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Table of Contents
CASE REPORT
Year : 2015  |  Volume : 18  |  Issue : 1  |  Page : 108-110
 

Generalized chorea due to delayed encephalopathy after acute carbon monoxide intoxication


1 Department of Neurology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan
2 Department of Medicine, Taoyuan Armed Forces General Hospital, Taoyuan, Taiwan

Date of Submission04-Jun-2014
Date of Decision04-Aug-2014
Date of Acceptance29-Aug-2014
Date of Web Publication10-Feb-2015

Correspondence Address:
Yueh-Feng Sung
Department of Neurology, Tri-Service General Hospital, National Defense Medical Center, No. 325, Section 2, Cheng-Gong Road, Neihu 114, Taipei
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.144288

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   Abstract 

Movement disorder due to delayed encephalopathy after carbon monoxide (CO) intoxication is uncommon. Generalized chorea, presenting as an initial symptom of delayed encephalopathy, is extremely rare. We describe a 60-year-old woman, who had completely recovered from acute CO poisoning, developed mental and behavioral changes, urinary incontinence and generalized chorea 2 weeks thereafter. T2-weighted brain magnetic resonance imaging showed extensive hyperintensity of the bilateral periventricular and subcortical white matter and the globus pallidus. Brain single-photon emission computed tomography (SPECT) with technetium-99 ethylene cysteine dimer showed inhomogeneous perfusion in the cerebral cortex, with decreased uptake in bilateral frontal regions. Delayed encephalopathy after acute CO intoxication was diagnosed, and the symptoms gradually improved after hyperbaric oxygen therapy (HBOT). This case report demonstrates that generalized chorea may be one of the initial presenting symptoms of delayed encephalopathy after acute CO intoxication. We hypothesize that the generalized chorea in our patient may have been caused by the subcortical white matter lesions, which most likely interrupted the basal ganglia-thalamocortical circuits and that HBOT may be the treatment of choice for such patients.


Keywords: Chorea, carbon monoxide, hyperbaric oxygen therapy, magnetic resonance imaging


How to cite this article:
Sung YF, Chen MH, Peng GS, Lee JT. Generalized chorea due to delayed encephalopathy after acute carbon monoxide intoxication. Ann Indian Acad Neurol 2015;18:108-10

How to cite this URL:
Sung YF, Chen MH, Peng GS, Lee JT. Generalized chorea due to delayed encephalopathy after acute carbon monoxide intoxication. Ann Indian Acad Neurol [serial online] 2015 [cited 2018 Feb 25];18:108-10. Available from: http://www.annalsofian.org/text.asp?2015/18/1/108/144288



   Introduction Top


The incidence of delayed encephalopathy after carbon monoxide (CO) intoxication is low. Delayed encephalopathy has been shown to occur 14-45 days after complete recovery from the acute stage of CO intoxication and is characterized by mental impairment, motor dysfunction, dementia, or psychosis. [1] Chorea is characterized by a continuous flow of random, brief, involuntary muscle contractions and can result from a wide variety of causes such as hypoxic-ischemic injury, drugs, toxins, infections, autoimmune disorders and endocrine and electrolyte abnormalities. [2] Chorea presenting as a symptom of delayed encephalopathy is extremely rare. We herein report the case of a woman with generalized chorea as one of the first manifestations of delayed encephalopathy following acute CO intoxication. Her symptoms improved gradually after hyperbaric oxygen therapy (HBOT).


   Case Report Top


A 60-year-old woman, with a history of major depression, attempted suicide by burning charcoal in a closed room 3 weeks before admission. She was found unconscious and was immediately sent to a nearby hospital. She regained full consciousness from the acute stage of CO poisoning within 1 day under high-flow oxygen therapy. She was well after discharge, and no medications were prescribed. One week prior to admission, she began to experience cognitive impairment, behavioral changes, unsteady gait and urinary incontinence. Restlessness and involuntary movements of the limbs were also observed by her family. Due to the persistent symptoms, she was admitted to our hospital.

On examination, she was found to be alert but could not obey or understand orders. Her blood pressure was 128/84 mmHg, heart rate 79 bpm, respiration rate 18 breaths/min, and body temperature 36.5˚C. Pupil light reflexes and eye movements were normal. She exhibited akinetic mutism, rigidity of the limbs, and a short-stepped gait. Deep tendon reflexes were normal, muscle strength of the limbs was Medical Research Council Grade 4, and Babinski's sign was absent. She was found to exhibit persistent, rapid, irregular, involuntary choreic movements of the whole body, which were invisible during sleep. Blood studies, including biochemistry, endocrine and autoimmune tests, were normal.

