Table 2 Non-immunosuppresive drugs used in the therapy of SS patients
Drug | Number of times used | Monotherapy as initial treatment | As initial treatment in combination with other drugs | As second or further-line treatment option | Improvement | No improvement | Change of treatment | Comments |
|---|---|---|---|---|---|---|---|---|
Dantrolene | 15 | 9 | 4 | 2 | 13 | 2 | 3 | Dantrolene improved muscular symptoms but no other manifestations. It was used as a first option in combination with corticosteroids in two cases [6, 46] and in another case with immunoglobulin therapy [47], although, subsequently, immunoglobulin therapy was replaced by corticosteroids. Although improvement was recorded in 13 patients, such improvement only lasted for a short time in one of them, which led to the change in treatment [32]. In another case, the treatment was changed to immunoglobulin therapy because dantrolene is not a radical treatment [3]. For one of the cases that did not respond adequately, a change of treatment was not recorded in the article [45]. |
Carbamacepine/Oxcarbacepine | 9 | 1 | 7 | 1 | 4 | 5 | 5 | In two cases carbamazepine was combined with corticosteroids [2, 41]. In five cases, the treatment was changed due to lack of effectiveness [9, 32, 40, 42, 70]. In one of them, it was necessary to use botulinum toxin to control masticatory spasms [32]. In another of these cases, authors stated that carbamazepine, phenobarbital, quinine sulfate, and chlorpromazine were tested during hospitalization and they were not effective for spasms [40]. In one case, carbamazepine was used with gabapentin without result [70]. |
Phenitoin | 7 | 0 | 4 | 3 | 3 | 4 | 1 | In the 3 SS patients in whom there was an improvement, phenitoin was the first option in combination with corticosteroids [5, 34, 35]. In another patient, it was combined with blaclofen and prednisone without response [33]. In one case, phenytoin was used as a second option and no satisfactory response was obtained. But after several treatment options, phenytoin was maintained combined with corticoids and mycophenolate [37]. In another case, phenytoin was used as second option treatment with carbamazepine, but it was not effective [32]. Averianov reported its use, but without effectiveness [36]. |
Baclofen | 3 | 1 | 2 | 0 | 0 | 3 | 3 | It has been used as a first option treatment only in one SS patient [50]. In another patient, multiple muscle relaxants and anticonvulsants were used without satisfactory results [42]. Baclofen was used in combination with phenytoin and prednisone in another patient [33]. In none of the cases the treatment had a good clinical response. |
Clotiapine and biperidene | 1 | 1 | 0 | 0 | 0 | 1 | 1 | It was not effective [51]. |
Clonazepam | 3 | 0 | 2 | 1 | 0 | 3 | 3 | Clonazepam was used in combination with carbamazepine without improvement [9] and the SS patient required changing treatment to dantrolene. Clonazepam was used in a patient adding it to dantrolene, but it was necessary to change treatment to phenytoin and carbamazepine [32]. In one patient clonazepam was used together with several other drugs such as dantrolene, carbamazepine or diazepam [42]. |
Tetrazepam | 1 | 0 | 1 | 0 | 1 | 0 | 0 | Tetrazepam in combination with carbamazepine improved spasms in a patient with SS [39]. |
Otilonium bromide | 1 | 0 | 1 | 0 | 1 | 0 | 0 | It was used in combination with carbamazepine with disappearance of spasms and diarrhea [38] |
Phenobarbital | 1 | 0 | 0 | 1 | 0 | 1 | 1 | Phenobarbital was used in a SS patient who was also receiving carbamazepine, quinine sulfate and chlorpromazine treatment. But it was not effective either [40]. |
Quinine sulfate | 1 | 0 | 0 | 1 | 0 | 1 | 1 | Quinine sulfate was used in a patient who was also receiving carbamazepine, phenobarbital and chlorpromazine treatment. But it was not effective either [40]. |
Chlorpromazine | 1 | 0 | 0 | 1 | 0 | 1 | 0 | Chlorpromazine was used in a patient who was also receiving carbamazepine, quinine sulfate and phenobarbital treatment. But it was not effective either [40]. |
Acetazolamide | 1 | 0 | 0 | 1 | 1 | 0 | 0 | Acetazolamide improved muscle symptoms in a patient [36]. It was used in this patient after thioridazine, haloperidol and phenytoin were unsuccessful. |
Neostigmine | 1 | 0 | 1 | 0 | 1 | 0 | 0 | Neostigmine was used in combination with traditional Chinese medicine in a patient with myasthenia. The authors stated that she remained stable after 8 months of follow-up [40]. |
Botulinum toxin | 3 | 0 | 1 | 2 | 3 | 0 | 1 | Botulinum toxin was used together with corticosteroids in a patient as a primary therapy. Treatment was changed by adding azathioprine [59]. In one case, botulinum toxin was added to the treatment to control masticatory spasms [47]. In another case, it was used after several previous treatments with dantrolene, diazepam, clonazepam, phenytoin and carbamazepine [32]. |
Amitriptilin | 1 | 0 | 1 | 0 | 1 | 0 | 0 | Amitriptilin was used in combination with corticosteroids as a maintenance therapy [61]. |
Thioridazine | 1 | 1 | 0 | 0 | 0 | 1 | 1 | [36] |
Haloperidol | 1 | 0 | 0 | 1 | 0 | 1 | 1 | [36] |
Diazepam | 2 | 1 | 0 | 1 | 1 | 1 | 1 | It was combined with several other treatments such as immunoglobulin therapy, cyclophosphamide and azatioprine [51]. Although this patient improved, it is difficult to attribute her improvement to diazepam. In another patient diazepam was used together with multiple muscle relaxants and anticonvulsants without satisfactory results [42]. |
Midazolam | 1 | 1 | 0 | 0 | 0 | 1 | 0 | The patient developed a neuroleptic malignant syndrome after the onset of iv midazolam and died [52]. |