International 2019; volume 11:8257

Alternating flexed-extended followed by inversion of the left foot, sug- posturing in progressive gesting dystonia. He had been bedridden in Correspondence: Nobuyuki Ishii, Division of a nursing home for 2 years and had had dif- Neurology, Respirology, Endocrinology and supranuclear palsy ficulty communicating for 1 year. Metabolism, Department of Internal On admission, he expressed no mean- Medicine, University of Miyazaki, 5200 Nobuyuki Ishii, Hitoshi Mochizuki ingful words and instead exhibited stereo- Kihara, Kiyotake, Miyazaki, 889-1692, Japan. Tel: +81-985-85-2965 - Fax: +81-985-85-1869 Division of Neurology, Respirology, typed moaning or groaning, which was caused by dysphonia and nonfluent aphasia. E-mail: [email protected] Endocrinology and Metabolism, revealed bilateral Department of Internal Medicine, Key words: progressive supranuclear palsy; vertical gaze palsy with preserved oculo- University of Miyazaki, Japan dystonia; decerebrate state; decorticate state; cephalic reflexes, axial rather than limb abnormal posturing. rigidity, preserved but sluggish bilateral pupillary light reflexes, and prominent neck Contributions: NI and HM, design and coordi- dystonia. The deep tendon reflexes were nation of the study, analysis and interpretation Abstract exaggerated and the Babinski response was of the data, collection of the material, drafting of the manuscript. A 69-year-old man who had been positive bilaterally. In addition, the upper bedridden in nursing home because of a 5- extremities showed flexed posturing on the right side (Figure 1A, red arrowheads) and Conflict of interest: The authors declare no year history of progressive supranuclear potential conflict of interest. palsy (PSP) was admitted due to aspiration extended posturing on the left (red arrows), and the left lower limb exhibited flexion pneumonia. Besides neck dystonia in exten- Funding: This study was partly supported by contracture. Magnetic resonance imaging of sion, he showed “alternating flexed–extend- JSPS KAKENHI Grant Number 19K20715 the brain revealed global cerebral atrophy ed posturing”, in which the arm was flexed (NI). on one side and extended on the other. that predominantly affected the cortex and Magnetic resonance imaging of the brain (Figure 1B-D). The patient was Received for publication: 27 July 2019. revealed global cerebral atrophy that pre- treated with ampicillin/sulbactam for 7 days Acceptedonly for publication: 12 August 2019. dominantly affected the cortex and mid- and discharged to a nursing home. This work is licensed under a Creative brain. The mechanisms of complex postur- Commons Attribution NonCommercial 4.0 ing in late-stage PSP may sometimes be License (CC BY-NC 4.0). related to decortication and decerebration as Discussion use well as dystonia, and “alternating flexed– ©Copyright: the Author(s), 2019 extended posturing” might be one of the PSP is the most common atypical Licensee PAGEPress, Italy phenotypes of pathological progression in parkinsonian syndrome, and typically pres- Neurology International 2019; 11:8257 PSP. ents with vertical supranuclear gaze palsy, doi:10.4081/ni.2019.8257 postural instability with falls, and progres- sive cognitive disturbance.1 Patients with parkinsonism sometimes exhibit character- which often accompanies PSP,5 rather than Introduction istic abnormal postures such as neck dysto- by decortication or decerebration. In the nia in extension in PSP, Pisa syndrome and Patients with parkinsonism sometimes early disease stage, the patient exhibited camptocormia in Parkinson’s disease, and develop characteristic abnormal postures. rigidity in the left limbs, which was predic- dropped head in multiple system atrophy.2,3 Progressive supranuclear palsy (PSP), tive of left limb dystonia and fixed contrac- In addition to neck dystonia, our patient which is an atypical parkinsonian syndrome ture of the left lower limbs. The right upper exhibited “alternating flexed–extended pos- characterized by early postural instability, limb exhibited flexed posturing, which turing,” in which the arm was flexed on one falls, vertical supranuclear palsy, and pro- could not differentiate decorticate and dys- side and extended on the other. gressive dementia, usually causes disease- tonic posturing. It is impossible to exclude We speculate that this characteristic specific postures such as neck dystonia in the possibility that dystonia may have con- posturing may be caused by simultaneous extension.1 Here, we report a patientNon-commercial with tributed to the final posturing. decerebration and decortication as well as PSP who presented with “alternating The laterality of the patient’s abnormal dystonia. Decerebrate posturing is caused flexed–extended posturing,” in which the posturing is likely related to the pathologi- by midbrain lesions that release the vestibu- arm was flexed on one side and extended on cal progression of PSP. PSP causes degener- lospinal postural reflexes from forebrain the other. This posturing may potentially be control and activate the extensor muscles, ation of both the cerebral cortex and mid- caused by small differences in the degree of while decorticate posturing is due to dys- brain, which leads, for example, to demen- left vs. right neurodegeneration in the cere- function of the cerebral cortex that releases tia and supranuclear gaze palsy, respective- bral cortex and midbrain, both structures 1 other spinal reflexes.4 These two postures ly. In our patient, besides dystonia, slight that are affected by PSP.1 are not disease-specific, and patients with differences in pathological progression in neurodegenerative diseases usually mani- the cortex and midbrain may have caused concurrent decorticate and decerebrate pos- Case Report fest them in the bilateral limbs. Our patient, however, presented simultaneously with turing in the late clinical course. A 69-year-old man with a 5-year history flexed posturing in the right limbs and Furthermore, the prominent asymmetrical of PSP was admitted due to aspiration pneu- extended posturing in the left limbs. posture might indicate a possible diagnosis monia. When he was initially diagnosed In contrast with the upper limbs, this of PSP with corticobasal syndrome (PSP– with PSP, he exhibited rigidity of the left patient’s left hip and knee showed flexed CBS) rather than classic PSP (Richardson extremities and the trunk. As the disease posturing, and his foot was inverted. These syndrome).6 progressed, he developed neck dorsiflexion postures were likely caused by dystonia,

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References 1. Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol 2009;8:270-9. 2. Savica R, Kumar N, Ahlskog JE, et al. Parkinsonism and dropped head: dysto- nia, myopathy or both? Parkinsonism Relat Disord 2012;18:30-4. 3. Doherty KM, van de Warrenburg BP, Peralta MC, et al. Postural deformities in Parkinson’s disease. Lancet Neurol 2011;10:538-49. 4. Posner JB, Plum F. Plum and Posner’s diagnosis of stupor and . fourth ed. Oxford University Press, New York, 2007. 5. Barclay CL, Lang AE. Dystonia in pro- gressive supranuclear palsy. J Neurol Neurosurg Psychiatry 1997;62:352-6. 6. Ling H, de Silva R, Massey LA, et al. Characteristics of progressive supranu- clear palsy presenting with corticobasal onlysyndrome: a cortical variant. Figure 1. A) Alternating flexed–extended posture in the upper limbs; flexed right arm Neuropathol Appl Neurobiol (arrowheads) and extended left arm (arrows). B-D) Fluid-attenuated inversion recovery 2014;40:149-63. (FLAIR) MRI with diffuse atrophy in the midbrain and cerebral cortex. use

to decortication and decerebration as well Conclusions as dystonia, and “alternating flexed–extend- ed posturing” might be one of the pheno- The mechanisms of complex posturing types of pathological progression in PSP, in late-stage PSP may sometimes be related especially PSP-CBS.

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