Chrysostomos Katsenos

Costas Mandragos

Despoina Androulaki

E Marchioni

H Lassmann

J de Seze

J Khan

MA Lana-Peixoto

NL Sicotte

R Sonneville

RC Dale

RK Garg

S Love

Stavroula Lyra

Theodoros Tsoutsouras

BMC Research Notes

English

Crossref

Katsenos et al. BMC Research Notes 2013, 6:22http://www.biomedcentral.com/1756-0500/6/22CASE REPORT Open AccessA 17 year-old girl with a demyelinating diseaserequiring mechanical ventilation: a case reportChrysostomos Katsenos1*, Despoina Androulaki1, Stavroula Lyra2, Theodoros Tsoutsouras1 and Costas Mandragos1AbstractBackground: Demyelinating diseases cause destruction of the myelin sheath, while axons are relatively spared.Pathologically, demyelination can be the result of an inflammatory process, viral infection, acquired metabolicderangement and ischemic insult. Three diseases that can cause inflammatory demyelination of the CNS are:Multiple sclerosis (MS), Acute disseminated encephalomyelitis (ADEM) and Acute hemorrhagic leucoencephalitis.Differentiation is not always easy and there is considerable overlaping. Data about adults with acute demyelinationrequiring ICU admission is limited.Case presentation: A 17 year old Greek female was hospitalised in the ICU because of acute respiratory failurerequiring mechanical ventilation. She had a history of febrile disease one month before, acute onset of paraplegia,diplopia, progressive arm weakness and dyspnea. Her consciousness was not impaired. A demyelinating centralnervous system (CNS) disease, possibly post infectious encephalomyelitis (ADEM) was the underlying condition. TheMRI of the brain disclosed diffused expanded cerebral lesions involving the optic nerve, basal ganglia cerebellum,pons and medulla oblongata. There was also extended involvement of the cervical and thoracic part of the spinalcord. CSF leukocyte count was elevated with lymphocyte predominance. The patient required mechanicalventilation for two months. Then she was transferred to a rehabilitation centre. Three years later she remainsparaplegic. Since then she has not suffered any other demyelination attack.Conclusions: Demyelinating diseases can cause acute respiratory failure when the spinal cord is affected. Severeforms of these diseases, making necessary ICU admission, is less frequently reported. Intensivists should be aware ofthe features of these rare diseases.BackgroundThe term demyelination describes a destruction of themyelin sheath. Axons that have been demyelinated, se-condarily, tend to atrophy and degenerate. Demyelinationaccording to pathogenesis can be classified into several ca-tegories: inflammatory, viral, metabolic, hypoxic-ischemicand compressive [1].Inflammatory demyelination occurs in multiplesclerosis (MS), acute disseminated encephalomyelitis(ADEM) and acute hemorrhagic leucoencephalitis(AHL). MS can be classified, according to clinical andpathological features, into three main subtypes: clas-sical, acute and concentric sclerosis. Devic’s disease(neuromyelitis optica) is correlated with a specific* Correspondence: kats_ch@otenet.gr1Korgialenio-Benakio Red Cross Hospital of Athens, ICU, Athanasaki 1 str,Ampelokipoi 11526, Athens, GreeceFull list of author information is available at the end of the article© 2013 Katsenos et al.; licensee BioMed CentrCommons Attribution License (http://creativecreproduction in any medium, provided the orautoantibody (NMO-IgG). It was considered a variantof MS, but recent pathological, immunopathogenicand imaging studies suggest distinctive features [2].Although there are specific pathological featuresassociated with these diseases, differential diagnosisis mainly based on clinical and medical imaginggrounds. In addition, connective tissue diseases, bac-terial and viral infections and other conditions, cancause transverse myelopathy.Excluding Devic’s disease, there is no biological markerthat can help differentiate these diseases. Clinical fea-tures depend on the region of the CNS that