Treatment

Symptomatic Therapies

Current, non-specific treatments for NPC focus mainly on supportive care, aimed toward managing the symptoms of the disease. As such, these treatments have no effect on disease progression or long-term outcomes. Common problems and relevant supportive treatments are listed in Table 5.

Table 5. General symptomatic therapies provided in supportive care
Symptom Treatment
Feeding problems Most children with NPC eventually require gastrostomy feeding, with monitoring in case of
aspiration or nutritional compromise
Cataplexy Tricyclic antidepressants or CNS stimulants can be effective in controlling cataplexy
Dystonia and tremor Some patients respond to anti-cholinergic drugs
Movement restriction Physical therapy is indicated to maintain mobility as long as possible
Seizures Anti-epileptic drugs can control or diminish the frequency of seizures
Sleep disorders Melatonin or a nocturnal sedative may be indicated. In complex cases, formal evaluation by a
sleep specialist should be considered
Constipation Patients should have a regular bowel program to prevent severe constipation
Lung involvement Chest physical therapy with aggressive bronchodilation appears beneficial. Antibiotic therapy
should be provided in case of intercurrent pulmonary infection

© 2007 Blackwell Publishing Limited. Reproduced by permission.

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