Hostname: page-component-8448b6f56d-tj2md Total loading time: 0 Render date: 2024-04-19T00:20:58.998Z Has data issue: false hasContentIssue false
  • English
  • Français

The Neuropsychiatry of Adrenomyeloneuropathy

Published online by Cambridge University Press:  07 November 2014

Mark A. Walterfang ,
John O'Donovan ,
Michael C. Fahey  and
Dennis Velakoulis
Get access Rights & Permissions [Opens in a new window]

Abstract

Background: While the adult form of adrenoleukodystrophy (ALD) has been associated with an elevated rate of affective disturbance, the myeloneuropathic form of the disease known as adrenomyeloneuropathy (AMN) has been associated with only occasional cases of major mental illness. Given that cerebral involvement occurs in up to half of AMN sufferers, we hypothesized that rates of mental illness may match those with adult ALD.

Objective: To describe the psychiatric, cognitive, and disability variables in a sample of Australian AMN sufferers.

Methods: Ten genetically confirmed AMN sufferers underwent diagnostic psychiatric interview (Structured Clinical Interview for Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition Axis I Disorders), rating scales of psychiatric disturbance (Brief Psychiatric Rating Scale, Hospital Anxiety and Depression Scale, Beck Depression and Anxiety Inventories, and Short-Form 36), and cognitive function (the Neuropsychiatry Unit Cognitive Assessment Tool and Mini-Mental State Examination).

Results: While the group as a whole was generally cognitively intact, it demonstrated a higher than expected prevalence of lifetime and current major affective illness. Current symptom levels were low at the time of study participation. Psychopathology did not relate to adrenal status, nor to level of physical or functional impairment.

Conclusion: This small sample suggests that the level of psychiatric morbidity in AMN patients is elevated, and the rate of affective disturbance approaches those of adult ALD sufferers. This may reflect that AMN is not a “pure” myeloneuropathy, and that mild cerebral involvement may be associated with affective illness.

Type
Original Research
Information
CNS Spectrums , Volume 12 , Issue 9 , September 2007 , pp. 696 - 702
Copyright
Copyright © Cambridge University Press 2007

