Because ML I is classified as a sialidosis, it is sometimes referred to as sialidosis type II. A rarer form of sialidosis – sialidosis type 1– occurs in children and adolescents and is often referred to as the juvenile form of the disorder. Children usually begin to show symptoms during the second decade of life, and myoclonus and cherry-red macules are often the initial symptoms. Patients usually develop seizures and progressive deterioration of coordinated muscular and mental activities. Pathophysiology
The role of sialidase is to remove a particular form of sialic acid (a sugar molecule) from sugar-protein complexes (referred to as glycoproteins), which allows the cell to function properly. Because the enzyme is deficient, small chains containing the sugar-like material accumulate in neurons, bone marrow, and various cells that defend the body against infection. Diagnosis
The detection of high urinary sialyl oligosaccharides and the confirmation of lysosomal enzyme deficiency in leukocytes or cultured fibroblasts make the diagnosis. Management
Treatment of sialidosis is similar to the other progressive myoclonic epilepsy disorders, which consists of managing seizures and myoclonus along with palliative, supportive, and rehabilitative care. See also
Fucosidosis
Sialic acid
Neuraminidase
Mucolipidosis
Lysosomal storage disease

Notes
References
mucolipidoses at NINDS - article derived from detail sheet available here

External links

Sialidosis type 1 and 3 at NIHs Office of Rare Diseases
Sialidosis at NIHs Office of Rare Diseases
Side effects attributed to the atropine content (especially when taken in excess doses, or in children), include: flushing, dryness in many areas, urinary retention, insomnia, headache, anxiety, hyperthermia, and tachycardia. It is these side effects that make it undesirable for most patients to take higher amounts of the medicine. Dosing and administration
The recommended initial dosing is two tablets, and one tablet to be taken after each loose stool thereafter. The therapeutic dosage for Motofen should not exceed 8 tablets (8 milligrams of Difenoxin). There are currently no instructions indicating usage for irritable bowel syndrome (IBS) and Hyperhidrosis, although as stated above, it has been used successfully in treating symptoms of both disorders (which can accompany each other). There was a "Motofen half-strength" tablet, containing only 0.5 milligram of difenoxin, but it has since been discontinued. Only standard 1.0 milligram strength tablets are available by prescription. Availability, price, and supply
There is currently only one manufacturer of Motofen tablets: Valeant Pharmaceuticals. It acquired the drug from Amarin Pharmaceuticals in 2004. Valeants tablets are pentagonal shaped, impressed with a "V" on one side, and vertically scored on the other side. In addition, the number "0500" is impressed bisecting the score line perpendicularly. The aforementioned bisection renders "05" and "00" on the left and right side of the score, respectively. Amarins version were also pentagonal shaped, but impressed with an "A" on one side with "74" on the opposing.