Juvenile primary lateral sclerosis

Definition:

Juvenile primary lateral sclerosis (JPLS) is a very rare progressive paralytic disorder characterized by increasing weakness and stiffness of muscles in the arms, legs and face. Although primary lateral sclerosis is a sporadic disorder of adult middle age, this disease is also observed in children as JPLS. JPLS arises from dysfunction of the upper motoneurons and the causative gene is ALS2.

References:

[1]. Chris Panzeri, et al. The first ALS2 missense mutation associated with JPLS reveals new aspects of alsin biological function. Brain. 2006 Jul;129(Pt 7):1710-9. [Content Brief]

[2]. G G Gascon, et al. Familial childhood primary lateral sclerosis with associated gaze paresis. Neuropediatrics. 1995 Dec;26(6):313-9. [Content Brief]

[3]. M L Grunnet, et al. Primary lateral sclerosis in a child. Neurology. 1989 Nov;39(11):1530-2. [Content Brief]

[4]. Shinji Hadano, et al. Molecular and cellular function of ALS2/alsin: implication of membrane dynamics in neuronal development and degeneration. Neurochem Int. 2007 Jul-Sep;51(2-4):74-84. [Content Brief]

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