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Letter to the Editor
Reversible delayed post-hypoxic leukoencephalopathy
Leucoencefalopatía posthipóxica diferida reversible
M. Taintaa,
Corresponding author
mikeltainta@gmail.com

Corresponding author.
, P. de la Rivaa, M.Á. Urtasuna, J.F. Martí-Massóa,b,c
a Servicio de Neurología, Hospital Universitario Donostia, San Sebastián, Guipúzcoa, Spain
b Facultad de Medicina, Universidad País Vasco, San Sebastián, Guipúzcoa, Spain
c Área de Neurociencias, Instituto Biodonostia, San Sebastián, Guipúzcoa, Spain
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and a chest radiography revealed consolidations at both lung bases&#59; the results of a cranial CT scan and a blood test were normal&#46; A urine test revealed presence of benzodiazepines&#44; opioids&#44; and cannabis&#46; His personal history included paranoid schizophrenia&#44; beginning in adolescence and treated with olanzapine and amisulpride&#46; He also occasionally consumed opioid and sedative drugs&#44; cannabis&#44; and cocaine&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Two days after admission&#44; his level of consciousness recovered&#59; a neurological examination yielded normal results&#46; As a complication&#44; the patient developed a respiratory infection&#44; which responded well to antibiotics&#44; and acute renal damage secondary to an increased level of creatine kinase &#40;8277<span class="elsevierStyleHsp" style=""></span>U&#47;L&#41;&#44; due to probable rhabdomyolysis&#46; Twenty-one days after admission&#44; his condition suddenly worsened&#44; with the development of somnolence and bradypsychia&#46; He progressively began to present stereotypic movements&#44; motor disinhibition&#44; and reduced verbal communication&#44; with diminished fluency and impaired comprehension&#46; All limbs presented cogwheel rigidity&#46; Myotatic reflexes were normal and plantar reflexes were flexor bilaterally&#46; No focal deficit in strength or sensitivity was observed at any time&#46; He became unable to walk&#44; as gait deteriorated with a significant apraxic component&#46; In the following days&#44; clinical symptoms deteriorated until the patient&#39;s condition progressed to akinetic mutism&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">A brain magnetic resonance imaging &#40;MRI&#41; scan performed 3 weeks after the hypoxic event revealed extensive involvement of the white matter in T2-weighted and FLAIR sequences&#44; and restriction in diffusion-weighted sequences &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A and B&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">General analysis including a coagulation study&#44; autoimmunity study&#44; serology test&#44; copper test&#44; lactate tests&#44; and arylsulphatase A test yielded no relevant findings&#46; The patient received general care and rehabilitation therapy and his condition improved gradually&#59; after 2 months and a half&#44; he had recovered to a baseline state and was discharged&#46; One year after admission&#44; the patient remains asymptomatic and can perform the activities of daily living independently&#46; Likewise&#44; MRI scans show a very favourable evolution &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>C and D&#41;&#46; Considering the clinical and radiological recovery&#44; and having excluded other possible inflammatory or infectious causes&#44; final diagnosis was reversible DPHL&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">This condition is typically characterised by a biphasic course with an immediate recovery after an episode of cerebral hypoxia-induced coma&#59; onset is followed by neuropsychiatric symptoms days or weeks after the episode&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">1</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The cause most frequently associated with this entity is carbon monoxide poisoning&#44;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">2</span></a> but it may also occur after other such anoxic events as strangulation&#44;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">3</span></a> haemorrhagic shock&#44;<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">4&#44;5</span></a> or opiate or sedative agent abuse&#44;<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">6&#44;7</span></a> as in our case&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">The precise pathophysiological mechanism of DPHL is still to be determined&#44; but given the similarity between the demyelinating findings on the MRI and those typical of metachromatic leukodystrophy&#44; it has been suggested that a deficiency of arylsulphatase A &#40;which is necessary for myelin turnover&#41; may predispose to this syndrome&#46;<a class="elsevierStyleCrossRefs" href="#bib0105"><span class="elsevierStyleSup">8&#44;9</span></a> However&#44; several similar cases with normal levels of this enzyme have also been published&#46;<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">10</span></a> Other mechanisms involved are the myelin toxicity of some external agents&#44;<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">11</span></a> alterations in the regulation of white matter vascularisation&#44;<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">12</span></a> or the specific susceptibility of white matter oligodendrocytes to cerebral hypoxia&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">13</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The characteristic clinical symptoms include cognitive&#47;behavioural impairment&#44; disorientation&#44; frontal signs&#44; amnesia&#44; parkinsonism&#44; akinetic mutism&#44; and psychosis&#46; MRI images typically show hyperintensity in T2- and diffusion-weighted sequences&#59; cerebrospinal fluid analysis yields normal results&#46; There is no specific treatment&#44; and prognosis may vary&#59; complete recovery may be achieved&#44; probably depending on the patient&#39;s age&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">1</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">In conclusion&#44; DPHL is an infrequent entity with characteristic clinical symptoms which should be known in order to avoid administering unnecessary treatments and diagnostic tests&#59; cranial MRI scans are useful for diagnosis and follow-up&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Funding</span><p id="par0055" class="elsevierStylePara elsevierViewall">This study received no funding of any kind&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflicts of interest</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Tainta M&#44; de la Riva P&#44; Urtasun M&#193;&#44; Mart&#237;-Mass&#243; JF&#46; Leucoencefalopat&#237;a posthip&#243;xica diferida reversible&#46; Neurolog&#237;a&#46; 2018&#59;33&#58;59&#8211;61&#46;</p>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Axial MRI images obtained 3 weeks after the hypoxic event&#44; with restricted diffusion in the diffusion-weighted sequence &#40;A&#41; and hyperintensity in the T2-weighted sequence &#40;B&#41; extensively affecting the whole periventricular white matter&#46; Below&#44; diffusion-weighted &#40;C&#41; and T2-weighted sequences &#40;D&#41; obtained one year later show the resolution of both alterations&#46;</p>"
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Article information
ISSN: 21735808
Original language: English
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es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos