Copyright Article author (or theiremployer) 2017. Produced byBMJ Publishing Group Ltd (&BTS) under licence. 2 1 cetd1 eebr2016 December 13 Accepted 2016 April 17 Received [email protected] France; 75012, Paris Saint Antoine, Faubourg du rue 184 Antoine, Saint Universitaire Hôpital Respiratoire, Fonction et Somnologie de Unité Somnologie, Algologie Physiologie Département Launois, H Sandrine Dr to Correspondence France Grenoble, Hospital, University Grenoble Sleep, France Grenoble, University, Alpes Grenoble eateto hsooyand Physiology of Department U1042, Inserm Unit, HP2 tal et M, Joyeux-Faure N, Siyanko cite: To 208786 doi:10.1136/thoraxjnl-2016- Year] Month Day [ First: Online . Thorax ani SH, Launois Thorax OnlineFirst,publishedonJanuary13,2017as10.1136/thoraxjnl-2016-208786 Published laeinclude please lelrhpvniainwt ata rsueof pressure CO partial arterial with hypoventilation revealed gases alveolar blood arterial Morning nocturnal hypoxaemia. severe with events/hour) (AHI)=50 index irpe le,cnrlsepane (AHI=35 apnoea sleep central sleep, disrupted (Apnoea sleep hypopnoeas central revealed and and obtained apnoeas polysomno- was full in-laboratory graphy patient an the complaints, investigate To palsy. sleep dyspha- facial normal dis- as mild a such and eventually sequelae gia resumed neurological was and few with hospital she life, the stay, from hospital charged After recovery. slow lengthy and gradual was a with inten- She unit the care arrest. in sive remained cardiac and a resuscitated successfully experienced patient and vigi- the of lance, impairment bilat- syndrome, but paresis, pontomedullary tetra eral initiated exhibiting deteriorated, was acutely Treatment nystagmus. tures. and for palsy tive spinal cerebral facial and Blood meningism, toms, edce,wt oyms ne f2.7kg/m 21.67 of morning index daily mass body sleep, and a sleep unrefreshing with headaches, com- during fatigue, Clinic episode Sleep daytime pre- choking the she to of time when plaining third 2011, was the until for patient follow-up sented The to treatment. lost then decline sleep to patient persistent the continued showed but polysomnography a hypoventilation daytime gases for 1994, and apnoea clinic blood In to arterial year. return 1 and to in agreed investigationsfollow-up but further therapy refused and patient The kPa. 9.5 itramonocytogenes Listeria following hypoventilation central Acquired DISCUSSION BASED CASE onn reilbodgssaayi,con analysis, gases Once blood palate. arterial and soft morning polysomnography underwent the patient and the diagnosis. palsy, of again, facial respiration of elevation peripheral left ataxic asymmetrical time mild the awake, showed while at examination comorbidity Clinical no and in fever, of had onset follow- patient unit acute care the the intensive time, ing the that in hospitalised At been 1977. in severe cephalitis for except history unremarkable habitual medical was choking Her denied hypersomnolence. She nocturnal and fatigue. our snoring sleep, daytime to poor and presented episodes with woman Clinic 46-year-old Sleep a 1993, In REPORT CASE a report after We years several monocytogenes failure. diagnosed Listeria respiratory CHS, acquired of of cause case rare (CHS) a syndrome is hypoventilation central Acquired INTRODUCTION Pepin Jean-Louis Launois, H Sandrine LM ani SH, Launois 2 ndrc irsoi xma elcul- well as exam microscopic direct on rPaCO or tal et 1,2 . 