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Disorders of vitamin B6 metabolism
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
Almost all PNPO-deficient patients have a severe and acute early neonatal presentation with convulsions, myoclonus, rotatory eye movements, and sudden clonic contractions [4, 7]. It is important to note that many but not all patients are born prematurely between 22 and 35 weeks’ gestation. Fetal distress is common, as are “signs of asphyxia”. Most have had a low Apgar score and/or required intubation. Thus, PNPO deficiency must enter the differential diagnosis of hypoxic–ischemic encephalopathy (HIE) in a prematurely born infant. Seizures are resistant to conventional anticonvulsant therapy and can be fatal. EEG shows a burst-suppression pattern. There is often metabolic (lactic) acidosis as well as a tendency to hypoglycemia. There may be gastrointestinal problems such as abdominal distension, even ileus and vomiting. Very similar is the presentation of babies affected with the vitamin B6-dependent epilepsy caused by mutations in PROSC [6].
Neurological and neuromuscular disorders
Published in Rachel U Sidwell, Mike A Thomson, Concise Paediatrics, 2020
Rachel U Sidwell, Mike A Thomson
Burst suppression pattern. Isoelectric EEG with bursts of spikes and other activity. These findings indicate a hopeless prognosis for recovery in a child.
Special considerations: Alzheimer’s disease
Published in Hemanshu Prabhakar, Charu Mahajan, Indu Kapoor, Essentials of Geriatric Neuroanesthesia, 2019
Christopher G. Sinon, Sona Shah Arora, Amy D. Rodriguez, Paul S. García
Intraoperative “consciousness monitors” can help to simplify the use of EEG recording to guide perioperative anesthetic maintenance for patients with AD. As previously mentioned, anesthetic requirements are generally lower for geriatric patients, and this is especially true for patients with MCI or AD. Titrating anesthetic drugs to remain within the recommended index range for surgical anesthesia can reduce the likelihood of postoperative complications. Burst suppression has been linked to postoperative delirium (POD) and in some studies, persistent perioperative neurocognitive disorders (PPNDs) (see next section). Burst suppression varies in its detection by processed EEG monitors. Many devices simultaneously display the raw EEG waveform and may be of benefit beyond the dimensionless index derived from the EEG. It is reasonable to avoid certain medications associated with paroxysmal nocturnal dyspnea (PND) including benzodiazepines, long-acting narcotics, and known anticholinergics
Nonketotic Hyperglycinemia: Two Case Reports and Review
Published in The Neurodiagnostic Journal, 2019
Rajesh P. Poothrikovil, Khalid Al Thihli, Amna Al Futaisi, Fathiya Al Murshidi
First EEG was recorded on the ninth day of life. Background of this record showed excessive discontinuity of cerebral activity in the form of burst-suppression pattern. Intermittent high amplitude bursts of delta slowing (up to 400 µV) were noted intermixed with sharp waves on a low amplitude background. Inter-burst interval was varying with a maximum of up to 30 s (18–30 s). At times, these bursts show mild asymmetry and asynchrony (Figure 1). Plasma glycine lowered to almost half its previous levels with a mild improvement in GCS (7/15) after 24 h of sodium benzoate administration. Brain MRI done on the twelfth day showed agenesis of the corpus callosum (Figure 2). The patient was also started on an N-methyl-D-aspartate (NMDA) receptor antagonist and ketamine to reduce the cerebral cortex excitability. A second EEG was done on the thirteenth day which showed burst suppression pattern with paroxysmal high amplitude spike, polyspike and wave bursts on a low voltage suppression period. Compared to the previous record, the inter-burst interval was reduced from 18–30 s to about 10 s (Figure 3).
A novel VARS2 gene variant in a patient with epileptic encephalopathy
Published in Upsala Journal of Medical Sciences, 2019
Lucija Ruzman, Ivana Kolic, Jelena Radic Nisevic, Antonija Ruzic Barsic, Ingrid Skarpa Prpic, Igor Prpic
At the age of 6 months multiple seizure types (mastication, eyelid and lip twitching, myoclonic seizures of the trunk and extremities, tonic and clonic seizures) were noticed. Treatment attempts with several antiepileptic drugs including folic acid, cholecalciferol, pyridoxine and modified Atkins diet were not successful. EEG studies showed continuous burst–suppression pattern. All in all, the clinical status of the child developed into EE. Microcephaly, severe global hypotonia, failure to thrive, and feeding and breathing difficulties were more and more pronounced. Pendular nystagmus and oedema of hands and feet were periodically noticed. Echocardiography showed hypertrophic obstructive cardiomyopathy with fast progression, and atenolol treatment was initiated.
Successful treatment of severe, treatment resistant GHB withdrawal through thiopental-coma
Published in Substance Abuse, 2021
Cornelis F. Vos, Monica Pop-Purceleanu, Maarten J. W. van den Berg, Arnt F. A. Schellekens
Moreover, we used a thiopental-induced coma with EEG confirmed burst suppression. Burst suppression is an EEG pattern observed in inactivated brain states, such as deep sedation or coma.28 Drug-induced burst suppression is used for the treatment of refractory status epilepticus.28 After several unsuccessful attempts to taper sedatives, it was hypothesized that thiopental-induced coma therapy with burst suppression pattern on EEG might be effective in our patient. During the thiopental-induced coma, his EEG was continuously monitored to maintain burst suppression, and for early identification of toxicity (including isoelectric EEG).29