Neurogenic diabetes insipidus can be a failure of production at the hypothalamus, or a failure of release at the pituitary. Genetic
The most rare form of central DI is familial neurogenic diabetes insipidus. This form of DI is due to an inherited mutation of the arginine vasopressin-neurophysin II (AVP-NPII) gene, inherited in an autosomal dominant manner. At one point, only 45 families worldwide were known to possess this genetic trait. It is now more widely recognized, although the precise number of people affected with this form of DI is unknown at the present time.There is also an X-linked familial form.Wolfram Syndrome (also called DIDMOAD) is characterised by DI, diabetes mellitus, sensorineural deafness, and optic atrophy. Diagnosis
In many respects, the diagnosis of central diabetes insipidus begins as a diagnosis of exclusion. Specifically, other more common causes of polyuria and polydipsia are ruled out. Common rule outs include: diabetes mellitus, chronic kidney disease, hypokalemia, hypercalcemia, and psychogenic polydipsia. Once these conditions have been ruled out a water deprivation test is employed to confirm the diagnosis of CDI. Treatment
The disorder is treated with vasopressin analogs such as desmopressin. Nonetheless, many times desmopressin alone is not enough to bring under control all the symptoms, and another intervention must be implemented. See also
Fluid deprivation test

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History
Ataxia as a symptom was first described by French neurologist Duchenne de Boulogne in a subject with tabes dorsalis. By the late 19th and early 20th centuries, extensive research into the characterization, cause, and diagnostics of hereditary cerebellar ataxias was underway with the work of several prominent neurologists, including Jean-Martin Charcot, Pierre Marie, Nikolaus Friedreich, Adolph Strümpell, and others. Marie described a number of cases of hereditary, adult onset disease he thought to be clinically distinct from Friedreichs ataxia, spastic paraplegia, and other known types of ataxia, calling the syndrome hereditary cerebellar ataxia, though it became known Maries ataxia.While the hereditary patterns were clearly distinct, there was on-going debate well into the 1940s over whether Maries ataxia was really distinct from Freidreichs ataxia and Strümpells paraplegia and if these categorys themselves represented a single disease or many. This was because of the heterogenous nature of hereditary ataxias, the similarity of symptoms, and the lack of understood biochemical mechanisms. Further frustration with the ambiguity of terms introduced by Marie and Friedreich resulted in the creation of other systems to classify ataxias. Gordon Morgan Holmes and Godwin Greenfield each developed systems of categorizing ataxias, giving rise to categories called olivopontocerebellar atrophy and spinocerebellar degradation, though little consensus between the systems was achieved, and many terms are used interchangeably.In the depression era United States, the Schut family in Minnesota was one family known to carry a hereditary ataxia.