2 Thorax L)rhombencephalitis. (LM) 1,2 f67kaadPaO and kPa 6.7 of fl i ape eeposi- were samples uid aai Siyanko, Natalia 2017; fl 0:1 ez-iesymp- uenza-like – LM .doi:10.1136/thoraxjnl-2016-208786 3. – Hypopnoea rhomben- fi rming 2 rhombencephalitis 2 of ’ ai Joyeux-Faure, Marie 2 s a orbtgaulyicesdt ec mean 2014. a in reach hours 7 to of increased use events/ gradually nightly but 4.2 poor ventilation of was non-invasive AHI to adherence an Initially hour. showed noc- on to ventilation while slower polysomnogram turnal a were ventilation, 2012, was symptoms In improve. non-invasive daytime ventilation and with the nocturnal alveolar corrected after Although shortly rapidly study. initiated was sleep ventilation turnal essetdyieavoa hypoventilation alveolar with time) sleep daytime total (PaCO of 82% 64 satur- or persistent oxygen min of 287 90% of under ation index spent desaturation time events/hour, oxygen events/hour, ela E le tasuaeu CO (transcutaneous as sleep non-REM REM REM in as noted well during with were breathing events/hour hypoxaemia shallow sustained of 41 periods movement Prolonged and eye sleep. sleep non-rapid during (REM) events/hour 34 w on yoes eet nteae frespira- ( of area centres the tory in defects hypodense showed round MRI two Brainstem testing. genetic by excluded abnormality. testing no function showed pulmonary Standard impairment. 1 info eihrladcnrlreceptors. central and informa- peripheral integrates it from as breathing, tion of for crucial control is the nucleus tract solitary neighbouring the ( blunted was technique, closed-circuit Read iiac ttshv togefc nrespiratory on effect control. strong centre a have states Vigilance oblongata. medulla varia- CO and to networks blood in respond neuronal pons tions which by chemoreceptors, the Central modulated in is located and originates drive from respiratory automatic mammals, In DISCUSSION ihcnrlanesadhppeslsigu to up CO lasting Ventilatory hypopneas s. and 59 apnoeas alternated central breathing with Ataxic available). not was ment stasis. potnsi odon netosadis and infections foodborne opportunistic u assrsiaoycnr yfnto and dysfunction centre respiratory hypoventilation. central survival causes with less compatible However, but be death. may rapid damage in extensive results areas injury these Bilateral infection. of or tumour brain- tethering, been compression, stem have infarction, damage following brainstem reported to secondary CHS of PaCO ventilation maintain minute to allow and adjustments medulla ventral the of ,con ), LM 2 2 saga-oiiebclu htcncause can that bacillus gram-positive a is fi 76 P,PaO kPa, =7.64 nadto ospeci to addition In mn rfudcnrlceosensitivity chemo central profound rming fi 2 ue2 gure hs ti o upiigta cases that surprising not is it Thus, 2 1,2 ees r peda h surface the at spread are levels, 2 .Nnivsv ilvlnoc- bi-level Non-invasive ). 2 eadTamisier, Renaud epne sesdb the by assessed response, 96 P) h H was AHI The kPa). =9.61 PHOX-2B fi eprtr centres, respiratory c 2 n Hhomoeo- pH and hs clinic Chest uainwas mutation 2

measure- 1,2 fi gure

1 2 1

Thorax: first published as 10.1136/thoraxjnl-2016-208786 on 13 January 2017. Downloaded from from Downloaded 2017. January 13 on 10.1136/thoraxjnl-2016-208786 as published first Thorax: http://thorax.bmj.com/

on October 1, 2021 by guest. Protected by copyright. by Protected guest. by 2021 1, October on Chest clinic Chest clinic iual hnteteti delayed. par- is outcome, treatment fatal a when to leading ticularly often appears, dysfunction stem enepce ausaesonin circles. shown open are values expected Mean ainsdvlpn hmecpaii,a a h aefrour for in case absent non-speci the a usually was After are patient. as rhombencephalitis, factors developing predisposing patients listeriosis, of forms bcs rrobnehlts(rbantmencephalitis). brainstem (or cerebellar meningitis, rhombencephalitis encephalitis, to or lead abscess and (CNS) system barrier, blood-brain the cross hrb eaigtml igoi.BanC a enra or normal be may CT Brain diagnosis. timely delaying cerebrospinal thereby neuro- for negative and displaying be blood may often initial samples patients Although decreases sequelae. with rate logical mortality 30%, the approximately care, to supportive and therapy biotic soitdwt ihmraiyrate. mortality high a with associated 1.5– is for laboratory range the normal The circles). Hg (closed L/min/mm 0.6 was slope patient response The 2011. in performed oe ttepnielvladtemdlaoblongata. medulla 2 the and level is pontine atrophy the cerebellar at left noted severe the Moreover, in arrows). defects (see hypodense brainstem small severe several after showing years rhombencephalitis, 34 2011, in performed 2 Figure humans. in infection uncommon an unknown, is remains cephalitis prevalence exact its Although iue1 Figure hs clinic Chest rnvre()admdsgta B eerlMIscans MRI cerebral (B) mid-sagittal and (A) Transverse etltr epnet CO to response Ventilatory . /i/mHg. L/min/mm 3.5 fi rdoa hs,eiec fbrain of evidence phase, prodromal c ’s utrsaeeetal positive eventually are cultures LM, LM 2 a nettecnrlnervous central the infect can itramonocytogenes Listeria 3 3 ihpoe al anti- early proper With eas fisaiiyto ability its of Because 3 LM nieother Unlike rhomben- fl uid 3 iaea utpeasesslctdi h rise n cultures and for brainstem positive the in are located abscesses multiple bilateral h te,lk u ain,srie u otne orequire to continued but sleep. survived during patient, support our ventilator like other, the non-speci show may Autopsy the cerebellum. in in the abscesses and multiple stem reveals brain typically MRI but inconclusive ru gis ogntlcuewihwudntehbtthese exhibit not abnormalities. would MRI the which of after cause initial which types congenital years the lesions, 34 a brainstem of against hypodense obtained time argue two scans the revealed episode MRI at acute performed cerebral been but had infection scans MRI nor riayrcvr.T u nweg,ti aei nytethird hypoventilation. the central only is case of this report knowledge, extra- our her To recovery. for accounting ordinary likely unilateral, were lesions brainstem itr fsvr hmecpaii,MIbantmlesions the brainstem of sequel MRI a be rhombencephalitis, to considered severe were the of CHS: congenital de history case, clearly to our of compared of presence unusual aspect quite One was hypoventilation. however, central acquired of sis MRI and essadCSifcinbtas eas fteeteedelay extreme initial the severe of of because spite also in but infection outcome CNS positive and the sepsis of because only inwt xrm mareto etltr epnet CO to response ventilatory of impairment extreme with tion kont as ogntlCHS congenital cause to without (known hypoventilation alveolar daytime patient. our this in Fortunately, case the individuals. not was immunocompromised par- mortality, excess in to lead ticularly may delay This inconclusive. be may spinal eae eas yposaenon-speci are symptoms because delayed aey7mnh ae nsieo otra ventilation, nocturnal of spite in later infection, with months 7 acute diagnosed were the mately these patients during for previous syndrome two the different The quite patients. was three hypoventilation central acquired fl txcrsiaindrn aeuns n le,marked sleep, and wakefulness during respiration Ataxic mainbtuulycon usually but ammation fl ani SH, Launois i ieteaiainaeotnngtv n To MRI or CT and negative often are examination direct uid fi LM dnsi u ain eecnitn ihadiagno- a with consistent were patient our in ndings rise ein edn oaqie secondary acquired to leading lesions brainstem tal et LM. . 3 Thorax fi norptet ete eerlC scan CT cerebral neither patient, our In e poa n yona.Gvnthe Given hypopneas. and apnoeas ned 56 2017; 4 h oreadotoeo the of outcome and course The fi m h rsneo nltrlor unilateral of presence the rms igoi n ramn a be may treatment and Diagnosis 0 :1 6 – 1 .doi:10.1136/thoraxjnl-2016-208786 3. ,anra umnr func- pulmonary normal a ), u aei eakbenot remarkable is case Our LM fi 56 ,bodadcerebro- and blood c, bcs.Tepatient The abscess. PHOX-2B n idapproxi- died one mutation 5 while fi