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

REFERENCES

1.Budka, H, Sluga, E, Heiss, W. Spastic paraplegia associated with Addison's disease: adult variant of adrenoleukodystrophy. J Neurol. 1976;213:237–250.CrossRefGoogle ScholarPubMed
2.Schaumburg, H, Powers, J, Spencer, P, Raine, C, Prineas, J, Boehme, D. The myeloneuropathy variant of adrenoleukodystrophy. J Neuropathol Exp Neurol. 1976;35:312.CrossRefGoogle Scholar
3.Moser, H. Adrenoleukodystrophy: phenotype, genetics, pathogenesis and therapy. Brain. 1997;120(pt 8):1485–1508.CrossRefGoogle ScholarPubMed
4.Powers, JM, DeCiero, DP, Ito, M, Moser, AB, Moser, HW. Adrenomyeloneuropathy: a neuropathologic review featuring its noninflammatory myelopathy. J Neuropathol Exp Neurol. 2000;59:89–102.CrossRefGoogle ScholarPubMed
5.van Geel, BM, Assies, J, Wanders, RJ, Barth, PG. X-linked adrenoleukodystrophy: clinical presentation, diagnosis and therapy. J Neurol Neurosurg Psychiatry. 1997;63:4–14.CrossRefGoogle ScholarPubMed
6.van Geel, B, Assies, J, Weverling, G, Barth, P. Predominance of the adrenomyeloneuropathy phenotype of X-Iinked adrenoleukodystrophy in the Netherlands: a survey of 30 kindreds. Neurology. 1994;44:2343–2346.CrossRefGoogle ScholarPubMed
7.Moser, HW, Powers, JM, Smith, KD. Adrenoleukodystrophy: molecular genetics, pathology, and Lorenzo's oil. Brain Pathol. 1995;5:259–266.CrossRefGoogle ScholarPubMed
8.Takano, H, Koike, R, Onodera, O, Sasaki, R, Tsuji, S. Mutational analysis and genotypephenotype correlation of 29 unrelated Japanese patients with X-linked adrenoleukodystrophy. Arch Neurol. 1999;56:295–300.CrossRefGoogle ScholarPubMed
9.Auborg, P, Adamsbaum, C, Lavallard-Rousseau, M, et al.Brain MRI and electrophysiologic abnormalities in preclinical and clinical adrenomyeloneuropathy. Neurology. 1992;42:85–91.CrossRefGoogle Scholar
10.Schaumburg, HH, Powers, JM, Raine, CS, et al.Adrenomyeloneuropathy: a probable variant of adrenoleukodystrophy. II. General pathologic, neuropathologic, and biochemical aspects. Neurology. 1977;27:1114–1119.CrossRefGoogle ScholarPubMed
11.Cox, CS, Dubey, P, Raymond, GV, Mahmood, A, Moser, AB, Moser, HW. Cognitive evaluation of neurologically asymptomatic boys with X-linked adrenoleukodystrophy. Arch Neurol. 2006;63:69–73.CrossRefGoogle ScholarPubMed
12.Rosebush, PI, Garside, S, Levinson, AJ, Mazurek, MF. The neuropsychiatry of adultonset adrenoleukodystrophy. J Neuropsychiatry Clin Neurosci. 1999;11:315–327.CrossRefGoogle ScholarPubMed
13.Gothelf, D, Levite, R, Gadoth, N. Bipolar affective disorder heralding cerebral demyelination in adreno-myelo-leukodystrophy. Brain Dev. 2000;22:184–187.CrossRefGoogle ScholarPubMed
14.Luda, E, Barisone, MG. Adult-onset adrenoleukodystrophy: a clinical and neuropsychological study. Neurol Sci. 2001;22:21–25.CrossRefGoogle ScholarPubMed
15.Garside, S, Rosebush, PI, Levinson, AJ, Mazurek ME Late-onset adrenoleukodystrophy associated with long-standing psychiatric symptoms. J Clin Psychiatry. 1999;60:460–468.CrossRefGoogle Scholar
16.James, AC, Kaplan, P, Lees, A, Bradley, JJ. Schizophreniform psychosis and adrenomyeloneuropathy. JR Soc Med. 1984;77:882–884.CrossRefGoogle ScholarPubMed
17.Mahoney, FI, Barthel, DW. Functional evaluation: the Barthel Index. Md State Med J. 1965;14:61–65.Google ScholarPubMed
18.Kurtzke, JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology. 1983;33:1444–1452.CrossRefGoogle ScholarPubMed
19.First, MB, Spitzer, RL, Gibbon, M, Williams JBW. Structured Clinical Interview for DSM-IV Axis I Disorders. New York, NY: New York State Psychiatric Institute, Biometrics Research; 1994.Google Scholar
20.Beck, AT, Ward, CH, Mendelson, M, Mock, J, Erbaugh, J. An inventory for measuring depression. Arch Gen Psychiatry. 1961;4:561–571.CrossRefGoogle ScholarPubMed
21.Beck, AT, Epstein, N, Brown, G, Steer, RA. An inventory for measuring clinical anxiety: psychometric properties. J Consult Clin Psychol. 1988;56:893–897.CrossRefGoogle ScholarPubMed
22.Zigmond, AS, Snaith, RRThe hospital anxiety and depression scale. Acta Psychiatr Scand. 1983;67:361–370.CrossRefGoogle ScholarPubMed
23.Overall, JE, Gorham, DR. The brief psychiatric rating scale. Psychol Rep. 1962;10:799–812.CrossRefGoogle Scholar
24.Ware, JE Jr, Sherbourne, CD. The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care. 1992;30:473–483.CrossRefGoogle ScholarPubMed
25.Folstein, MF, Folstein, SE, McHugh, PR. “Mini-mental state”. A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res. 1975;12:189–198.CrossRefGoogle ScholarPubMed
26.Walterfang, M, Siu, R, Velakoulis, D. The NUCOG: validity and reliability of a brief cognitive screening tool in neuropsychiatric patients. Aust N Z J Psychiatry. 2006;40:995–1002.CrossRefGoogle ScholarPubMed
27.Bijl, RV, Ravelli, A, van Zessen, G. Prevalence of psychiatric disorder in the general population: results of the Netherlands Mental Health Survey and Incidence Study (NEMESIS). Soc Psychiatry Psychiatr Epidemiol. 1998;33:587–595.CrossRefGoogle ScholarPubMed
28.Kessler, RC, McGonagle, KA, Zhao, S, et al.Lifetime and 12-month prevalence of DSM-III-R psychiatric disorders in the United States. Results from the National Comorbidity Survey. Arch Gen Psychiatry. 1994;51:8–19.CrossRefGoogle ScholarPubMed
29.Dubey, P, Fatemi, A, Barker, PB, et al.Spectroscopic evidence of cerebral axonopathy in patients with “pure” adrenomyeloneuropathy. Neurology. 2005;64:304–310.CrossRefGoogle ScholarPubMed
30.Dubey, P, Fatemi, A, Huang, H, et al.Diffusion tensor-based imaging reveals occult abnormalities in adrenomyeloneuropathy. Ann Neurol. 2005;58:758–766.CrossRefGoogle ScholarPubMed