Benefit with methylprednisolone in continuous pulsetherapy in progressive primary form of multiple sclerosis: study of 11 cases in 11 years

Abstract

-Primary progressive multiple sclerosis (PPMS) is defined clinically with a progressive course from onset. There is no approved treatment for the PPMS. Methylprednisolone IV (MP) hastens the recovery from MS relapses. We studied 11 patients that met the MacDonald's diagnostic criteria for PPMS. The dose of MP was 30 mg/kg in 250 mL of glucose solution in three consecutive days during the first week, two doses during the second and one dose in the third week. One weekly session for eight consecutive weeks was given. After, a oncea week/ eight-week interval was maintained. The medium EDSS before treatment was 6.2, and after 11.2 years of treatment, the EDSS was 4.9. Although we studied a small sample of PPMS we may conclude that therapy with IVMP prevents clinical worsening of MS in the majority of patients with improvement in EDSS scores.KEy WOrDS: progressive primary form, multiple sclerosis, methylprednisolone. Benefício do uso de pulsoterapia contínua com metilprednisolona na forma primária progressiva da esclerose múltipla: estudo de 11 casos em 11 anosResumo -A forma progressiva da esclerose múltipla (FPEM) é definida como progressiva desde o início. Não há tratamento eficaz para esta forma. A metilprednisolona por via endovenosa (MPEV) é usada para os surtos de exacerbação da EM. Estudamos 11 pacientes que preenchiam os critérios de MacDonald para FPMS. A dose inicial de MPEV foi de 30 mg/kg em 250 mL de soro glicosado por três dias consecutivos na primeira semana, duas doses na segunda e uma dose na terceira semana. Seguiu-se uma sessão semanal por oito semanas. Após manteve-se uma dose semanal a cada oito semanas. A média do EDDS foi 9,6 antes e 4,9 após 11,2 anos de tratamento. Embora tenhamos estudado número reduzido de casos, podemos dizer que o uso de MPEV impede a progressão da FPEM na maioria dos pacientes estudados com melhora do EDDS. Multiple sclerosis (MS) might be considered a disease with different clinical phenotypes rather than an entity encompassing several distinct diseases 1 and the primary progressive multiple sclerosis (PPMS) is defined clinically with a progressive course from onset with no distinct relapses 2 . A study with 1844 patients with MS, 58% had relapsing-remitting (rrMS), 27% had secondary progressive (SPMS), 6% progressive relapsing (PrMS) and 9% PPMS forms. Median age at onset of progressive phase was similar in SPMS and in PPMS cases from onset 1,2 . In another study the mean age at onset was 40.1 years in the PPMS type and 29.2 years in the rrMS. The initial symptoms were motor in 57%, cerebellar or brainstem in 24%; sensory in 15%; and optic neuropathy in 5% of the PPMS form 3,4 . The disability was similar in PPMS, PrMS and SPMS cases and probably the mechanisms of demyelinating was common in all forms of MS 4 . There is a consensus that intravenous methylprednisolone (IVMP) hastens the recovery from MS relapses [1][2][3] . Nowadays it is considered the standard treatment for relapses of MS [1][2][3]

Progressive Onset Multiple Sclerosis: Demographic, Clinical and Laboratory Characteristics of Patients With and Without Relapses in the Course

“…All the patients who received high dose methylprednisolone treatment in the past either for relapses or not described somewhat improvement. Some important clinical benefits have been observed with pulse steroid therapy alone or in conjunction with mitoxantrone or cyclophosphamide in cases with PPMS (27)(28)(29)(30).…”

Progressive primary form of multiple sclerosis: clinical and radiological improvement with methylprednisolone in continuous pulsetherapy in one case for 16 years

“…The EDDS 6 score was 6 (ability to walk with unilateral support no more than 100 m without rest). Treatment consists of continuous IVMP as we related in our protocol 4 . The patient agreed with this kind of treatment signing an informed consent.…”

“…dISCUSSIOn There is no approved treatment for the PPMS. Nevertheless, some important clinical benefits have been observed after treatment in progressive forms with highdose methylprednisolone alone 4,7,8 or with cyclophosphamide 9 . There is consensus that IVMP is considered the 5 .…”

“…Variability in the glucocorticoid receptor gene is a potential explanation for differences in glucocorticoids sensitivity and may influence the disease course 3 . We have shown the benefit of methylprednisolone in continuous pulse therapy (IVMP) in PPMS 4 . The improvement of the clinical course and radiological findings in one patient of our group with the PPMS form motivated us to relate this case.…”