Rapid progression of amyotrophic lateral sclerosis in an acromegalic patient after surgical resection of a growth hormone-producing pituitary adenoma

Agnes Mondok, Z. Arányi, Gabor G. Kovacs, S. Czirják, Peter Pusztai, I. Varga, K. Rácz

Research output: Contribution to journalArticle

2 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)315-318
Number of pages4
JournalNeurologist
Volume16
Issue number5
DOIs
Publication statusPublished - Sep 2010

Fingerprint

Acromegaly
Amyotrophic Lateral Sclerosis
Pituitary Neoplasms
Growth Hormone
Somatomedins
Riluzole
Dysarthria
Octreotide
Muscular Atrophy
Human Growth Hormone
Insulin-Like Growth Factor I
Upper Extremity
Nervous System
Signs and Symptoms
Spinal Cord
Quality of Life
Clinical Trials
Neurons
Brain
Therapeutics

Keywords

  • acromegaly
  • amyotrophic lateral sclerosis
  • growth hormone
  • insulin-like growth factor-I
  • neuroprotection

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Rapid progression of amyotrophic lateral sclerosis in an acromegalic patient after surgical resection of a growth hormone-producing pituitary adenoma. / Mondok, Agnes; Arányi, Z.; Kovacs, Gabor G.; Czirják, S.; Pusztai, Peter; Varga, I.; Rácz, K.

In: Neurologist, Vol. 16, No. 5, 09.2010, p. 315-318.

Research output: Contribution to journalArticle

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