Electroencephalography showed a poorly sustained alpha background and generalized slow waves, predominantly at the bilateral fronto-temporal lobes, indicating cortical dysfunction. T2-weighted and fluid-attenuated inversion recovery (FLAIR) brain magnetic resonance imaging (MRI) showed bilateral, symmetric, confluent areas of high signal intensity in the periventricular, subcortical white matter, corona radiata, centrum semiovale and globus pallidus [Figure 1]a and b. Diffusion-weighted MRI and the corresponding apparent diffusion coefficient map showed restricted water diffusion at the periventricular white matter, corona radiata, and centrum semiovale, suggesting cytotoxic edema [Figure 1]c and d. Brain single-photon emission computed tomography (SPECT) with technetium-99 ethylene cysteine dimer showed inhomogeneous perfusion in the cerebral cortex, with decreased uptake in bilateral frontal regions [Figure 1]e. Uptake in bilateral basal ganglia and the thalamus was normal.
Figure 1: Fluid-attenuated inversion recovery (FLAIR) (a) and T2-weighted (b) brain magnetic resonance imaging (MRI) showing bilateral, symmetric, confl uent areas of high signal intensity in the periventricular, subcortical white matter, corona radiata, centrum semiovale and globus pallidus. Diffusionweighted MRI (c) and the corresponding apparent diffusion coeffi cient map (d) showing restricted water diffusion at the periventricular white matter, corona radiata and centrum semiovale. Brain single-photon emission computed tomography (e) showing inhomogeneous perfusion in the cerebral cortex, with relative decreased uptake in bilateral frontal regions

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The patient was diagnosed with delayed encephalopathy after acute CO intoxication. Neuroleptics for suppression of generalized chorea were not administered, for fear of exacerbating the  Parkinsonism More Details. She instead underwent HBOT. The symptoms of generalized chorea gradually improved and disappeared completely 2 weeks later. The cognitive impairment improved moderately after 20 sessions of HBOT. However, the urinary incontinence, mild rigidity of the limbs, and short-stepped gait remained unchanged after discharge. Follow-up brain MRI 8 months later showed more cortical atrophy and prominent hyperintensity of the bilateral periventricular white matter seen on FLAIR and T2-weighted image [Figure 2]a and b.
Figure 2: Follow-up brain MRI showing more cortical atrophy and prominent hyperintensity of the bilateral periventricular white matter seen on FLAIR (a) and T2-weighted image (b)

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   Discussion Top


Delayed encephalopathy after CO intoxication is characterized by mental impairment, psychosis, urinary incontinence, or motor dysfunction, which develops between a few days and a few weeks after acute CO poisoning. [1] Thom hypothesized that CO activates polymorphonuclear leucocytes, which diapedese and cause brain lipid peroxidation, leading to the delayed effects of CO poisoning. [3] The most characteristic neuropathologic findings following CO intoxication are necrosis of the bilateral globus pallidus and progressive white matter demyelination. Involvement of the centrum semiovale is considered to be more related to delayed neuropsychiatric syndrome. [4] Some patients with follow-up MRI have revealed a decrease in signal abnormalities of white matter lesions, accompanied with a lessening in the severity of symptoms, suggesting a reversible process. [4],[5]

While delayed encephalopathy following CO intoxication is not normally associated with involuntary movements, it may give rise to chorea in rare cases. Choi et al., reported 242 patients with CO poisoning, 32 (13.2%) of whom developed delayed movement disorders. Of these 32 patients, 23 (71.9%) had parkinsonism, five dystonia, three chorea and one myoclonus. [5] Song and Chung reported a patient with delayed encephalopathy due to CO intoxication suffering from chorea involving a unilateral lower limb. There were no basal ganglia lesions, only bilateral white matter lesions, in this patient. [6] In addition to CO, some toxins have been reported to cause choreic movement. [1],[ 7],[8],[9],[10],[11] The toxic causes of chorea and the radiological findings are listed in [Table 1].
Table 1: The toxic causes of chorea and the radiological findings

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The probable pathophysiologic mechanism of chorea seems to result from hypoactivity in the indirect pathway from the basal ganglia-thalamocortical motor circuits. [2] We hypothesize that the generalized chorea in our patient may have been caused by the subcortical white matter lesions, which most likely interrupted the basal ganglia-thalamocortical circuits. It remains unclear why similar neuroimaging changes after CO intoxication result in different types of movement disorders.