is affectedand on the extent of demyelination. Generally, MS ismore common in adults, tends to relapse, and is usuallymonosymptomatic. While ADEM is more common inchildren, frequently accompanying encephalopathy withseizures. It is not relapsing and follows a history of pre-cipitating infection within two weeks. Optic neuritis isal Ltd. This is an Open Access article distributed under the terms of the Creativeommons.org/licenses/by/2.0), which permits unrestricted use, distribution, andiginal work is properly cited.Figure 2 A. Flair axial of the brain; extensive lesions mainly inbrain stem and cerebellum. B: Spine T2 sagital. Extensiveconfluent lesions extending cranially up to the medulla oblongataand caudally to the middle thoracic spine.Katsenos et al. BMC Research Notes 2013, 6:22 Page 2 of 4http://www.biomedcentral.com/1756-0500/6/22unilateral in MS and bilateral in ADEM. The CSF exam-ination commonly shows oligoclonal IgG in MS andlymphocytosis with increased protein in ADEM, whilethe MRI shows disseminated inflammatory lesions,which are periventricular or callosal in MS and corticalor in deep gray matter in ADEM.When the spinal cord is involved in ADEM, lesionsare large, swollen and thoracic, whereas in MS they areclearly smaller, more discrete and cervical. The followup can distinguish the two diseases. In ADEM newlesions should not occur and original lesions areexpected to resolve partially or completely. If a newlesion occurs after the acute/subacute phase, a diagnosisof MS is more probable. In many cases, however, thefinal diagnosis can only be made after a considerablylong period and follow up examinations [3-7]. AlthoughADEM in adults has been studied, there is limited dataof severe forms requiring ICU admission. Commoncauses for referral to ICU are seizures, impaired con-sciousness and fever. Prognosis is poor and survivorshave functional impairments [8].Case presentationA 17 year old Greek female was hospitalised in the ICUbecause of acute respiratory failure requiring mechanicalventilation. A degenerative central nervous system (CNS)disease, possibly post infectious encephalomyelitis (ADEM)was the underlying condition. Upon hospital admission thepatient complained of headache, low back pain and fever.Within days, the patient progressively deteriorated withmuscle weakness in the upper and lower extremities.The MRI of the brain was normal (Figure 1A), but thecervical and upper thoracic MRI revealed intramedullaryinflammatory lesions extending from C3 to Th5(Figure 1B). The patient was treated with high doses ofFigure 1 A. MRI Flair axial of the brain; normal. B: T2 sagitalcervical and upper thoracic spine; intramedullary lesions of cervicaland thoracic spine C3 –Th5.corticosteroids (methylprednisolone 1gr per day for4 days), with no response. Gradually, visual disturbancesdeveloped and the patient complained of dyspnea. Anoxygen saturation of 85% was observed by pulse oxyme-try, and oxygen administration (Venturi mask 50%) fol-lowed. Her condition rapidly deteriorated. The patientwas intubated immediately, supported with mechanicalventilation and transferred to the ICU. (No spirometricevaluation or blood gas analysis was performed).Intravenous immune globulin (IVIG) treatment wasadministered (30 grams per day for 4 days). An MRI ofthe brain revealed diffused extensive lesions, mainly inbrain stem and cerebellum, with a high T2 signal com-patible with myelitis (Figure 2A). The MRI of the spinerevealed extensive confluent lesions extending craniallyup to the medulla oblongata and caudally to the middleof the thoracic spine (Figure 2B). CSF was abnormalFigure 3 A. Flair axial brain; normal after four months.B: Cervical spine T2 sagital. After four months, most of theintramedullary lesions have been disappeared.Katsenos et al. BMC Research Notes 2013, 6:22 Page 3 of 4http://www.biomedcentral.com/1756-0500/6/22(WBC = 240/ml, mainly lymphocytes, total protein =750 mg/dl, glucose ratio CSF/serum = 45/92 