L'E.E.G. Dans Les Hyperglycinémies Sans Cétose

Misès, J; Moussalli-Salefranque, F.; Plouin, Perrine; Temam, G.; Saudubray, Jean Marie

doi:10.1016/s0370-4475(78)80125-7

Cited by 10 publications

(5 citation statements)

References 6 publications

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“…The earliest such tracing was seen in a 30-h-old infant who had become symptomatic at age 12 h (Dalla Bernardina et al, 1978,1979). In a recent survey of EEG tracings taken from 12 neonates with NKH before their 9th day of life, at least 11 had this burst-suppression pattern (Mises et al, 1978). Similar tracings were, however, also obtained in two of 13 newborns with leucinosis and in two of five newborns with methylmalonic acidaemia (Mises et al, 1977).…”

Section: Clinical Pic3vrementioning

confidence: 69%

“…While one waits for laboratory confirmation, the EEG may serve as an important diagnostic tool. A typical pattern of intermittent very low activity and of synchronous, high amplitude paroxysmal activity was described in t971 by Finnish authors (Sepp~il/iinen and Sim il~i, 1971) and subsequently seen by others (Mises et al, 1977(Mises et al, , 1978Datla Bernardina et al, 1978, 1979; Brun et af., 1979) including ourselves (Steinmann and Gitzelmann, 1979). It was termed 'burst-suppression' pattern.…”

Section: Clinical Pic3vrementioning

confidence: 74%

See 1 more Smart Citation

Clinical and therapeutic aspects of non‐ketotic hyperglycinaemia

Gitzelmann

Steinmann

1982

J of Inher Metab Disea

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Section: Clinical Pic3vrementioning

confidence: 69%

Section: Clinical Pic3vrementioning

confidence: 74%

Clinical and therapeutic aspects of non‐ketotic hyperglycinaemia

Gitzelmann

Steinmann

1982

J of Inher Metab Disea

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“…In 31 cases, at least some information on the EEG is available. A detail-ed description was given by Seppalainen and Simila (1971), Dalla Bernardina et al (1978) and Mises et al (1978). In all cases, the EEG is severely abnormal.…”

Section: Electroencephalographic Datamentioning

confidence: 94%

“…Further observations, both clinical (Ferdinand et al 1970, Leupold et al 1974, Rampini et al 1967, Rees et al 1972, von Scbreier et al 1964, Simila et al 1970, liter et al 1968) and biochemical (Ando et al 1968, Gerritsen et al 1965, Reploh et al 1973, Sass-Kortsak et al 1965, Trijbels et al 1974, Bachmann et al 1971, Baumgartner et al 1969, Rampini et al 1967, Seppalainen and Simila 1971, liter et al 1968, Levy et al 1972, Morel 1976, Perry et al 1975) have confirmed the specific character of nonketotic hyperglycinemia. The EEG features, although distinctive, have been less extensively studied (Seppalainen and Simila 1971, Dalla Bernardina et al 1977, Mises et al 1978.…”

Section: Infantile Spasms Myoclonia Neonatal Neurologic Disorder Metamentioning

confidence: 99%

Glycine Encephalopathy

Bernardina

Aicardi

Goutières³

et al. 1979

Neuropediatrics

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4 cases of nonketotic hyperglycinemia (glycine encephalopathy), one with autopsy, are presented and the literature on 61 cases is reviewed. Major clinical signs include early hypotonia, lethargy and erratic and massive myoclonias with respiratory distrubances, starting during the first days of life after a symptom-free interval. Early death is common. Survivors are severely retarded and exhibit various types of seizures including infantile spasms. The EEG pattern consists initially of periodical paroxysmal bursts on an almost flat tracing, evolving later into a hypsarrhythmic pattern. Spongiosis of the myelinated pathways is the main pathological finding. Elevated CSF glycine seems to be the essential determinant of the neurological disturbances and it is, therefore, suggested that the term glycine encephalopathy be used instead of non-ketotic hyperglycinemia. A classification of disorders associated with hyperglycinemia is proposed.

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“…Mises et al [21] reported that EEGs in NKH were so characteristic in the first 15 days that they permitted correct diagnosis in 50%. Thomson et al [22] stated that the excitatory effects of glycine might be the underlying mechanism for the high frequency of bursts in NKH.…”

Section: Discussionmentioning

confidence: 99%

Amplitude-Integrated Electroencephalography in Newborns with Inborn Errors of Metabolism

et al. 2012

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Background: The utility of amplitude-integrated electroencephalography (aEEG) monitoring has been established for patients with neonatal hypoxic-ischemic encephalopathy. Objective: To evaluate the role of aEEG in the diagnostic process and treatment of patients with encephalopathy due to inborn errors of metabolism. Methods: Cases collected through an international registry were divided into 5 groups of metabolic disorders. Common aEEG features were sought for each group. Results: In total, 21/30 (70%) cases had abnormal aEEG background patterns, 18/30 (60%) showed seizure activity. Patients with disorders of energy metabolism, hyperammonemia, and organic/amino acidopathies often showed marked aEEG depression with seizure activity. In contrast, aEEGs of patients with peroxisomal disorders did not show major background abnormalities but seizures were present in 5/6 subjects. We report two features of interest: firstly, two tracings displayed an unusual upward shift of the lower aEEG amplitude margin. Secondly, aEEGs of infants with non-ketotic hyperglycinemia showed a pattern we refer to as ‘high-frequency burst-suppression pattern’. Conclusions: aEEG in patients with inborn errors of metabolism frequently reveals abnormalities and assists clinicians in the clinical assessment, management and monitoring of these patients.

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L'E.E.G. Dans Les Hyperglycinémies Sans Cétose

Cited by 10 publications

References 6 publications

Clinical and therapeutic aspects of non‐ketotic hyperglycinaemia

Clinical and therapeutic aspects of non‐ketotic hyperglycinaemia

Glycine Encephalopathy

Amplitude-Integrated Electroencephalography in Newborns with Inborn Errors of Metabolism

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