scholarly journals Long-term treatment with subcutaneous immunoglobulin in multifocal motor neuropathy

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Luca Gentile ◽  
Massimo Russo ◽  
Carmelo Rodolico ◽  
Ilenia Arimatea ◽  
Giuseppe Vita ◽  
...  

AbstractMultifocal motor neuropathy (MMN) is a rare disease with a prevalence of less than 1 per 100,000 people. Intravenous immunoglobulin (IVIG) therapy, performed for a long-term period, has been demonstrated able to improve the clinical picture of MMN patients, ameliorating motor symptoms and/or preventing disease progression. Treatment with subcutaneous immunoglobulin (SCIg) has been shown to be as effective as IVIG. However, previously published data showed that follow-up of MMN patients in treatment with SCIg lasted no more than 56 months. We report herein the results of a long-term SCIg treatment follow up (up to 96 months) in a group of 8 MMN patients (6 M; 2F), previously stabilized with IVIG therapy. Clinical follow-up included the administration of Medical Research Council (MRC) sum-score, the Overall Neuropathy Limitation Scale (ONLS) and the Life Quality Index questionnaire (LQI) at baseline and then every 6 months. Once converted to SCIg, patients’ responsiveness was quite good. Strength and motor functions remained stable or even improved during this long-term follow-up with benefits on walking capability, resistance to physical efforts and ability in hand fine movements.

2021 ◽  
Vol 429 ◽  
pp. 117814
Author(s):  
Luca Gentile ◽  
Massimo Russo ◽  
Carmelo Rodolico ◽  
Ilenia Arimatea ◽  
Giuseppe Vita ◽  
...  

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012618
Author(s):  
Grayson Beecher ◽  
Shahar Shelly ◽  
P. James B. Dyck ◽  
Michelle L. Mauermann ◽  
Jennifer M Martinez-Thompson ◽  
...  

Objectives:To longitudinally investigate patients with multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, quantifying timing and location of sensory involvements in motor-onset patients, along with clinico-histopathological and electrophysiological findings to ascertain differences in patients with and without monoclonal gammopathy of uncertain significance (MGUS).Methods:Patients with MADSAM neuropathy seen at Mayo Clinic and tested for monoclonal gammopathy and ganglioside antibodies, were retrospectively reviewed (January 1st, 2007-December 31st, 2018).Results:Of 76 patients with MADSAM, 53% had pure motor, 16% pure sensory, 30% sensorimotor and 1% cranial nerve onsets. Motor-onset patients were initially diagnosed as multifocal motor neuropathy (MMN). MGUS occurred in 25% (89% IgM subtype), associating with ganglioside autoantibodies (p<0.001) and higher IgM titers (p<0.04). Median time to sensory involvements (confirmed by electrophysiology) in motor-onset patients was 18 months (range: 6-180). Compared to initial motor nerve involvements, subsequent sensory findings were within the same territory 35% (14/40), outside 20% (8/40), or both 45% (18/40). Brachial and lumbosacral plexus MRI was abnormal in 87% (34/39) and 84% (21/25), respectively, identifying hypertrophy and increased T2 signal predominantly in brachial plexus trunks (64%), divisions (69%), and cords (69%), and intrapelvic sciatic (64%) and femoral (44%) nerves. Proximal fascicular nerve biopsies (n=9) more frequently demonstrated onion-bulb pathology (p=0.001) and endoneurial inflammation (p=0.01) than distal biopsies (n=17). MRI and biopsy findings were similar amongst patient subgroups. Initial Inflammatory Neuropathy Cause and Treatment (INCAT) disability scores were higher in patients with MGUS relative to without (p=0.02). Long-term treatment responsiveness by INCAT score reduction ≥1 or motor Neuropathy Impairment Score (mNIS) >8 point reduction occurred in 75% (49/65) irrespective of MGUS or motor-onsets. Most required ongoing immunotherapy (86%). Patients with MGUS more commonly required dual-agent immunotherapy for stability (p=0.02).Discussion:Pure motor-onsets are the most common MADSAM presentation. Long-term follow-up, repeat electrophysiology and nerve pathology help distinguish motor-onset MADSAM from MMN. Better long-term immunotherapy responsiveness occurs in motor-onset MADSAM compared to MMN reports. Patients having MGUS commonly require dual immunotherapy.Classification of Evidence:This study provides Class II evidence that most clinical, electrophysiological, and histopathological findings were similar between patients with MADSAM with and without monoclonal gammopathy of unknown significance.


