Biomaterial Database

[The E.E.G. and E.M.G. findings in sudanophilic leucodystrophy (author's transl)]. 1978

M Rodiere, and M Georgescu, and J Alric, and D Brunel, and J Cadilhac

Four patients with infantile familial sudanophilic leucodystrophy confirmed histologically and biochemically were investigated by E.E.G. and E.M.G. The E.E.G. showed a severe, non-specific slow wave abnormality, of moderate voltage and disorganized in 2 cases, with some critical' tonic' discharge. E.M.G. study was normal in relaxed muscle; peripheral nerve stimulation, however, revealed marked slowing of motor conduction velocities favouring a segmental demyelinisation process, which was ultimately confirmed histologically. This study underlines the value of investigation of infantile encephalopathies.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008297	Male		Males
D009435	Synaptic Transmission	The communication from a NEURON to a target (neuron, muscle, or secretory cell) across a SYNAPSE. In chemical synaptic transmission, the presynaptic neuron releases a NEUROTRANSMITTER that diffuses across the synaptic cleft and binds to specific synaptic receptors, activating them. The activated receptors modulate specific ion channels and/or second-messenger systems in the postsynaptic cell. In electrical synaptic transmission, electrical signals are communicated as an ionic current flow across ELECTRICAL SYNAPSES.	Neural Transmission,Neurotransmission,Transmission, Neural,Transmission, Synaptic
D009469	Neuromuscular Junction	The synapse between a neuron and a muscle.	Myoneural Junction,Nerve-Muscle Preparation,Junction, Myoneural,Junction, Neuromuscular,Junctions, Myoneural,Junctions, Neuromuscular,Myoneural Junctions,Nerve Muscle Preparation,Nerve-Muscle Preparations,Neuromuscular Junctions,Preparation, Nerve-Muscle,Preparations, Nerve-Muscle
D010525	Peripheral Nerves	The nerves outside of the brain and spinal cord, including the autonomic, cranial, and spinal nerves. Peripheral nerves contain non-neuronal cells and connective tissue as well as axons. The connective tissue layers include, from the outside to the inside, the epineurium, the perineurium, and the endoneurium.	Endoneurium,Epineurium,Perineurium,Endoneuriums,Epineuriums,Nerve, Peripheral,Nerves, Peripheral,Perineuriums,Peripheral Nerve
D002540	Cerebral Cortex	The thin layer of GRAY MATTER on the surface of the CEREBRAL HEMISPHERES that develops from the TELENCEPHALON and folds into gyri and sulci. It reaches its highest development in humans and is responsible for intellectual faculties and higher mental functions.	Allocortex,Archipallium,Cortex Cerebri,Cortical Plate,Paleocortex,Periallocortex,Allocortices,Archipalliums,Cerebral Cortices,Cortex Cerebrus,Cortex, Cerebral,Cortical Plates,Paleocortices,Periallocortices,Plate, Cortical
D002549	Diffuse Cerebral Sclerosis of Schilder	A rare central nervous system demyelinating condition affecting children and young adults. Pathologic findings include a large, sharply defined, asymmetric focus of myelin destruction that may involve an entire lobe or cerebral hemisphere. The clinical course tends to be progressive and includes dementia, cortical blindness, cortical deafness, spastic hemiplegia, and pseudobulbar palsy. Concentric sclerosis of Balo is differentiated from diffuse cerebral sclerosis of Schilder by the pathologic finding of alternating bands of destruction and preservation of myelin in concentric rings. Alpers' Syndrome refers to a heterogeneous group of diseases that feature progressive cerebral deterioration and liver disease. (From Adams et al., Principles of Neurology, 6th ed, p914; Dev Neurosci 1991;13(4-5):267-73)	Alpers Syndrome,Balo Concentric Sclerosis,Cerebral Sclerosis, Diffuse,Encephalitis Periaxialis,Myelinoclastic Diffuse Sclerosis,Poliodystrophia Cerebri,Schilder Disease,Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis,Alpers Disease,Alpers Progressive Infantile Poliodystrophy,Alpers' Disease,Alpers' Syndrome,Alpers-Huttenlocher Syndrome,Balo's Concentric Sclerosis,Encephalitis Periaxialis Concentrica,Encephalitis Periaxialis Diffusa,Neuronal Degeneration Of Childhood With Liver Disease, Progressive,Progressive Neuronal Degeneration of Childhood with Liver Disease,Progressive Sclerosing Poliodystrophy,Schilder's Disease,Sudanophilic Cerebral Sclerosis,Alper Disease,Alper Syndrome,Alper's Disease,Alper's Syndrome,Alpers Huttenlocher Syndrome,Concentric Sclerosis, Balo,Concentric Sclerosis, Balo's,Diffuse Cerebral Scleroses,Diffuse Cerebral Sclerosis,Diffuse Scleroses, Myelinoclastic,Diffuse Sclerosis, Myelinoclastic,Disease, Alpers',Disease, Schilder,Disease, Schilder's,Myelinoclastic Diffuse Scleroses,Progressive Sclerosing Poliodystrophies,Schilders Disease,Scleroses, Balo's Concentric,Scleroses, Myelinoclastic Diffuse,Sclerosis, Diffuse Cerebral,Sclerosis, Myelinoclastic Diffuse,Syndrome, Alpers,Syndrome, Alpers-Huttenlocher
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D004569	Electroencephalography	Recording of electric currents developed in the brain by means of electrodes applied to the scalp, to the surface of the brain, or placed within the substance of the brain.	EEG,Electroencephalogram,Electroencephalograms