Chronic Cortical Stimulation for Amyotropic Lateral Sclerosis: A Report of Four Consecutive Operated Cases after a 2-Year Follow-up: Technical Case Report

OBJECTIVE AND IMPORTANCE: From April to July 2002, four consecutive patients affected by amyotrophic lateral sclerosis (ALS) were surgically treated with bilateral chronic cortical stimulation. CLINICAL PRESENTATION: The preoperative diagnostic assessment was based on the results of a neurological examination, integrated by the ALS functional rating scale, electromyography, magnetic resonance imaging, and single-photon emission computed tomography (SPECT). In particular, SPECT provided a remarkable contribution in terms of its ability to demonstrate specific morphological and metabolic ALS lesions of the brain. INTERVENTION: The postoperative neurological follow-up was based mainly on the assessment of neurological status and the ALS functional rating scale, together with SPECT. Two years after surgery, at clinical examination, two patients demonstrated mild progression of their illness, and SPECT findings disclosed complete disappearance of ALS cerebral lesions, with apparent recovery of brain anatomic integrity. The third patient committed suicide. In the fourth patient, the disease seemed to have progressed inexorably at the time of clinical and SPECT monitoring, after transient improvement during the first 3 months after surgery. CONCLUSION: These preliminary results are surprising, because they suggest that chronic cortical stimulation can play a role against ALS and deserve confirmation in larger numbers of patients and for a longer follow-up. We present the theoretical grounds for these findings, as well as the diagnostic and surgical procedures and results.

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