2012 ◽  
Vol 7 (2) ◽  
pp. 128 ◽  
Author(s):  
Leonard H van den Berg ◽  

Multifocal motor neuropathy (MMN) is a rare, purely motor neuropathy. It is a progressive disorder, most patients eventually developing severe fatigue and weakness in the arm muscles that severely impair daily functioning and quality of life. Unlike other motor neuropathies such as motor neurone disease, MMN is treatable with regular infusions of intravenous immunoglobulin (IVIg). Four double-blind, randomised, placebo-controlled studies have shown that in the short term, IVIg significantly improves muscle strength and disability in more than 70 % of patients. The 11 observational studies reviewed in this article confirm that long-term maintenance treatment with IVIg maintains clinical improvement compared to pre-treatment baseline in most patients. Infusions are generally well tolerated, but regular monitoring and re-evaluation of the IVIg maintenance regimen is essential, as most patients need progressive increases in dosage or reduced intervals between infusions to maintain their response to treatment. In the absence of accepted predictive markers, maintenance IVIg should be individualised, based on each patient’s initial response, disability and the interval between the first infusion and decline in muscle strength.


2013 ◽  
Author(s):  
Christina Marel ◽  
Maree Teesson ◽  
Shane Darke ◽  
Katherine Mills ◽  
Joanne Ross ◽  
...  

2020 ◽  
Author(s):  
Na Wu ◽  
Yuhong Chen ◽  
Yaping Yang ◽  
Xinghuai Sun

Abstract Background: To investigate the corneal biomechanical changes in primary open angle glaucoma (POAG) patients treated with long-term prostaglandin analogue (PGA). Methods: 111 newly diagnosed POAG patients, including 43 high tension glaucoma (HTG) and 68 normal tension glaucoma (NTG), were measured by Corvis ST to obtain intraocular pressure (IOP), central corneal thickness (CCT) and corneal biomechanical parameters at baseline and at each follow-up visit after initiation of PGA treatment. The follow-up measurements were analyzed by the generalized estimate equation model with an exchangeable correlation structure. Restricted cubic spline was employed to estimate the dose-response relation between follow-up time and corneal biomechanics.Results: The mean follow-up time was 10.3 ± 7.02 months. Deformation amplitude (β=-0.0015, P=0.016), the first applanation velocity (AV1, β=-0.0004, P=0.00058) decreased and the first applanation time (AT1, β=0.0089, P<0.000001) increased statistically significantly with PGA therapy over time after adjusting for age, gender, axial length, corneal curvature, IOP and CCT. In addition, AT1 was lower (7.2950 ± 0.2707 in NTG and 7.5889 ± 0.2873 in HTG, P=0.00011) and AV1 was greater (0.1478 ± 0.0187 in NTG and 0.1314 ± 0.0191 in HTG, P=0.00002) in NTG than in HTG after adjusting for confounding factors.Conclusions: Chronic use of PGA probably influences the corneal biomechanical properties directly, which is to make cornea less deformable. Besides, corneas in NTG tended to be more deformable compared to those in HTG with long-term treatment of PGA.


Author(s):  
Gennaro Ratti ◽  
Antonio Maglione ◽  
Emilia Biglietto ◽  
Cinzia Monda ◽  
Ciro Elettrico ◽  
...  

Long term treatment with ticagrelor 60 mg and low-dose aspirin are indicated after acute coronary syndrome (ACS). We retrospectively reviewed aggregate data of 187 patients (155 M and 38 F) (mean age 63.8±9 years) in follow up after ACS with at least one high risk condition (Multivessel disease, diabetes, GFR<60 mL/min, history of prior myocardial infarction, age >65 years) treated with ticagrelor 60 mg twice daily (after 90 mg twice daily for 12 months). The results were compared with findings (characteristics of the patients at baseline, outcomes, bleeding) of PEGASUS-TIMI 54 trial and Eu Label. The highrisk groups were represented as follows: multivessel disease 105 pts (82%), diabetes 63 pts (33%), GFR< 60 mL/min 27 pts (14%), history of prior MI 33 pts (17%), >65 year aged 85 pts (45%). Treatment was withdrawn in 7 patients: 3 cases showed atrial fibrillation and were placed on oral anticoagulant drugs, one developed intracranial bleeding, in three patients a temporary withdrawal was due to surgery (1 colon polyposis and 2 cases of bladder papilloma). Chest pain without myocardial infarction occurred in 16 patients (revascularization was required in 9 patients). Dyspnea was present in 15 patients, but was not a cause for discontinuation of therapy. Long term treatment with ticagrelor 60 mg twice daily plus aspirin 100 mg/day showed a favourable benefit/risk profile after ACS.  In this study all patients had been given ticagrelor 90 mg twice daily for 12 months and the 60 mg twice daily dosage was started immediately thereafter, unlike PEGASUS-TIMI 54 trial in which it was prescribed within a period ranging from 1 day to 1 year after discontinuation of the 90 mg dose. This makes our results more consistent with current clinical practice. However, a careful outpatient follow-up and constant counseling are mandatory to check out compliance to therapy and adverse side effects.


1998 ◽  
Vol 26 ◽  
pp. 103-110 ◽  
Author(s):  
D. Cucinotta ◽  
D. De Leo ◽  
L. Frattola ◽  
M. Trabucchi ◽  
M.G. Albizatti ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document