Table 3.
Overview on medication for palliative therapies in NCL.
| Symptom | Substance | Comment |
|---|---|---|
| Epilepsya | Valproate | Advantage: mood stabilizing effect, useful in juvenile NCL patients with psychotic symptoms |
| Lamotrigine | ||
| Topiramate | Increase dosage slowly to minimize side effects such as speech disturbance (starting dose 0.5 mg/kg/d). Agitation may be a side effect. In this case discontinue the drug. | |
| Levetiracetame | Severe agitation is a possible side effect in juvenile NLC. | |
| Diazepam, lorazepam | Acute therapy of prolonged grand mal seizures | |
| Myoclonus | Levetiracetame | Also effective as anti-epileptic medication (especially in late infantile NCL) |
| Zonisamide | ||
| Piracetame | High dosage required (300–350 mg/kg/d) | |
| Spasticity | Baclofen (1st choice) | Frequently high dosage required |
| Tizanidine (2nd choice) | Good effect also against dyskinesia | |
| Tetrahydrocannabinol | “Add-on” medication, increase dosage slowly up to 0.07 mg/kg/d, | |
| Botulinum toxin | Local application by injection to muscles; always accompanied by physical therapy |
a
Avoid overtreatment, as most seizures in NCL are treatment-resistant.