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Cognitive Disorder NOS With Arnold-Chiari I Malformation

TO THE EDITOR: In certain circumstances, cognitive disorders can result from a general medical condition. We present a case consistent with a diagnosis of Cognitive Disorder, Not Otherwise Specified (CD–NOS) as a consequence of an Arnold-Chiari I malformation (AC-IM).

AC-I and II malformations are conditions in which the cerebellum protrudes into the spinal canal. AC-IM usually causes symptoms (headaches, vertigo, spastic quadriparesis, weakness) in young adults and is often associated with syringomyelia,¹ whereas the more lethal AC-IIM is associated with myelomeningocele and hydrocephalus and is usually apparent at birth.

Case Report
ME is a 28 year-old Black female diagnosed with AC-IM at 15 after undergoing an MRI to assess her scoliosis. Since this time, she has required multiple surgical interventions to treat hydrocephalus caused by ventriculoperitoneal (VP) shunt blockade. In August 2004, a similar episode with resulting CSF infection led to a quadriparesis. Despite the quadriparesis, ME displayed no social or occupational dysfunction and continued her work as a daycare teacher.

In October 2004, ME was admitted to the hospital with decreased level of consciousness and was again diagnosed with hydrocephalus. Her shunt was replaced, and, several weeks later, a psychiatric consultation was requested as new cognitive deficits were noted.

Psychiatric review of systems was negative for symptoms of depression, anxiety, psychosis, and delirium. There was no history of substance abuse and no evidence of Axis II pathology. Pertinent mental status findings included quadriparesis, a blank stare, flat affect, and cognitive impairment. MMSE score was 14/30, with orientation and attention being most affected. More detailed cognitive testing revealed no evidence of agnosia, disturbance in executive functioning, or ability to problem-solve. Furthermore, her family validated that she was able to converse logically and retrieve long-term memories.

Discussion
Our team struggled with determining the correct Axis I diagnosis. The patient does not meet criteria for a dementia, because she does not have the progressive course or memory impairment with disturbance in two or more cognitive domains. As such, the diagnosis of CD–NOS was most appropriate, because of the limited deficits in attention and memory.

The literature describes this condition with many different terms, such as predementia, mild cognitive impairment (MCI), and cognitive impairment, no dementia (CIND).² DSM–IV-TR uses CD, Not Otherwise Specified, and, in Appendix B, lists a term called Mild Neurocognitive Disorder (MND).³ Our patient meets the criteria for MND, in that her deficits cause functional impairment, are a direct result of her neurological condition, and are limited to two cognitive domains.

A recent article suggests possible benefits to treating these patients with a cholinesterase inhibitor.⁴ However, the evidence is far from convincing, and we elected not to treat this patient pharmacologically.

The authors thank the patient and her family for their full cooperation and assistance.

REFERENCES

1. Cheng JS, Nash J, Meyer GA: Chiari Type I malformation revisited: diagnosis and treatment. Neurologist 2002; 8:357–362[Medline]
2. Tuokko H, Frerichs RJ: Cognitive impairment with no dementia (CIND): longitudinal studies, the findings, and the issues. Clin Neuropsychol 2000 14:504-525
3. Diagnostic and Statistical Manual of Mental Disorders, 4th Ed., Text Revision. Washington, DC, American Psychiatric Association, 2000
4. Salloway S, Ferris S, Kluger A, et al: Efficacy of donepezil in mild cognitive impairment: a randomized, placebo-controlled trial. Neurology 2004; 63:651–657[Abstract/Free Full Text]

Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Email this article to a Colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My Articles & Searches Download to citation manager Citing Articles via Google Scholar Articles by Pearce, M. Articles by Bhalerao, S. Search for Related Content PubMed Citation Articles by Pearce, M. Articles by Bhalerao, S. Syndromes Secondary to General Medical Disorders

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