The general organization and structure of the nerve ring, the main mass of central nervous system neuropil, in the small soil nematode Caenorhabditis elegans is described. The nerve ring receives sensory input from the anterior tip of the animal by means of six nerve bundles, all nerve fibers of which have centrally located cell bodies. The anterior sensory structures are classically divided into two types, papillary and amphidial, and are assumed responsible for mechano-and chemoreception, respectively. Papillary fibers enter directly into the nerve ring, whereas amphidial fibers enter the ventral ganglion, a posterior extension of the nerve ring, in a circuitous manner which is not discussed in detail. Of those papillary fibers which project into the nerve ring neuropil, 22 end in easily characterized sensory structures whereas 14 terminate distally near sensory organs but have no function which can be deduced on the basis of comparative morphology. After entering the ring the fibers maintain their identity and do not anastamose with one another. Cell bodies of each papillary sensory neuron have been mapped around the nerve ring. The cephalic musculature is shown to consist of 32 muscle cells which form four longitudinal submedial groups of eight muscles each. Innervation of this musculature occurs wholly within the CNS by means of processes of the muscle cells which are sent centrally. The anterior 16 cephalic muscle cells are innervated by the ring only, in well delimited regions termed muscle plates. The posterior 16 are dually innervated by means of processes sent both to the nerve ring plates and to their nearest medial longtitudinal nerve cord. The nerve ring neuropil is characterized as having fibers containing one of four morphologically distinct vesicle types. Gap junction contacts are observed within the main neuropil involving one of these fiber types and within the muscle plate regions among muscle processes, which do not contain vesicles. An evolutionarily primitive sensory-motor synapse within the nerve ring is described from an identified sensory neuron onto an identified cephalic muscle cell process. Comparisons are made with the nervous system of Ascaris lumbricoides, the only other nematode to be extensively studied, to illustrate the conservativeness of the nemic nervous system.
We examined the phenotypic variation and clinical genetics in nine families with generalized epilepsy with febrile seizures plus (GEFS+). This genetic epilepsy syndrome with heterogeneous phenotypes was hitherto described in only one family. We obtained genealogical information on 799 individuals and conducted detailed evaluation of 272 individuals. Ninety‐one individuals had a history of seizures and 63 had epilepsy consistent with the GEFS+ syndrome. Epilepsy phenotypes were febrile seizures (FS) in 31, febrile seizures plus (FS+) in 15, FS+ with other seizure types (atonic, myoclonic, absence, or complex partial) in 8, and myoclonic–astatic epilepsy in 9 individuals. Inheritance was autosomal dominant with approximately 60% penetrance. This study confirms and expands the spectrum of GEFS+ and provides new insights into the phenotypic relationships and genetics of FS and the generalized epilepsies of childhood. Moreover, the ability to identify large families with this newly recognized common, childhood‐onset, generalized genetic epilepsy syndrome suggests that it should be a prime target for attempts to identify genes relevant to FS and generalized epilepsy. Ann Neurol 1999;45:75–81
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