Neurogenic arthrogryposis multiplex congenita: Clinical and MRI findings

Spectrum of Spinal Cord, Spinal Root, and Brain MRI Abnormalities in Congenital Zika Syndrome with and without Arthrogryposis

“…Arthrogryposis multiplex congenita, often known simply as arthrogryposis, is a syndrome characterized by joint contractures, present since birth, affecting Ն2 areas of the body. [15][16][17][18][19][20] These joint malformations can be attributed to different disorders, such as defects of uterine environment, disorders of connective tissues, muscular dystrophies, and other abnormalities or conditions that affect the central or peripheral nervous systems in at least one of the components of the motor pathways from the spinal cord to muscles. 16,18 Regardless of the cause, children affected by arthrogryposis have onset and severe weakness early in intrauterine life, with immobilization of joints at different developmental stages.…”

A Possible Case of Arthrogryposis Multiplex from Medieval Canterbury

“…6,7 Recent work has shown that the condition is related to degeneration of the anterior horn cells of the spinal cord. 8 Despite the early origin of the degeneration, 12± 14 weeks after gestation, 9 and a possible genetic linkage, 10 there is very little evidence for familial occurrence in the isolated form of arthrogryposis. The condition is not con®ned to humans, examples have been reported in lambs and calves.…”

Generalised spinal cord atrophy, Chiari-I malformation, and syringomyelia

“…Focal spinal cord atrophy may be due to Chiari malformation, previous CNS infection, trauma, radiotherapy, spinal surgery, immunological disorder, vascular abnormality or CNS tumour. 1 ± 3,6 ± 8 Generalised spinal cord atrophy is rare and may be due to Chiari malformation, luetic CNS infection, 9 and hereditary disorders like arthrogryposis multiplex congenita, 10 Chediak-Higashi syndrome, 5 adrenoleucodystrophy, 4 SjoÈ gren syndrome, 11 familial spastic paraparesis, 12 and hereditary motor and sensory neuropathy with pyramidal signs (HMSN-V). 6 Apart from these causes, generalised spinal cord atrophy may be attributed to a collapse of a formerly holocord syringomyelia, leading to reduction of the spinal cord Cord atrophy, Chiari malformation, and syrinx J Finsterer et al diameter, mimicking spinal cord atrophy.…”