Thursday, January 25, 2018

Kennedy's Disease - Beyond Motor Neurons

The link below opens a recent study published in the Journal of Neurology, Neurosurgery & Psychiatry. It explains SBMA (Kennedy’s Disease) very well. The report is not just focused on the motor neurons. It goes into detail explaining other aspects of the condition and other potential opportunities for a treatment.

Beyondmotor neurons: expanding the clinical spectrum in Kennedy’s disease

Raquel Manzano, Gianni Sorarú, Christopher Grunseich, Pietro Fratta, Emanuela Zuccaro, Maria Pennuto, Carlo Rinaldi

“…Compared with other motor neuron diseases, such as amyotrophic lateral sclerosis (ALS), disease progression is relatively slow, with muscle strength declining by 2% per year. Recent evidence has shown that the muscle atrophy in SBMA is not solely secondary to the motor neuron degeneration but also consequence of a primary myopathic process. In addition, SBMA is frequently complicated by other signs and symptoms such as gynaecomastia, impotence, testicular atrophy and metabolic changes, suggesting a multisystem involvement in the disease. These features frequently manifest early in the disease course and can contribute substantially to the morbidity. In this review, we discuss the non-motor neuron abnormalities underlining SBMA symptomatology. We propose that an improved understanding of these features not only could result in better management of the patients SBMA, but also has the potential to shed new light into the disease pathogenesis, lead to the discovery of biomarkers for disease progression and open new treatment avenues. …”

“… Motor neurons are particularly sensitive to alterations in the proteostasis network, likely due to their extreme polarisation and post-mitotic nature. On the other side, alterations of protein quality control in muscle, by tilting the balance towards increased protein degradation, are likely the underlying cause of the muscle atrophy observed in this disease. …”

I feel this information should be shared with your primary care doctors. It might help explain other symptoms not normally associated with Kennedy’s Disease.

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