Camptocormia is a marked involuntary anteroflexed position of the trunk. In contrast with other skeletal disorders of the spine such as kiphosis, the deformity is not fixed and is correctable by passive extension or lying in the supine position. It is recognized and accepted as an axial feature of Parkinson's disease.1 The pathophysiology is unclear and may relate to dystonia or a myopathy of antigravitational muscles responsible for trunk extension.2, 3 We describe a case of camptocormia in a patient with Alzheimer's disease. Our patient is an 82-year-old man, with a 4-year history of progressive memory and functional activity impairment, who fulfilled NINCDS-ADRDA criteria for probable Alzheimer's disease. Approximately 10 years previously, a mild kiphosis was observed, which increased gradually, reaching its most severe stage 2 years ago. He had never complained of back pain. The neurological examination revealed anterior flexion of the trunk that was exacerbated when walking. He was able to correct his posture by leaning his back against the wall and standing up straight. He could also lie down flat in a supine position, but upon walking he resumed a flexed posture (Figure 1). He overcame the camptocormia by pushing his hands down against his thighs. Besides the forward flexion of the trunk he presented a discrete scoliosis with concavity to the left. His muscle strength was normal, with global hyporeflexia. There were no extrapyramidal signs. He has been using donepezil 10 mg/day for 3 years, and had never taken neuroleptics or any other medication with extrapyramidal side effects. He also had sleep apnea confirmed by polysomnography and a chronic lumbosacral radiculopathy in the L4, L5, and S1 territories on electroneuromyography. His laboratory exams were normal, including thyroid, hepatic, and renal function tests. Moreover, tests for inflammatory activity were negative. We tried a symptomatic treatment of camptocormia with levodopa 375 mg/day, which caused no remission or response, but after he developed visual hallucinations this treatment was withdrawn. Patient in standing position (with his characteristics of put his hands on thighs) and recumbent position. Patient consent was obtained for publication of this figure. Our patient fulfilled the criteria for camptocormia, with marked flexion of the spine most prominent on standing. He had no family history of Parkinson's disease or any Parkinsonian features. He never manifested other signs of dystonia or another movement disorder, and had not used neuroleptics, although he has been using a cholinesterase inhibiting medication (donepezil) for a long period. To our knowledge, there have been no other reports of camptocormia as an adverse effect of cholinesterase inhibitors, although Pisa syndrome (pleurothotonus) has been reported.4, 5 Camptocormia symptoms preceded the introduction of donepezil in our case, but in the last 2 years there has been a marked worsening while using this drug. This worsening could be inherent to the evolution of camptocormia itself.2, 3, 6 We were unable to find any other etiology for camptocormia, as listed by Azher and Jankovic.6 Our treatment with L-dopa failed, in the same manner as described by the above authors.6 Our patient had sleep apnea, a finding present in 25% of Parkinsonian patients with camptocormia,6 yet sleep apnea is also a common disorder of parkinsonian syndromes and the elderly. In contrast to other studies reporting back pain in 80% of the cases, our patient had no such complaint.2 Some authors have attributed camptocormia to an axial dystonia and associated it to diseases of the dopaminergic system, such as Parkinson's disease and multiple system atrophy. On the other hand, some authors have associated camptocormia to myopathic abnormalities of paraspinal muscles stemming from under use of the spinal extension muscles with fatty involution, in association with Parkinson's disease.3, 7 To our knowledge, this is the first case of camptocormia associated with Alzheimer's disease, in a patient with no Parkinsonian features and no prior use of antipsychotic medication. Any link with donepezil is hard to ascertain since camptocormia had appeared earlier than dementia symptoms, but it is possible that its course was worsened by use of this cholinergic inhibitor. Sonia Brucki MD*, Ricardo Nitrini MD , * Department of Neurology, University of São Paulo, Brazil, Department of Neurology, University of São Paulo, Brazil.
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Jun 23, 2024
Jérôme Bouchard-Tremblay + 10
Open Access