Background: Status dystonicus (SD) is the term used for extreme, continuous, generalized muscle contractions that are poorly responsive to treatment. Here, we report a rare case of acute hypoxic ischemic encephalopathy presenting with SD.

Case Report: A young male sustained cerebral hypoxia following a cardiac event and presented with opisthotonic posturing and dystonia refractory to medical therapy. His serum creatine phosphokinase was high and his urine tested positive for myoglobin.

Discussion: SD as an acute sequelae following acute brain hypoxia is rare. Management of brain anoxia is challenging, even more so when the presentation is compounded by refractory SD.

Keywords: Dystonia, emergency, hypoxia, status, anoxia

Citation: Ray S, Sidhu RJS, Pal PK, Yadav R. Status dystonicus as an acute sequelae following anoxic cerebral damage. Tremor Other Hyperkinet Mov. 2019; 9. doi: 10.7916/98r0-743

Pal, Pramod Kumar

Ray, Somdattaa

Sidhu, Ravinder Jeet Singh

Yadav, Ravi

English

Columbia University Academic Commons

Case ReportsStatus Dystonicus as an Acute Sequelae Following Anoxic Cerebral DamageSomdattaa Ray, Ravinder Jeet Singh Sidhu, Pramod Kumar Pal & Ravi Yadav*Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, INAbstractBackground: Status dystonicus (SD) is the term used for extreme, continuous, generalized muscle contractions that are poorly responsive to treatment. Here,we report a rare case of acute hypoxic ischemic encephalopathy presenting with SD.Case Report: A young male sustained cerebral hypoxia following a cardiac event and presented with opisthotonic posturing and dystonia refractory to medicaltherapy. His serum creatine phosphokinase was high and his urine tested positive for myoglobin.Discussion: SD as an acute sequelae following acute brain hypoxia is rare. Management of brain anoxia is challenging, even more so when the presentation iscompounded by refractory SD.Keywords: Dystonia, emergency, hypoxia, status, anoxiaCitation: Ray S, Sidhu RJS, Pal PK, Yadav R. Status dystonicus as an acute sequelae following anoxic cerebral damage. Tremor Other Hyperkinet Mov. 2019; 9.doi: 10.7916/98r0-7438*To whom correspondence should be addressed. E-mail: docravi20@yahoo.comEditor: Elan D. Louis, Yale University, USAReceived: December 21, 2018 Accepted: January 17, 2019 Published: April 15, 2019Copyright: ’ 2019 Ray et al. This is an open-access article distributed under the terms of the Creative Commons Attribution–Noncommercial–No Derivatives License, which permitsthe user to copy, distribute, and transmit the work provided that the original authors and source are credited; that no commercial use is made of the work; and that the work is not alteredor transformed.Funding: None.Financial Disclosures: None.Conflict of Interest: The authors report no conflict of interest.Ethics Statement: All patients that appear on video have provided written informed consent; authorization for the videotaping and for publication of the videotape was provided.IntroductionContinuous or intermittent muscle contractions resulting in abnor-mal and repeated patterened movements characterize dystonia thatcan progressively worsen causing extreme, generalized continuousspasms termed status dystonicus (SD).1–3,24 SD is a potentially life-threatening crisis. Rhabdomyolysis, myoglobinuria, and hyperpyrexiaare a few additional features that may be associated with SD.4,5 Secon-dary dystonia is the most common underlying cause of SD, withcerebral palsy the cause in 59.3% of patients.6Vascular and metabolic theories have been proposed as the causeof sensitivity of the basal ganglia to anoxic damage. Certain vasculardomains of the basal ganglia are hypoperfused, making it veryvulnerable to hypoxia. Hypoxia has been shown to increase striatalextracellular glutamate. The high oxidative metabolism of striatummakes it prone to vascular damage.7The time of onset of dystonia following cerebral anoxia after theperinatal period is variable, ranging from 1 week to 36 months.8 Wedescribe a 13-year-old male who developed SD as an acute mani-festation of cerebral anoxia following cardiac arrest.Case reportA 13-year-old male with no known comorbidities was well until20 days before admission to the hospital. While cycling back fromschool he had developed sudden onset of altered sensorium with loss ofconsciousness. The