Infantile sclerosis

Vigabatrin is used as an adjunctive antiepileptic in patients with resistant partial epilepsy with or without secondary generalization, unresponsive to other therapy [2]. Nowadays, vigabatrin is rarely used in the treatment of partial seizures due to several irreversible visual field constrictions associated with its chronic use [57-62], It is regarded by many authorities as a drug of choice in infants with west syndrome (infantile spasms), particularly in cases associated with tuberous sclerosis [62],... 

Tuberous Sclerosis as an Underlying Basis for Infantile Spasm Raymond S. Yeung... 

Milner LS, Wei SH, Houser MT (1991) Amelioration of glomerular injury in doxorubicin hydrochloride nephrosis by dimethylthiourea. J Lab Clin Med 118 427 134 Milner LS, Wei S, Kazakoff P et al. (1994) Synergistic effect of fish oil diet and dimethylurea in acute adriamycin nephrosis. AM J Med Sci 308 266-270 Mizuno S, Mizuno-Horikawa Y, Yue BF et al. (1999) Nephrotic mice (ICGN strain) A model of diffuse mesangial sclerosis in infantile nephrotic syndrome. Am J Nephrol 19 73-82... 

Corticotropin, an anterior pituitary hormone, is indicated as a diagnostic test of adrenocortical function, as a replacement hormone, in the treatment of multiple sclerosis, in the treatment of severe allergic reactions, collagen disorders, dermatologic disorders, inflammation, and of infantile spasm. 

Spence SJ, Nakagawa J, Sankar R, Shields WD. Phenobarbital interferes with the efficacy of vigabatrin in treating infantile spaans in patients with tuberous sclerosis. Epilepsia (2000) 41 (Suppl 7), 189. 

A 6-month-old boy with infantile spasms and tuberous sclerosis complex was treated with vigabatrin for 1 month. A routine follow-up MRI demonstrated asymptomatic diffusion abnormalities in bilateral thalami. Similar abnormalities were also identified in globi pallidi and brainstem. 

Other authors, described a deficiency in about 1,5-2,0% of the biopsies. Half of these biopsies came from patients with a clinical symptomatology of exercise intolerance with muscular pain and/or fatigue. The other half came from patients displaying a wide diversity of partly well defined clinical entities, among others collagen vascular disease, neuropathy, amyotrophic lateral sclerosis, dystrophy, cardioskeletal myopathy, spinal muscular atrophy, paroxysmal myoglobinuria, facio-scapulo-humeral syndrome, infantile hypotonia and so on. Therefore some authors, consider the deficiency state as a normal variant, not associated with any particular clinical picture, others, however, as a cause of exercise intolerance... 

Subjects with the juvenile form were the first reported cases with degenerative diffuse sclerosis (Scholz 1925 Bielschowsky and Henneberg 1928 Norman 1947). Here, manifestations appear between six and nine years of age. Although in these cases the disease may last up to eight years, its course, in general, is not much longer than in the infantile form (Hollander and Pilz 1964). 

---