LETTER TO EDITOR Year : 2023  |  Volume : 71  |  Issue : 5  |  Page : 1068-1069

Respıratuar Changes ın Creutzfeldt-Jakob Dısease: Bıot Lıke Respıratuar Pattern

Ince Aysenur, Kozak H Huseyın
Neurology Department, Meram Medıcıne Faculty Hospıtal, Necmettın Erbakan Unıversıty, Konya, Turkey

Date of Submission20-Feb-2023Date of Decision13-Apr-2023Date of Acceptance14-Apr-2023Date of Web Publication18-Oct-2023

Correspondence Address:
Ince Aysenur
School of Medical Education Konya, Konya
Turkey

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/0028-3886.388043

How to cite this article:
Aysenur I, Huseyın KH. Respıratuar Changes ın Creutzfeldt-Jakob Dısease: Bıot Lıke Respıratuar Pattern. Neurol India 2023;71:1068-9

Sir,

Creuztfeld-Jakob disease (CJD) is a rare progressive neurodegenerative disease. Pathological prion protein accumulation plays a role in its pathophysiology.[1] Pathological protein deposition, spongiform degeneration, and neuronal loss are most common in the quadrigeminal body, substantia nigra, pontine nucleus, inferior olivary nucleus, and posterior horn. Involvement of the spinal cord and brain stem is very rare.[2] In our case, a CJD patient with brain stem involvement is discussed.

A 59-year-old male patient with complaints of increasing sleepiness and disorientation, which lasted for approximately 40 days, presented with complaints of increased sleepiness, instability in walking, and inadequate response to verbal stimuli. No pathological reflex was detected. The patient's general physical examination was normal and it was decided to investigate the etiology of the change of consciousness. Routine blood and cerebrospinal fluid tests were found to be within normal limits. Diffusion magnetic resonance imaging had a cortical ribbon image with increased diffusion in the frontal, parietal, and occipital regions but cranial magnetic resonance imaging was normal. Widespread low-amplitude delta and sparse theta waves were seen in his EEG. 14.3.3 protein sent from cerebrospinal fluid was reported as positive. The patient was diagnosed with probable CJD according to the 2018 CDC CJD diagnostic criteria.[3] The patient, whose neurological status deteriorated during the examinations and whose brain stem reflexes could not be obtained, was taken to intensive care follow-up. A significant change in respiratory amplitude was observed in the follow-up of the intubated patient. A directly proportional relationship was observed between the patient's respiratory depth and pulse. It was observed that there was a sudden increase in respiratory amplitude and a decrease in respiratory rate after bradycardia (45-55/min), which lasted approximately 30 seconds to 1 minute, and then the patient's pulse first came to the normal range, then tachycardic (100-112/min). This cycle continued in the same way as the respiratory amplitude decreased again. This breathing pattern was repeated in this way in cycles of 3 to 4 minutes for 4 days. With these respiratory-pulse pattern findings, the patient died 6 days after intubation due to respiratory arrest.

In the literature, respiratory arrest associated with respiratory pattern change and the need for mechanical ventilators in CJD cases have been emphasized. In our case, changes in cardiac rhythm were observed with a biot-like respiratory pattern, which was not defined in the literature in CJD cases. Although pathological protein accumulation is mentioned in the foreground in the pathophysiological studies, recent animal studies show that microglia activation and inflammatory cytokines play a role in the pathophysiology of the disease in CJD cases. It was thought that the findings and clinical changes in the patient we followed may be related to this secondary response.[4] In conclusion, clinical and laboratory follow-up of CJD cases can provide important information about the clinical course and prognosis of the disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 

  References 
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    2.Gascon Bayarri J, Campdelacreu J, Monesterio C. Central apnea in a sporadic case of Creutzfeld-Jakob disease with brainstem involvement. Acta Neurol Belg 2010;110:113-5.  
    3.Watson N, Brandel JP. The importance of ongoing international surveillance for Creutzfeldt–Jakob disease. Nature 2021;17:362-79.  
    4.Obst J, Simon E, Mancuso R, Gomez-Nicola D. The role of microglia in prion diseases: A paradigm of functional diversity. Front Aging Neurosci 2017;9:207. doı: 10.3389/fnagi.2017.00207.