patient was given cardiopulmonary resuscitationand revived and subsequently intubated. An echocardiogram showedregional wall motion abnormalities in the anterior wall of the leftanterior descending artery, suggesting anterior wall myocardialinfarction, and an ejection fraction of 40%. He received aspirin andenoxaparin. Magnetic resonance imaging (MRI) of the brain showedbilateral cortical swelling, fluid-attenuated inversion recovery (FLAIR)and T2 hyperintensities in bilateral thalami, posterior putamen withrestriction on diffusion-weighted images suggesting hypoxic-ischemicdamage (Figure 1). On the 10th day after the first symptom, the patientdeveloped abnormal posturing of all four limbs with hyperextensionof the back (Video 1). On the 16th day, opisthotonic posturing andabnormal posturing of the limbs worsened with no triggering factors.The patient had one episode of tonic–clonic seizure. He had no priorhistory of chest pain, palpitation, exertional breathlessness, or syncope.Freely available onlineTremor and Other Hyperkinetic Movementshttp://www.tremorjournal.org Columbia University Libraries1There was no family history of dystonia or any other movementdisorders. The patient was examined on the 20th day after the firstsymptom. On examination, the patient was drowsy and did notrespond to painful stimuli. His pupils were equally bilaterally reactive.The oculocephalic reflex was present. There was no autonomicdysfunction.Investigations revealed a creatine phosphokinase (CPK) value of1,499 IU/L, which progressively increased to 2,411 IU/L in the next10 days, and his urine was persistently positive for myoglobin. Serumhomocysteine was elevated (24.96 mmol/L). Electroencephalography(EEG) suggested diffuse cerebral dysfunction. A repeat echocardio-gram showed left-ventricle segmental hypokinesia and the ejectionfraction was 47%.The patient was first started on intravenous promethazine 25 mgtwice daily and haloperidol 2.5 mg four times a day. Owing to a lackof improvement, he was subsequently started on tetrabenazine andthen trihexylphenidyl. The patient was then intubated and started ondiazepam 5 mg per day, increasing to 15 mg per day. There wasa significant improvement with tetrabenazine and trihexyphenidyl;the doses were increased to tetrabenazine 75 mg per day and trihexy-phenidyl 18 mg per day in divided doses. He was also started on oralbaclofen 20 mg per day and levodopa/carbidopa 110 mg three timesa day. The patient was started on adequate rehydration and antipyreticsfor fever and intravenous levetiracetam for seizure. For cardiacdysfunction, dysfunction, he was treated with dual antiplatelettherapy and digoxin 0.25 mg, half a tablet once daily for five daysa week. The QT interval was monitored while the patient was ontetrabenazine and there was no prolongation of the QT interval.The patient had a significant reduction in generalized dystonia bythe ninth day of admission to the intensive care unit (ICU), and showedno opisthotonic or dystonic spasms by the 11th day of ICU care.At discharge, the patient was bed bound, his eyes opened spontaneously,all four limbs moved spontaneously, dystonia was less severe, andthere was no speech output. The patient was lost to follow-up 6 monthsafter discharge.Figure 1. Selective MRI Image is Displayed with Salient Features.(A) The axial T2 fluid-attenuated inversion recovery sequence showinghyperintensity in the putamen bilaterally suggesting anoxic injury.Video 1. This Video Grab shows the Opisthotonic Dystonic Posturingin the Patient Following Anoxic Brain Injury. The patient is seenexperiencing opisthotonic posturing and generalized dystonia.Ray S, Sidhu RJS, Pal PK, et al. Status Dystonicus Following Anoxic Cerebral DamageTremor and Other Hyperkinetic Movementshttp://www.tremorjournal.org Columbia University Libraries2DiscussionNon-progressive cerebral insults such as perinatal hypoxia andkernicterus can cause chorea, athetosis, and dystonia while cerebralinfarction has resulted in ballistic choreic or athetoid movements.9,10Anoxic brain damage can also cause action myoclonus, tremors, andakinetic rigid syndrome.14,15,8 Involuntary movements may develop atthe time of insult or following resolution of acute neurological deficits.In the case of perinatal damage, abnormal movements appear afterneurological maturation. Children with perinatal asphyxia showed