ABSTRACT
A 2-year-old boy presented with an early form of benign partial epilepsy with centro-temporal spikes (BCERS) and a severe speech delay. Family video analysis revealed an early regression of babbling and stagnation since the age of 12 months. Complete recovery occurred with anti-epileptic treatment. The deficit corresponded to a transient speech apraxia attributed to an epileptic disconnection of networks coordinating speech articulation. This observation is, to the best of our knowledge, the first demonstration that delayed emergence of language can be due to an epileptic dysfunction interfering with prelinguistic skills and therefore mimicking a developmental delay.
Subject(s)
Epilepsy, Temporal Lobe/complications , Language Disorders/etiology , Speech Disorders/etiology , Child, Preschool , Electroencephalography , Epilepsy, Temporal Lobe/diagnosis , Humans , Language Disorders/diagnosis , Language Tests , Male , Neuropsychological Tests , Prospective Studies , Severity of Illness Index , Speech Disorders/diagnosisABSTRACT
We report on a three and a half year old child with episodic sinus bradycardia during habitual seizures and prolonged interictal discharges due to focal cortical dysplasia in the anterior 2/3 of the insula and the inferior frontal cortex. Seizure-induced bradycardia is rarely reported in children. Bradycardia is suspected to be related to sudden death, a rare complication of a chronic seizure disorder. Several well-documented cases in adult patients reveal a high incidence of temporal epilepsy, but MRI and PET studies in healthy subjects suggest a major role of the insular cortex, especially the right, in cardiac regulation. Our finding underlines the predominance of the right insula in cardiac control, which already seems to be present in children.
Subject(s)
Bradycardia/etiology , Brain Diseases/complications , Brain Diseases/pathology , Cerebral Cortex/pathology , Frontal Lobe/pathology , Bradycardia/diagnosis , Brain Diseases/physiopathology , Cerebral Cortex/physiopathology , Child, Preschool , Electrocardiography , Electroencephalography , Female , Frontal Lobe/physiopathology , Functional Laterality/physiology , Humans , Magnetic Resonance Imaging , Seizures/diagnosis , Seizures/etiologyABSTRACT
Benign familial nocturnal alternating hemiplegia of childhood refers to recurrent attacks of hemiplegia arising from sleep, described in young children without neurologic or mental impairment. It is probably migraine related. The authors report two unrelated patients with nocturnal attacks starting at 22 and 31 months, followed by daytime episodes in one. The authors confirm the benign course of this disorder. It is distinct from the classic malignant form of alternating hemiplegia of childhood.
Subject(s)
Hemiplegia/physiopathology , Sleep , Child , Child, Preschool , Follow-Up Studies , Humans , Male , PrognosisABSTRACT
We report clinical, neuroradiological and neuropathological findings of monozygotic twin sisters born at 30 weeks' gestation, with pontocerebellar hypoplasia (PCH) similar but not identical to type 2 PCH. They presented with hypertonia, jitteriness, spontaneous and provoked myoclonic jerks (hyperekplexia), apnoeic episodes, and progressive microcephaly. They died at 7 weeks of age from respiratory failure.
Subject(s)
Brain Diseases/diagnosis , Cerebellum/abnormalities , Diseases in Twins , Infant, Premature, Diseases , Pons/abnormalities , Brain Diseases/pathology , Cerebellum/pathology , Contracture/etiology , Diagnosis, Differential , Disease Progression , Female , Humans , Infant, Newborn , Magnetic Resonance Imaging , Microcephaly/etiology , Muscle Hypertonia/etiology , Myoclonus/etiology , Olivary Nucleus/pathology , Pons/pathology , Reflex, Abnormal , Twins, MonozygoticABSTRACT
A 15-year-old adolescent with pneumococcal meningitis and increased intracranial pressure presented clinical and neurophysiological evidence of the locked-in syndrome. MRI studies showed an area of infarction involving the ventral part of the medulla.
