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BRIEF COMMUNICATIONS: Oliver Sacks and Melanie Shulman Steroid dementia: An overlooked diagnosis? Neurology 2005; 64: 707-709 [Abstract] [Full text] [PDF]

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Reply to Norra et al

Oliver Sacks, M.D., Melanie Shulman, M.D.   (13 June 2005)

Steroid dementia: An overlooked diagnosis?

Christine Norra, MD, Marlies Arndt, Hanns J. Kunert   (13 June 2005)

Reply to Norra et al 13 June 2005
Oliver Sacks, M.D., NYU Medical Center 2 Horatio St. #3G, New York, NY 10014, Melanie Shulman, M.D. Send Correspondence to journal: Re: Reply to Norra et al mail{at}oliversacks.com Oliver Sacks, M.D., et al.	The brief but striking case history by Norra et al, along with the paper by Brunner et al strengthens our feeling that substantial doses (and even single doses) of steroids may induce long- lasting cognitive impairments more commonly than generally supposed. Sometimes a condition may be strangely overlooked--as with muscular dystropy in the 1860s, and Tourette's syndrome a century later--until it is focused on. This may be the case with steroid dementia.
Steroid dementia: An overlooked diagnosis? 13 June 2005
Christine Norra, MD, Dept. of Psychiatry and Psychotherapy, University Hospital RWTH Aachen, Pauwelsstrasse 30, D-52074 Aachen, Germany, Marlies Arndt, Hanns J. Kunert Send Correspondence to journal: Re: Steroid dementia: An overlooked diagnosis? cnorra{at}ukaachen.de Christine Norra, MD, et al.	Sacks and Shulman [1] reported a 72-year-old patient who developed severe dementia after taking an increased dosage of prednisolone for at least 3 months but resolved after discontinuing medication. Six months later, neurobehavioral testing showed a tendency to perseveration, impulsivity, inattentiveness in scanning, and memory retrieval deficits. Fourteen months later, there was still mild dysfunction in executive function and memory. They concluded that this reversible steroid-induced dementia suggested a prefrontal-hippocampal dysfunction. In another recent issue, Brunner et al [2] reported reversible impairments in verbal learning and memory on a 5-day high dose treatment with glucocorticoids in patients with acute neuritis and multiple sclerosis. We observed different cognitive effects of a twenty-day self-induced high-dose corticosteroid treatment in a 54-year-old general practitioner. [3] Due to apoplectiform deafness, she accidentally administered 100 mg dexamethasone per day intravenously before hospital admission with manic psychosis. Psychotic symptoms subsided immediately with antipsychotics and stepwise reduction of steroids. Blood and diagnostic procedures (whole brain CT and MRI, EEG, SPECT) were normal. The patient refused a lumbar puncture. There were no previous neuropsychiatric illnesses. Initial neuropsychological examinations showed seriously impaired cognitive capabilities (intelligence, abstract thinking and problem solving, attention, learning and memory). Follow-up testing (one, two, four and six months) revealed improved general cognitive functioning (WAIS: IQ from 96 to 126). However, serious deficits in learning and memory with a high tendency to confabulate still were obvious (WMS-R, selective reminding). Autobiographic memory and implicit, non-declarative memory were intact, but the patient remained sub-euphoric, emotionally indifferent and suggestible with some reduction of insight. Attentional functions (alertness, divided attention) were all normal. Working memory was slightly decreased only at initial testing. This rare case confirms studies showing that exogenous steroids can cause dysfunctions in declarative, hippocampus-dependent memory processing. [2,4] According to Sacks and Shulman [1], the persisting prominent confabulations and other psychopathological signs indicate that glucocorticosteroids may also induce functional disorder of additional brain regions (e.g., frontal and temporal lobe) which are important for cognitive and emotional processing. As the cortisone-induced cognitive disorder had not remitted completely after six months, reversibility of deficits in learning and memory remains unconfirmed in our patient. It is possible that the elderly are at greater risk for persistence of cognitive impairment after taking steroids. [5] The neuropsychological test results together with the psychiatric disorder point to dysfunctional temporo-frontal circuits in steroid dementia. References 1. Sacks O, Shulman M. Steroid dementia: An overlooked diagnosis? Neurology 2005;64:707-709. 2. Brunner R, Schaefer D, Hess K, Parzer P, Resch F, Schwab S. Effect of corticosteroids on short-term and long term memory. Neurology 2005;64:335- 337. 3. Arndt M, Kunert HJ, Sass H, Norra C. Cognitive impairment after accidental high-dose corticosteroid therapy. Nervenarzt 2004;75:904-907. 4. Newcomer JW, Craft S, Hershey T, Askins K, Bardgett ME. Glucocorticoid- induced impairment in declarative memory performance in healthy humans induced by stress-level cortisol treatment. J Neurosci 1994;14:2047-2053. 5. Wolkowitz OM, Reus VI, Canick J, Levin B, Lupien S. Glucocorticoid medication, memory and Steroid Psychosis in medical illness. Ann NY Acad Scien 1997;823:81-96.