Early Psychomotor Development Research Articles

Self‐Induced Pattern‐Sensitive Epilepsy

Case Report: A 6‐year‐old girl developed self‐induced pattern‐sensitive epilepsy. Her elder sister is normal, and there is no family history of epilepsy. She was born after a 39–week gestation at a birth weight of 3,120 g. Her early psychomotor development was normal. She spoke her first meaningful words at 12 months and walked along at 18 months. At 6 years, she developed repetitive myoclonic jerks of the shoulder and arms, mainly on voluntary viewing of vertically striped patterns such as her mother's clothes, gates, and escalator steps. Neither spontaneous nor photosensitive seizures occurred. At 7 years, she first experienced a generalized tonic‐clonic seizure after seeing the mesh of a screen window. Physical and neurologic examinations were unremarkable. The interictal EEGs showed diffuse polyspike‐and‐wave complexes but no photosensitivity, and computed tomography on the head revealed no abnormalities. She was treated with valproate (VPA), which partially suppressed her seizures. After age 10 years, the seizures increased in frequency and occurred exclusively by self‐induction. She displayed an irresistible urge to approach the mesh of screen windows at home every morning and developed myoclonic jerks after staring at the pattern. Even when her parents scolded her not to approach the window and drew a curtain over it, she deliberately opened the curtain and glanced at the mesh, inducing the jerks. She did not seem to relieve any tension through having the seizures. At that age, EEGs showed an ictal generalized polyspike‐and‐wave complex with myoclonic jerks a few seconds after viewing the same mesh pattern as that from her house. Interictal recordings had normal background activities. The EEGs after viewing other patterns were not obtained. Visual evoked responses to flashes were normal. At age 11 years, she developed another generalized tonic‐clonic seizure after viewing a vertically striped pattern in a gate. After an increasing in the dose of VPA, her self‐induced seizures were controlled by the end of her 12th year. However, she had an obsessive interest in patterns until age 12 years. She demanded to view the vertical strips of a notebook, even if her mother prohibited viewing it and concealed the notebook. She did not have other behavioral problems, but her intelligence quotient was 71 on the Kyoto Scale of Psychological Development. Her visual memory was especially poor in the test. She is now 13 years old and still taking VPA. She is seizure free and shows no more obsessive behavior toward patterns. Conclusions: In this patient, only pattern‐sensitive seizures occurred, and only self‐induced seizures after age 10 years. The seizures were primarily myoclonic with rare generalized tonic‐clonic seizures. She also displayed obsessive behavior toward patterns. She did not have photosensitive seizures, and her EEGs showed no photosensitivity. This is not in accordance with the previous reports on the close relation between pattern‐sensitive and photosensitive epilepsy. Her low intelligence quotient was mostly caused by her poor visual memory. This suggests that pattern‐sensitive epilepsy is linked to focal dysfunction involving the occipital lobe.

Positron emission tomography in neuronal ceroid lipofuscinosis (Jansky-Bielschowsky disease): a case report

We report on a 13-year-old girl with late infantile neuronal ceroid lipofuscinosis (NCL) in whom PET scanning with [ 18F]-2-fluoro-2-deoxy- d-glucose ([ 18F]/FDG) was performed. Early psychomotor development was normal. At the age of 2 years, neurological signs such as hypotonia and incoordination appeared, followed by visual failure and ataxia. At the age of 4, funduscopic examination showed macular degeneration and papillary atrophy. At the age of 9, myoclonic jerks were observed; subsequently, generalized seizures together with failing vision, mental deterioration, and visual and auditory hallucinations appeared. Brain MRI showed severe cortical and subcortical atrophy. A skin biopsy detected the presence of ‘finger-print’ inclusions in the cytoplasm of smooth muscle fibers. Late infantile NCL (Jansky-Bielschowsky disease) was diagnosed. FDG/PET revealed a severe reduction of metabolism in all the cortical and subcortical structures. A regional analysis of the distribution of the tracer revealed marked bilateral hypometabolism, particularly in calcarine, lateral, occipital, and temporal cortices and in the thalamus.

Hypothalamic hamartomas and ictal laughter: evolution of a characteristic epileptic syndrome and diagnostic value of magnetic resonance imaging.

Detailed study of 4 patients and review of the literature allowed us to delineate further the epileptic syndrome associated with hypothalamic hamartomas, which characteristically begins in infancy with laughing seizures. Because early childhood psychomotor development is usually normal, the condition appears benign and may not even be recognized. The episodes of laughter are brief, frequent, and mechanical in nature. These features distinguish it from other forms of epileptic laughter, particularly that which occurs in temporal lobe epilepsy. Subsequently, the seizures become longer, other seizure types appear, and between the ages of 4 and 10 years, the clinical and electroencephalographic features of secondary generalized epilepsy develop. Cognitive deterioration occurs and severe behavior problems are frequent. Prognosis for seizure control and social adjustment is poor. Cortical abnormality occurs in association with the hypothalamic hamartoma. The lesions are best detected by magnetic resonance imaging but may be difficult to identify by computed tomographic scanning.

Effects of intrauterine growth and gestational age upon infants' early psychomotor development in Brazil.

In a sample of 245 Brazilian infants, the effects of intrauterine growth and gestational age upon psychomotor development were studied through ages 4 to 18 mo. Gestational age was more important than intrauterine growth in determining psychomotor development in the first 18 mo. of life. Intrauterine growth had a significant but smaller and more stable effect than gestational age upon psychomotor development at all ages. Maternal parity, interval between the birth of the child studied and the next oldest sibling, 5-min. Apgar score, and socioeconomic status were also relevant to psychomotor development at these early ages.

Towards a Theory of Knowledge Acquisition for Black Children

The purpose of this paper is twofold. Initially, the paper describes the impact of educational research and theory on the policies and practices implemented in elementary schools, and the potentially devastating effects such practices can have on the student clientele. It is argued that the perceptions of Black children as portrayed in educational theory have been misguided, based on the assumptions, theories, and research studies that have questionable historical premises. As a result of these theoretical misconceptions, schooling practices are inappropriate and stifle the intellectual growth and development of these children. The focus then shifts to the main thesis which describes the early and advanced psycho-motor development of Black children and the need for a socially interactive knowledge acquisition pattern compatible with their learning needs. The social interactive learning style in elementary curriculum theory formation is an important but relatively unexplored aspect of social science research.

Evolution clinique de la dystrophie neuro-axonale infantile à la lumière de la chronologie des atrophies systématisées sous-jacentes

The anatomo-clinical study of 4 cases of infantile neuroaxonal dystrophy enables us: 1. (1) to describe its clinical features: the disease starts usually at the end of the first year of life after a normal early psychomotor development; severe hypotonia, arrest of mental development followed by a rapidly progressive dementia, optic atrophy, progressive appearance of hypertonia with a pyramidal syndrome, bulbar signs, sphincter disturbances, death between the ages of 4 and 10 years; 2. (2) to stress the main histological features of the disorder: presence of spheroid bodies in the grey matter with a clear-cut predominance in the sensory bulbo-spinal relay nuclei, multiple system atrophies (optic and pyramidal pathways, cerebellar cortex, posterior columns), diffuse sclerosis of the white matter, accumulation of neutral fats in the striatum and the pallidum, histiocytic proliferation in the splenic Malpighian follicles; 3. (3) to confirm, through electron microscopy, the localization of the spheroids in the axon terminals and the presynaptic endings; 4. (4) to attempt a correlation between the clinical features and the evolution of the lesions in the central nervous system.