mg/dl andno oligoclonal bands of IgG). CSF PCR examinationwas negative for HSV1,2, enteroviruses, n. meningitis,streptococcus pneumonia, listeria, haemophilus in-fluenza, TBC. In the ICU no additional data was found byrepeated cerebral MRI. Neutropenia developed and bonemarrow examination was performed, which disclosed anormal bone marrow. The neutropenia probably was dueto neutrophil’s peripheral destruction, as the patient suf-fered from severe sepsis, due to ventilator associatedpneumonia. All attempts to liberate the patient from theventilator failed, as the patient could not use the intercos-tal muscles. A new CSF examination showed normal,serum NMO-IgG, ANA and ACE were negative. Thepatient underwent tracheostomy and gastrostomy.A month later, the patient’s condition graduallyimproved, allowing her exemption from the ventilator. Anew MRI of the brain was normal (Figure 3A) and mostof the intramedullary lesions had vanished (Figure 3B).Her consciousness level improved. Some motion wasobserved in her arms. Although blindness remained inher right eye, gradual improvement allowed closure oftracheostomy and gastrostomy. However she could notmove her lower extremities and had no sense of touch.During the last weeks of hospitalisation she had a per-sistent bacteriuria by multidrug resistant gram(−) bac-teria. She also experienced a right pneumothorax,conservatively treated with success.Three years after discharge the patient is still paraple-gic despite the resolution of the lesions in MRI. Shecomplains of a bilateral loss in her senses of heat andtouch at the level of the thighs.DiscussionDifferential diagnosis between demyelinating diseases isnot easy due to a lack of specific biological markers. In ourcase, the history of a febrile disease a month before the de-velopment of neurological symptoms supports the diagno-sis of ADEM. Our patient did not suffer any demyelinationattack during the three years of follow up. This is also sup-portive to the diagnosis of ADEM, which is monophasicillness as opposed to relapsing-remitting MS. Contrary tothis diagnosis is the fact that patient’s consciousnessremained intact while she had no seizures. Devic’s diseasewas ruled out by the absence of the NMO-IgG.A possible cause of the acute respiratory failure is theextensive spinal cord involvement, as the patient underclinical examination displayed prolonged intercostalmuscle weakness. Unfortunately respiratory functiontesting was not done before intubation and the respira-tory failure was not well studied.A persistent paraplegia along with a loss of senses inheat and touch despite MRI normalisation is anotherinteresting point worthy of observation. One possible ex-planation is the development of critical illness polyneu-ropathy (CIP) and myopathy (CIM) during the prolongedICU stay. These two clinical entities may occur concomi-tantly and are associated with sepsis, multiorgan failureand possibly with corticosteroid treatment [9].Our patient was treated with high doses of corticoste-roids and suffered many times from sepsis and severesepsis during her long ICU hospitalisation. Unfortu-nately, she was not available for nerve conduction stu-dies and electromyography.Another explanation is that the traditional MRI in MSand in other demyelinating diseases has a poor correl-ation with clinical disability. The MRI can detectchanges in inflammatory activity but for quantificationof intact myelin and remyelination, magnetization trans-fer imaging has been proposed. Diffusion tensor imagingcan be used to monitor tract-specific changes that maybe more closely linked to clinical measures and may beparticularly powerful when combined with functionalmeasures, such as functional MRI [10,11].ConclusionSevere forms of demyelinating diseases, requiring ICUadmission, bear a poor prognosis. Clinical recovery maybe limited despite resolution of imaging changes.ConsentWritten informed consent was obtained from the pa-tient, who is now 20 years