The demography, survival, and motor phenotypes of amyotrophic lateral sclerosis (ALS) patients have been rarely described in Hispanic countries. The clinical characteristics and survival of a series of Mexican ALS patients are described. Mexican patients with definite ALS were included in a five-year retrospective longitudinal study. Their demographic and clinical features, cumulative survival rates, and independent predictive factors for survival were analysed. Sixty-one definite ALS patients were included. The median follow-up period was 35 months (range 12-108 months). Males were predominant (1.8: 1), the mean age at onset was 47.5 ± 10.5 years, and the median interval from onset to diagnosis was 12 months. Spinal onset occurred in 66% of patients. Upper motor neuron phenotype was predominant in 53% of patients. The overall mean survival from onset was 68.6 months, and from diagnosis was 57.8 months. Longer survival was determined in patients aged ≤ 40 years (54.7 months) compared with other age groups (p = 0.006). In conclusion, the clinical heterogeneity, male predominance, and survival rates in our sample are consistent with those of other studies. Patients in this series had a younger age at onset and a clear trend toward longer survival compared with those of other population studies.
Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disorder characterized by rapid deterioration and selective death of motor neurons (MNs) in central nervous system. There is no effective therapy for ALS patients. Riluzole, only slightly delays disease progression. Edaravone was FDA approved as an intravenous therapy, however, this drug is expensive and has been applied in few cases in our country. There is compelling evidence patients who attend an ALS multidisciplinary clinic experience improved survival. An ALS multidisciplinary clinic was launched in our institution and adapted to our healthcare system. It is described the processes involved in the evaluation of patients in an all day long session where ALS patients are evaluated by several professionals from different specialties specially trained in the needs of subjects living with this disorder. We consider that this model of healthcare for patients with ALS may be useful in another healthcare institutions in our country.
A 9-year-old girl with akinetic-rigid parkinsonism with tremor is described. She was hospitalized with neuroleptic malignant syndrome that started 3 days after anticonvulsant drug treatment owing to epileptic seizures. Cranial magnetic resonance imaging (MRI) was normal, and during the follow-up, magnetic resonance spectroscopy revealed a decrement on N-acetylaspartate in the basal ganglia, suggesting neuronal dysfunction. The basal ganglia and dopamine are involved in the pathophysiology of parkinsonism and neuroleptic malignant syndrome and have been recognized in seizure propagation and seizure threshold. Parkinsonism in children is considered an acquired, secondary, and reversible disorder with a dramatic improvement to treatment. However, our patient still has parkinsonism 2 years after diagnosis. This case represents the unusual presentation of epilepsy, parkinsonism, and neuroleptic malignant syndrome, which might have a common pathophysiologic pathway (dopaminergic dysfunction) involving the basal ganglia and the hypothalamus.
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