A population‐based and cross‐sectional study of the long‐term prognosis in multifocal motor neuropathy

Abstract

A population‐based, cross‐sectional study of patients referred to the Danish hospital system between 1985 and 2006 was conducted to evaluate the long‐term outcome in Danish patients treated for multifocal motor neuropathy (MMN). Thirty‐four MMN patients were identified, three had died of unrelated diseases, 10 were excluded, one did not reply to study request and 20 were included. The median disease duration was 24 years (interquartile range: 18.5‐31.0). Compared to 24 healthy matched control subjects, the Rasch‐built Overall Disability Scale for Multifocal Motor Neuropathy was reduced by 9%, the Neuropathy Impairment Score showed a 3‐fold increase, the isokinetic strength was reduced by 29%, the grip strength by 56%, the Timed 25‐Foot Walk was prolonged by 13% and the EQ‐5D‐5 L‐Index value was impaired by 20%. The isokinetic strength was significantly more impaired at the wrist and ankle as compared to the elbow and knee, and one patient had lost ambulation because of instability at the ankle. Patients were considerably more fatigued and had substantially impaired hand dexterity, while mood, aerobic capacity, social adjustment, and working capacity were not affected. Regression analysis showed that lag‐time until start of initial therapy lead to impaired long‐term outcome without any effect of disease duration. Long‐term prognosis in treated MMN is characterized by moderate to severe impairment primarily affecting dexterity and stability at the ankle. Our observations support previous observations that the long‐term impairment in MMN might be improved following earlier start of therapy and that an effect of disease duration cannot be demonstrated.

Chronic inflammatory neuropathies and their impact on activities and participation

“…The degree of limitations in activity in our sample corresponds to previous studies that exclusively used RODS scores [7, 31, 32]. In line with earlier studies, we found that MMN not only affects the upper limb, but also results in significant limitations in activities related to standing and walking, indicating a more widespread loss of muscle function in both upper and lower limbs [3, 33].…”

“…Furthermore, no rehabilitation physician was involved in fewer than half of the CIDP patients who reported mild to severe limitations in activities. Previous studies showed that a majority of CIDP patients have moderate disabilities with physical and functional impairments and a few with severe disabilities at long term [7, 41]. Early initiation of appropriate treatment may prevent axonal loss, which has been shown to be an important determinant of long‐term prognosis [7, 42, 43].…”

Treatment Protocol for Secondary Burning Mouth Syndrome inCandida albicans- or Non-albicans-Positive Patients

“…5). prognosis and low treatment efficacy are associated with severe initial symptoms [33] and longer disease durations [35][36][37].…”

Recovery of foot drop in chronic inflammatory demyelinating polyneuropathy (CIDP)

“…14 In addition, delay in treatment onset also appears to negatively impact long-term recovery. 15 Of their 60 patients with CIDP, Sghrilanzani et al 13 16 We presume that early recovery of foot drop after initiation of disease modifying treatment is mainly due to resolution of proximal conduction blocks. 4,17 This mechanism is most likely in two of our patients who at onset, prior to treatment, had 0/5 ADF MRC power but an evocable TA CMAP >/= 2 mV, and who at 1 year, after treatment, had full (5/5) or nearly full (4.5/5) ADF MRC power.…”

“…Axon loss on sural nerve biopsy is associated with a poorer long‐term prognosis, 12 and in two studies, higher motor response amplitudes were associated with a better outcome 13 or with responsiveness to IVIg 14 . In addition, delay in treatment onset also appears to negatively impact long‐term recovery 15 …”