old, for publication of thiscase report and accompanying images. A copy of thewritten consent is available for review by the Editor-in-Chief of this journal.AbbreviationsMRI: Magnetic resonance imaging; ADEM: Acute disseminatedencephalomyelitis; MS: Multiple sclerosis; CSF: Cerebrospinal fluid;ANA: Antinuclear antibodies; ACE: Angiotensin converting enzyme;CIP: Critical illness polyneuropathy; CIM: Critical illness myopathy.Competing interestsThe authors declare that they have no competing interests.Authors’ contributionsCK, cared for the patient and co-ordinated the presentation. DA reviewedthe laboratory test results and prepared the case presentation. SL, reviewedthe MRI and provided the legends. TT did the follow up of the patient andcontributed in writing. CM cared for the patient and supervised the writingof the manuscript. All authors read and approved the final manuscript.AcknowledgementsThe authors acknowledge the patient for her kind permission to use hermedical file for this publication.The authors have no financial funding to disclose for this publication.Author details1Korgialenio-Benakio Red Cross Hospital of Athens, ICU, Athanasaki 1 str,Ampelokipoi 11526, Athens, Greece. 2Korgialenio-Benakio Red Cross Hospitalof Athens, MRI UNIT, Athanasaki 1 str, Ampelokipoi 11526, Athens, Greece.Katsenos et al. BMC Research Notes 2013, 6:22 Page 4 of 4http://www.biomedcentral.com/1756-0500/6/22Received: 21 May 2012 Accepted: 2 January 2013Published: 18 January 2013References1. Love S: Demyelinating diseases. J Clin Pathol 2006, 59:1151–1159.2. Lana-Peixoto MA: Devic’s neuromyelitis optica. Arq Neuropsiquiatr 2008,66(1):120–138.3. Dale RC, Branson JA: Acute disseminated encephalomyelitis or multiplesclerosis: can the initial presentation help in establishing a correctdiagnosis? Arch Dis Child 2005, 90:636–639.4. Lassmann H: Acute disseminated encephalomyelitis and multiplesclerosis. Brain 2010, 133:317–319.5. Garg RK: Acute disseminated encephalomyelitis. Postgrad Med J 2003,79:11–17.6. Marchioni E, Bono G, Tavazzi E, Antinori A, Minoli L, Ceroni M: Recruitmentcriteria for acute disseminated encephalomyelitis studies: the need forconsensus. Neurol Sci 2008, 29(4):203–204.7. de Seze J, Debouverie M, Zephir H, Lebrun CH, Blanc F, Bourg V, Wiertlewski S,Pittion S, et al: Acute fulminant demyelinating disease: a descriptive study of60 patients. Arch Neurol 2007, 64(10):1426–1432.8. Sonneville R, Demeret S, Klein I, Bouadma L, Mourvillier B, Audibert JSLegriel S, Bolgert F, Regnier B, Wolff M: Acute disseminatedencephalomyelitis in the intensive care unit: clinical features andoutcome of 20 adults. Intensive Care Med 2008, 34(3):528–532.9. Khan J, Harrison TB, Rich MM: Mechanisms of neuromuscular dysfunctionin critical illness. Crit Care Clin 2008, 24(1):165–177. x.10. Sicotte NL: Neuroimaging in multiple sclerosis: neurotherapeuticimplications. Neurotherapeutics 2011, 8(1):54–62.11. Sicotte NL: Magnetic resonance imaging in multiple sclerosis: the role ofconventional imaging. Neurol Clin 2011, 29(2):343–356.doi:10.1186/1756-0500-6-22Cite this article as: Katsenos et al.: A 17 year-old girl with ademyelinating disease requiring mechanical ventilation: a case report.BMC Research Notes 2013 6:22.Submit your next manuscript to BioMed Centraland take full advantage of: • Convenient online submission• Thorough peer review• No space constraints or color ﬁgure charges• Immediate publication on acceptance• Inclusion in PubMed, CAS, Scopus and Google Scholar• Research which is freely available for redistributionSubmit your manuscript at www.biomedcentral.com/submit

A 17 year-old girl with a demyelinating disease requiring mechanical ventilation: a case report

https://bmcresnotes.biomedcentral.com/track/pdf/10.1186/1756-0500-6-22?site=bmcresnotes.biomedcentral.com

A 17 year-old girl with a demyelinating disease requiring mechanical ventilation: a case report

Abstract

Similar works

Full text

Available Versions

Crossref