Survival and prognostic factors of motor neuron disease in a multi-ethnic Asian population

Abstract

Our objective was to determine the survival and prognostic factors of motor neuron disease (MND) in a multi-ethnic cohort of Malaysian patients. All patients seen at a university medical centre between January 2000 and December 2009 had their case records reviewed for demographic, clinical and follow-up data. Mortality data, if unavailable from records, were obtained by telephone interview of relatives or from the national mortality registry. Of the 73 patients, 64.4% were Chinese, 19.2% Malays and 16.4% Indians. Male: female ratio was 1.43: 1. Mean age at onset was 51.5 + 11.3 years. Onset was spinal in 75.3% and bulbar in 24.7% of the patients; 94.5% were ALS and 5.5% were progressive muscular atrophy (PMA). Overall median survival was 44.9 + 5.8 months. Ethnic Indians had shorter interval from symptom onset to diagnosis and shorter median survival compared to non-Indians. On Cox proportional hazards analysis, poor prognostic factors were bulbar onset, shorter interval from symptom onset to diagnosis and worse functional score at presentation. In conclusion, age of onset and median survival duration are similar to previous reports in Asians. Clinical features and prognostic factors are similar to other populations. In our cohort, ethnic Indians had more rapid disease course accounting for their shorter survival.

Amyotrophic lateral sclerosis and motor neuron syndromes in Asia

“…Reports of classical ALS phenotypes in the Asian population suggest similar subtypes to those seen in the Caucasian patients, with limb-onset ALS accounting for the majority (around 75%) followed by bulbar-onset ALS 13 14 16 69. Compared with the mean disease survival in ALS from western populations, a recent study of 549 Japanese patients with sporadic ALS reported a longer median survival (without the use of tracheostomy positive pressure ventilation (TPPV)) at 48 months 11.…”

“…Similar access to such care may not be available to patients from countries where financial resources are limited and the healthcare costs are borne by patients. This has been shown at least in studies from India, Malaysia and China where limitations exist on patients' affordability of treatments as well as patients' willingness to accept life-prolonging measures such as artificial feeding and respiratory support 13 16 69. There are circumstances where the choice of not accepting life-prolonging interventions relates to cultural factors, but it is likely that financial costs are prohibitive.…”

Targeted assessment of lower motor neuron burden is associated with survival in amyotrophic lateral sclerosis

“…These range from fulminant disease with survival less than three years to a 'tail' of 10-20% of patients with a very slowly progressive or plateau course (3)(4)(5). Estimating prognosis is of great importance to patients, with older age, lower score on the ALSFRS-R, shorter diagnostic delay with more rapid progression, bulbar onset, presence of frontotemporal dementia (FTD), smoking and low body-mass index all portending a shorter survival (3,(6)(7)(8)(9)(10)(11)(12). Some factors, such as site of disease onset (bulbar versus limb), have a more complex effect on survival that is influenced by age and gender (13,14).…”

The first Malay database toward the ethnic-specific target molecular variation

“…Leukemia is the most common cancer among children below 14 years old. Mendelian genetic disorders such as thalassemia [ 11 ], Duchenne muscular atrophy [ 12 ], spinal muscular atrophy [ 13 ], retinoblastoma [ 14 ], G6PD deficiency [ 15 ] and orofacial clefts [ 16 ] are also relatively common in the country.…”