Case Report/Case SeriesRapid Progression of Amyotrophic Lateral Sclerosis in an Acromegalic Patient After Surgical Resection of a Growth Hormone-Producing Pituitary AdenomaMondok, Agnes MD*; Aranyi, Zsuzsanna MD†; Kovacs, Gabor G. MD‡; Czirjak, Sandor MD, DSc§; Pusztai, Peter MD*; Varga, Ibolya ChemD¶; Racz, Karoly MD, DSc* Author Information From the *Second Department of Medicine and †Department of Neurology, Semmelweis University, Budapest, Hungary; ‡Department of Neuropathology, National institute of Psychiatry, Budapest, Hungary; §National Institute of Neurosurgery, Budapest, Hungary; and ¶Molecular Medicine Research Group, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary. Gabor G. Kovacs currently at Institute of Neurology, Medical University of Vienna, Austria. Reprints: Karoly Racz, MD, DSc, 2nd Department of Medicine, Semmelweis University, 46 Szentkiralyi, Budapest H-1088, Hungary. E-mail: [email protected]. The Neurologist: September 2010 - Volume 16 - Issue 5 - p 315-318 doi: 10.1097/NRL.0b013e3181b46fef Buy Metrics Abstract Introduction: Insulin-like growth factor-1 (IGF-1) promotes the survival of neurons, mediates neuritic growth, and in 1 clinical trial human recombinant IGF-1 delayed the progression of functional impairment and decline of health-related quality of life in patients with amyotrophic lateral sclerosis (ALS). Case Report: We describe a case of a 65-year-old woman with a 2-year history of symptoms and signs of acromegaly because of a pituitary microadenoma. The patient posed a challenging diagnostic dilemma because of the presence of dysarthria, which was initially considered as the consequence of acromegaly. After octreotide long-acting release (LAR) treatment, the patient underwent uneventful pituitary surgery. Although postoperative evaluation indicated a cure of acromegaly, progressive bulbar symptoms developed, which were followed by upper limb weakness and muscle atrophy. Neurologic investigations confirmed the diagnosis of ALS and riluzole therapy was given. One year after surgery growth-hormone deficiency was diagnosed, but a trial with human recombinant growth hormone failed to produce any significant improvement. Two years after surgery the patient died of a sudden respiratory arrest. Histopathologic examination of the brain and spinal cord confirmed the diagnosis of ALS. Conclusions: This is the first report showing a rapid progression of ALS after a surgical cure of coexisting acromegaly presumably because of cessation of high endogenous IGF-I levels. © 2010 Lippincott Williams & Wilkins, Inc.