Some authors have reported that chorea after CO intoxication is usually alleviated by neuroleptics; the fact that the chorea does not relapse after cessation of neuroleptics suggests that these patients may have functional rather than anatomic impairment. [11],[12] However, neuroleptics that may aggravate parkinsonism should be of concern for patients with both chorea and parkinsonism.

HBOT administered within 24 hours after acute CO poisoning can reduce the risk of cognitive sequelae. However, the role of HBOT in delayed neuropsychiatric syndrome after acute CO poisoning is still being debated. Chang et al., reported 9 patients with delayed neuropsychiatric syndrome following CO poisoning, all of whom showed a good response to HBOT. [13] Our patient experienced complete disappearance of the generalized chorea, followed by moderate improvements in cognitive function after HBOT. We speculate that HBOT had greater benefits for demyelinating leukoencephalopathy than globus pallidus lesions in this patient, based on the clinical observation that she completely recovered from the chorea and mental disturbances, but not parkinsonism. Further study is needed to explore the relationship between lesion sites and involuntary movements, as well as the therapeutic mechanisms of HBOT, in patients with delayed encephalopathy after CO intoxication.

 
   References Top

1.
Hsiao CL, Kuo HC, Huang CC. Delayed encephalopathy after carbon monoxide intoxication - long-term prognosis and correlation of clinical manifestations and neuroimages. Acta Neurol Taiwan 2004;13:64-70.  Back to cited text no. 1
    
2.
Cardoso F, Seppi K, Mair KJ, Wenning GK, Poewe W. Seminar on choreas. Lancet Neurol 2006;5:589-602.  Back to cited text no. 2
    
3.
Thom SR. Functional inhibition of leukocyte B2 integrins by hyperbaric oxygen in carbon monoxide-mediated brain injury in rats. Toxicol Appl Pharmacol 1993;123:248-56.  Back to cited text no. 3
    
4.
Chang KH, Han MH, Kim HS, Wie BA, Han MC. Delayed encephalopathy after acute carbon monoxide intoxication: MR imaging features and distribution of cerebral white matter lesions. Radiology 1992;184:117-22.  Back to cited text no. 4
    
5.
Choi IS, Cheon HY. Delayed movement disorders after carbon monoxide poisoning. Eur Neurol 1999;42:141-4.  Back to cited text no. 5
    
6.
Song IU, Chung SW. Chorea as the first neurological symptom of delayed encephalopathy after carbon monoxide intoxication. Intern Med 2010;49:1037-9.  Back to cited text no. 6
    
7.
Warner GT, McAuley JH. Alcohol-induced paroxysmal nonkinesogenic dyskinesia after pallidal hypoxic insult. Mov Disord 2003;18:455-6.  Back to cited text no. 7
    
8.
Li Y, Ren H, Ren M, Cui F, Yang F, Chen Z, et al. Morvan syndrome plus thyroid dysfunction: A case with chronic mercury exposure. Neurol India 2014;62:218-9.  Back to cited text no. 8
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9.
Haug BA, Schoenle PW, Karch BJ, Bardosi A, Holzgraefe M. Morvan′s fibrillary chorea. A case with possible manganese poisoning. Clin Neurol Neurosurg 1989;91:53-9.  Back to cited text no. 9
    
10.
Spitz M, Lucato LT, Haddad MS, Barbosa ER. Choreoathetosis secondary to lead toxicity. Arq Neuropsiquiatr 2008;66:575-7.  Back to cited text no. 10
    
11.
Park S, Choi IS. Chorea following acute carbon monoxide poisoning. Yonsei Med J 2004;45:363-6.  Back to cited text no. 11
    
12.
Davous P, Rondot P, Marion MH, Gueguen B. Severe chorea after acute carbon monoxide poisoning. J Neurol Neurosurg Psychiatry 1986;49:206-8.  Back to cited text no. 12
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13.
Chang DC, Lee JT, Lo CP, Fan YM, Huang KL, Kang BH, et al. Hyperbaric oxygen ameliorates delayed neuropsychiatric syndrome of carbon monoxide poisoning. Undersea Hyperb Med 2010;37:23-33.  Back to cited text no. 13
    


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