title: Neurological symptoms in a patient with isolated adrenocorticotropin deficiency: case report and literature review.
abstract: BACKGROUND
  Isolated adrenocorticotropic hormone (ACTH) deficiency is a pituitary disorder characterized by reduction only in the secretion of ACTH. Although the underlying mechanism remains to be elucidated, numbers of cases with this entity have been increasing. We experienced a case presenting with gait disturbance necessitating differential diagnosis from idiopathic normal pressure hydrocephalus (iNPH).
  
  
  METHODS
  A 69-year-old female with a complaint of difficulty walking and suspected to have iNPH at a prior hospital was referred to our department. For the prior three years, she had suffered from a progressive gait disturbance. Magnetic resonance imaging (MRI) revealed global ventricular dilatation. The typical features of the gait in iNPH cases were all identifiable. Neuropsychological dementia scale tests showed deterioration. However, the major feature of a disproportionately enlarged subarachnoid-space on MRI was not obvious. The patient developed progressively worsening fatigue during hospitalization. Her symptoms resembled those of hypothalamic-pituitary tumor patients. Serum ACTH and cortisol levels were low. While corticotrophin releasing hormone stress tests showed no response, other stress tests using thyrotropin releasing hormone, luteinizing hormone releasing hormone, and growth hormone releasing hormone yielded normal responses, indicating a diagnosis of isolated ACTH deficiency. We initiated corticosteroid therapy, and her gait disturbance improved promptly.
  
  
  CONCLUSIONS
  Isolated ACTH deficiency may have major significance to the differential diagnosis of iNPH. Early consideration of this entity is anticipated to facilitate making an early diagnosis.
authors.affiliation: Department of Neurosurgery, Kyoto Prefectural University Graduate School of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan. yoursongmysong@hotmail.com.
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Goto
authors.affiliation: Department of Neurosurgery, Kyoto Prefectural University Graduate School of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan. tatsu@koto.kpu-m.ac.jp.
authors.forename: Kazunori
authors.initials: K
authors.lastname: Tatsuzawa
authors.affiliation: Department of Neurosurgery, Kyoto Prefectural University Graduate School of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan. kazuyasu@koto.kpu-m.ac.jp.
authors.forename: Kazuyasu
authors.initials: K
authors.lastname: Aita
authors.affiliation: Department of Neurosurgery, Kyoto Prefectural University Graduate School of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan. yfuruno@koto.kpu-m.ac.jp.
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Furuno
authors.affiliation: Department of Neurosurgery, Kyoto Prefectural University Graduate School of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan. taku-626@koto.kpu-m.ac.jp.
authors.forename: Takuya
authors.initials: T
authors.lastname: Kawabe
authors.affiliation: Department of Neurosurgery, Kyoto Prefectural University Graduate School of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan. kohwada@koto.kpu-m.ac.jp.
authors.forename: Kei
authors.initials: K
authors.lastname: Ohwada
authors.affiliation: Department of Neurosurgery, Kyoto Prefectural University Graduate School of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan. hiroyasu@koto.kpu-m.ac.jp.
authors.forename: Hiroyasu
authors.initials: H
authors.lastname: Sasajima
authors.affiliation: Department of Neurosurgery, Kyoto Prefectural University Graduate School of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan. mineura@koto.kpu-m.ac.jp.
authors.forename: Katsuyoshi
authors.initials: K
authors.lastname: Mineura
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000172:deficiency*; D000368:Aged; D003937:Diagnosis, Differential; D004700:Endocrine System Diseases / Q000150:complications* / Q000175:diagnosis / Q000523:psychology; D005260:Female; D020233:Gait Disorders, Neurologic / Q000175:diagnosis / Q000209:etiology; D030342:Genetic Diseases, Inborn / Q000150:complications* / Q000175:diagnosis / Q000523:psychology; D006801:Humans; D006850:Hydrocephalus, Normal Pressure / Q000175:diagnosis / Q000209:etiology; D007003:Hypoglycemia / Q000150:complications* / Q000175:diagnosis / Q000523:psychology; D008279:Magnetic Resonance Imaging; D009422:Nervous System Diseases / Q000175:diagnosis / Q000209:etiology*
chemical_list: D000324:Adrenocorticotropic Hormone