theearliest movement disorders, at 10–18 months of age.12 Dystonia hasbeen associated with lesions of the contralateral putamen, posteriorand postero-lateral external globus pallidus, and red nucleus.11 In acase series, putamen lesions were found in 14 patients of whom10 patients had dystonia.8 In a review of 12 patients who sufferedischemic insult, six patients developed a pure dystonic syndromeranging from 1 week to 36 months after the onset of hypoxia that wasgradually progressive. Four patients initially developed akinetic–rigid syndrome followed by dystonia.8 The age of occurrence ofanoxic injury is vital in predicting the outcome, with akinetic–rigidsyndrome developing in older patients and dystonia developing inyounger patients. The pathophysiological mechanisms underlyingage-dependent differences may be because ageing of the nigrostriatalsystem renders older individuals more susceptible to parkinsonismfollowing anoxia.8SD starting shortly after cerebral anoxia has seldom been reported.Bhatt et al.8 reported four patients who developed dystonia rangingfrom 1–2 weeks after the occurrence of cerebral anoxia, but thesepatients were not reported to have SD.8 Our patient developeddystonia and opisthotonic posturing on the 10th day of cerebral anoxicinsult, which worsened on the 16th day and on the 20th day. At thetime of examination in the casualty department, he was experiencingcontinuous dystonic spasms with severe opisthotonus. Pierro et al.16reported a 14-month-old female who suffered cerebral anoxia afternearly drowning. On day 12 she developed decorticate posturing,dystonia, and opisthotonus and torsion spasms and subsequently wastransferred to the ICU on day 41 because of SD. She recovered fromher dystonic spasms within the next 20 days. Despite the recovery shewas still in a vegetative state. Another child was reported, a 15-monthmale who developed torsion spasms on day 5 after a cerebral anoxicinsult. After 3 months, his dystonia progressively worsened culminatingin SD requiring an ICU stay of 6 months.16 A 19-month-old child wasreported to have had developed SD 2 weeks after anoxic braindamage.25 Hence, to our knowledge, our patient is the third patientreported in the literature presenting with SD as an acute manifestationof cerebral anoxia.SD has been described in various conditions such as pantothenatekinase-associated neurodegeneration, perinatal hypoxia, megalencepha-lic leukoencephathy with subcortical cysts, primary generalized dystonia,Wilson disease, ataxia telangiectasia, and Aristaless related homeobox(ARX) syndrome.1,17–23 Fever, infections, exposure to medication, or itsabrupt cessation can be triggering factors.18 The tonic type of SD has beenmore commonly seen in males and is associated with higher mortality.6A new definition has been proposed for SD in view of the concurrentoccurrence of many hyperkinetic disorders with dystonia.26ConclusionSD is rare and life-threatening complication of dystonia in bothprimary and secondary dystonia and can be a presenting manifestationin patients who have suffered cerebral anoxia. Management of preci-pitating factors, intensive care, and treatment with multiple anti-dystonia medications are cardinal to overcome the crisis at the earliest.AcknowledgmentWe acknowledge the patient for his support and co-operation.References1. Opal P, Tintner R, Jankovic J, Leung J, Breakefield XO, Friedman J,et al. Intrafamilial phenotypic variability of the DYT1 dystonia: from asympto-matic TOR1A gene carrier status to dystonic storm. Mov Disord 2002;17:339–345. doi: 10.1002/mds.100962. Dalvi A, Fahn S, Ford B. Intrathecal baclofen in the treatment of dystonicstorm. Mov Disord 1998;13:611–612. doi: 10.1002/mds.8701303443. Vaamonde J, Narbona J, Weiser R, Garcia MA, Brannan T, Obeso JA.Dystonic storms: a practical management problem. Clin Neuropharmacol 1994;17:344–347. doi: 10.1097/00002826-199408000-000064. Jankovic J, Penn AS. Severe dystonia and myoglobinuria. Neurology 1982;32:1195. doi: 10.1212/WNL.32.10.11955. Marsden CD, Marion MH, Quinn N. 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Status Dystonicus as an Acute Sequelae Following Anoxic Cerebral Damage

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Status Dystonicus as an Acute Sequelae Following Anoxic Cerebral Damage

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