2 ’

c s ,

Thorax: first published as 10.1136/thoraxjnl-2016-208786 on 13 January 2017. Downloaded from from Downloaded 2017. January 13 on 10.1136/thoraxjnl-2016-208786 as published first Thorax: http://thorax.bmj.com/ on October 1, 2021 by guest. Protected by copyright. by Protected guest. by 2021 1, October on le ihoengtplsmorpy reilbodgssand gases blood testing. chemo-sensitivity arterial polysomnography, overnight during with evaluated sleep be should status respiratory rhombencephalitis, a eacueo death. of cause of a phase be acute may the during abnormalities Respiratory common occur. are to likely are rhombencephalitis noc- bi-level non-invasive hypoventilation. alveolar initiate correct to and ventilation years turnal 10 the additional of an Because took episode. acute the patient after years 15 approximately the for presented fi treatment patient and Our hypoventilation. diagnosis central acquired the of and rhombencephalitis the between ani SH, Launois alr uigaashsao ihcnurn umnr infec- use. respiratory pulmonary sedative concurrent acute or central with opioid for late-onset tion, or risk with anaesthesia at case during are the failure patients is impairment As such control lesions. hypoventilation, brainstem ventilatory to subclinical due display but recover s iet h le lncwt upco fsepapnoea sleep of suspicion a with Clinic Sleep the to time rst ihteicdneo itroi iig oecssof cases more rising, listeriosis of incidence the With ’ eutnet useivsiain n ramn,it treatment, and investigations pursue to reluctance s tal et . Thorax 2017; 0:1 3 – usto ainsmyapparently may patients of subset A .doi:10.1136/thoraxjnl-2016-208786 3. 1 hs ntercvr hs of phase recovery the in Thus, LM N neto and infection CNS LM LM eda L icelG,Nti E rahn:ryhiiy lsiiy chemosensitivity. plasticity, rhythmicity, Breathing: EE. Nattie GS, Mitchell JL, Feldman 2 lebre ,Wge .Lseiss eugn odon infection. foodborne resurgent a Listeriosis: M. Wagner F, Allerberger 3 es-ae E er-rvsE,Cchrn I, Ceccherini EM, Berry-Kravis DE, Weese-Mayer 1 REFERENCES review peer and Provenance consent Patient revisions. interests manuscript Competing and care patient the to contributed Contributors ihu ,BradnG oe PM, Roger G, Bernardin D, Milhaud Ondine P. Brodersen 6 PB, Hansen TH, Jensen 5 CM, Rand MS, Carroll PP, Patwari 4 ttmn:cneia eta yoetlto ydoe eei ai,danss and diagnosis, basis, genetic management. syndrome: hypoventilation central congenital statement: nuRvNeurosci Rev Annu maretdet itrarobnehltssequelae]. rhombencephalitis 1999;155:152 Listeria to due impairment encephalitis. stem dysregulation. autonomic and respiratory of model Neurobiol a Physiol gene: PHOX2B the and Infect 2010;16:16 H a novdi aucitdatn.SL S JF TadJLP and RT MJ-F, NS, SHL, drafting. manuscript in involved was SHL mJRsi rtCr Med Care Crit Respir J Am – Obtained. 4. 2010;173:322 – caNuo Scand Neurol Acta 23. 2003;26:239 oedeclared. None o omsind xenlype reviewed. peer externally commissioned; Not – – 35. 66. tal et tal et 1988;77:505 ogntlcnrlhpvniainsyndrome hypoventilation central Congenital . Cnrlanawt consciousness with apnea [Central . 2010;181:626 ’ us in curse s tal et – 6. .Anof itramonocytogenes listeria – e erl(Paris) Neurol Rev 44. fi ilASciia policy clinical ATS cial hs clinic Chest lnMicrobiol Clin Respir brain

3

Thorax: first published as 10.1136/thoraxjnl-2016-208786 on 13 January 2017. Downloaded from from Downloaded 2017. January 13 on 10.1136/thoraxjnl-2016-208786 as published first Thorax: http://thorax.bmj.com/

on October 1, 2021 by guest. Protected by copyright. by Protected guest. by 2021 1, October on Chest clinic