John Millichap, John R. Mytinger, Nicole Ryan, Grace Kwong, Joyce H Matsumoto, Joyce Y Wu, Jason T Lerner, Raman SankarAbstract:Summary Objective Hypsarrhythmia is the classic interictal electroencephalographic pattern associated with infantile spasms, and characterized by high voltage, disorganization, and multifocal independent epileptiform discharges. Given this seemingly simple definition, one might expect excellent interrater reliability (IRR) in the identification of this pattern. Alternatively, it may be argued that assessments of voltage and disorganization are fairly subjective, and thus quite challenging in borderline cases. We sought to test the IRR of Hypsarrhythmia assessment in a systematic fashion. Methods Six blinded pediatric electroencephalographers from four centers reviewed 22 electroencephalography (EEG) samples from patients with infantile spasms. Each sample was 5 min in duration and included only wakefulness. Raters determined if each EEG was abnormal and if Hypsarrhythmia was present/absent, and characterized relevant features: voltage, organization, epileptiform discharges, slowing, interictal attenuations, symmetry, and synchrony. In addition, raters indicated their level of confidence for each assessment. Multirater kappa statistics (κ) were calculated for the assessment of Hypsarrhythmia and each feature. Results Although IRR was favorable in determining whether a study was normal or abnormal (κ = 0.89), reliability was unfavorable for assessment of Hypsarrhythmia (κ = 0.40), modified Hypsarrhythmia (κ = 0.47), high voltage (κ = 0.37), disorganization (κ = 0.22), multifocal epileptiform discharges (κ = 0.68), interictal voltage attenuations (κ = 0.21), slowing (κ = 0.20), asymmetry (κ = 0.26), and asynchrony (κ = 0.08). Despite generally unsatisfactory interrater agreement, raters consistently reported high confidence in assessments. Significance This study contradicts the view that Hypsarrhythmia assessment is straightforward. Even small variability in the identification of Hypsarrhythmia has potentially deleterious consequences for clinical care, as its presence or absence impacts decisions to pursue high-risk and high-cost therapies. These inconsistencies may similarly confound studies in which abolition of Hypsarrhythmia is an outcome measure. There is a great need for practical, reliable, and unbiased measures of Hypsarrhythmia.
Ikuko Mizutani - One of the best experts on this subject based on the ideXlab platform.
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Leigh's Subacute Necrotizing Encephalomyelo‐pathy in a Child with Infantile Spasms and Hypsarrhythmia
Developmental Medicine & Child Neurology, 2008Co-Authors: Shigehiko Kamoshita, Ikuko Mizutani, Yukio FukuyamaAbstract:SUMMARY The pathological examination of the brain of a 7-month-old boy with infantile spasms and Hypsarrhythmia revealed a lesion characteristic of Leigh's subacute necrotizing encephalomyelopathy. The rarity of these two conditions in one patient is indicated in the brief review of the literature. RESUME Encephalomyelopathie necrosante subaigue de Leigh L'examen anatomique du cerveau d'un garcon de 7 mois ayant presente des spasms infantiles et une hypsarythmie a montre des lesions caracteristiques de l'encephalomyelopathie necrosante subaigue de Leigh. La rarete de cette association chez le meme sujet est soulignee par une breve revue de la litterature. ZUSAMMENFASSUNG Leigh's Subakute Nekrotisierende Encephalomyelopathie bei einem Kind mit infantilen Krampfen und Hypsarrhythmie Die pathologische Untersuchung des Gehirns eines 7 Monate alten Jungen mit infantilen Krampfen und Hypsarrhythmie ergab eine fur die Subakute Nekrotisierende Encephalomyelopathie nach Leigh typische Schadigung. Auf die Seltenheit dieser beiden Symptome bei einem Patienten wird in einem kurzen Uberblick uber die Literatur hingewiesen. RESUMEN Encefalomielopatia necrotizante subaguda de Leigh en un nino con espasmos infantiles e hipsarritmia El examen anatomo-patologico de un nino de 7 meses de edad con espasmos infantiles e hipsarritmia revelo una lesion caracteristica de encefalomielopatia necrotizante subaguda de Leigh. La rareza de esta coincidencia en un mismo paciente, se pone de manifiesto en una breve revision de la literatura.
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leigh s subacute necrotizing encephalomyelo pathy in a child with infantile spasms and Hypsarrhythmia
Developmental Medicine & Child Neurology, 2008Co-Authors: Shigehiko Kamoshita, Ikuko Mizutani, Yukio FukuyamaAbstract:SUMMARY The pathological examination of the brain of a 7-month-old boy with infantile spasms and Hypsarrhythmia revealed a lesion characteristic of Leigh's subacute necrotizing encephalomyelopathy. The rarity of these two conditions in one patient is indicated in the brief review of the literature. RESUME Encephalomyelopathie necrosante subaigue de Leigh L'examen anatomique du cerveau d'un garcon de 7 mois ayant presente des spasms infantiles et une hypsarythmie a montre des lesions caracteristiques de l'encephalomyelopathie necrosante subaigue de Leigh. La rarete de cette association chez le meme sujet est soulignee par une breve revue de la litterature. ZUSAMMENFASSUNG Leigh's Subakute Nekrotisierende Encephalomyelopathie bei einem Kind mit infantilen Krampfen und Hypsarrhythmie Die pathologische Untersuchung des Gehirns eines 7 Monate alten Jungen mit infantilen Krampfen und Hypsarrhythmie ergab eine fur die Subakute Nekrotisierende Encephalomyelopathie nach Leigh typische Schadigung. Auf die Seltenheit dieser beiden Symptome bei einem Patienten wird in einem kurzen Uberblick uber die Literatur hingewiesen. RESUMEN Encefalomielopatia necrotizante subaguda de Leigh en un nino con espasmos infantiles e hipsarritmia El examen anatomo-patologico de un nino de 7 meses de edad con espasmos infantiles e hipsarritmia revelo una lesion caracteristica de encefalomielopatia necrotizante subaguda de Leigh. La rareza de esta coincidencia en un mismo paciente, se pone de manifiesto en una breve revision de la literatura.