Posterior reversible encephalopathy syndrome presenting with thrombotic microangiopathy triggered by malignant hypertension: a case report and literature review.

cerebral blood flow haematology headache hypertensive encepha thrombophilia

Journal

BMJ neurology open
ISSN: 2632-6140
Titre abrégé: BMJ Neurol Open
Pays: England
ID NLM: 101775450

Informations de publication

Date de publication:
2022
Historique:
accepted: 07 08 2022
entrez: 14 9 2022
pubmed: 15 9 2022
medline: 15 9 2022
Statut: epublish

Résumé

Posterior reversible encephalopathy syndrome (PRES) is a disease characterised by reversible subcortical vasogenic oedema, neurological symptoms and abnormal findings on head imaging. It is recognised as one of the most prominent organ disorders in hypertensive emergencies but is rarely associated with thrombotic microangiopathy (TMA). A woman in her 40s with untreated hypertension had occasional headaches in the past 4 months. The headaches worsened during the 3 weeks prior to admission. On the day of admission, the patient presented with severe headache accompanied by frequent vomiting. MRI of the head revealed oedematous changes in the brainstem, including the subcortical, cerebellum and pons. Fundus examination revealed hypertensive retinopathy with papilloedema. Blood tests indicated thrombocytopenia, renal dysfunction and haemolytic anaemia, and a blood smear confirmed fragmented erythrocytes. Coombs' test, and tests for ADAMTS13 activity and infectious and autoimmune diseases were negative. The patient was diagnosed with PRES, secondary to malignant hypertension (MH) and associated with TMA. Antihypertensive therapy promptly improved the clinical symptoms, blood pressure, and the abnormal MRI and blood test findings. The patient was discharged from the hospital 20 days after admission. We report a rare case of PRES that was associated with TMA and triggered by MH. Antihypertensive therapy was effective in alleviating the associated adverse clinical symptoms. Differentiation of underlying diseases is essential for early intervention, since treatment depends on factors causing TMA.

Sections du résumé

Background UNASSIGNED
Posterior reversible encephalopathy syndrome (PRES) is a disease characterised by reversible subcortical vasogenic oedema, neurological symptoms and abnormal findings on head imaging. It is recognised as one of the most prominent organ disorders in hypertensive emergencies but is rarely associated with thrombotic microangiopathy (TMA).
Case presentation UNASSIGNED
A woman in her 40s with untreated hypertension had occasional headaches in the past 4 months. The headaches worsened during the 3 weeks prior to admission. On the day of admission, the patient presented with severe headache accompanied by frequent vomiting. MRI of the head revealed oedematous changes in the brainstem, including the subcortical, cerebellum and pons. Fundus examination revealed hypertensive retinopathy with papilloedema. Blood tests indicated thrombocytopenia, renal dysfunction and haemolytic anaemia, and a blood smear confirmed fragmented erythrocytes. Coombs' test, and tests for ADAMTS13 activity and infectious and autoimmune diseases were negative. The patient was diagnosed with PRES, secondary to malignant hypertension (MH) and associated with TMA. Antihypertensive therapy promptly improved the clinical symptoms, blood pressure, and the abnormal MRI and blood test findings. The patient was discharged from the hospital 20 days after admission.
Conclusions UNASSIGNED
We report a rare case of PRES that was associated with TMA and triggered by MH. Antihypertensive therapy was effective in alleviating the associated adverse clinical symptoms. Differentiation of underlying diseases is essential for early intervention, since treatment depends on factors causing TMA.

Identifiants

pubmed: 36101544
doi: 10.1136/bmjno-2022-000296
pii: bmjno-2022-000296
pmc: PMC9413191
doi:

Types de publication

Journal Article

Langues

eng

Pagination

e000296

Informations de copyright

© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

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Auteurs

Hitomi Onomura (H)

Department of Neurology, Toyota Memorial Hospital, Toyota, Aichi, Japan.

Takahiro Shimizu (T)

Department of Neurology, Toyota Memorial Hospital, Toyota, Aichi, Japan.

Junichiro Suzuki (J)

Department of Neurology, Toyota Memorial Hospital, Toyota, Aichi, Japan.

Noriyoshi Nakai (N)

Department of Neurology, Toyota Memorial Hospital, Toyota, Aichi, Japan.

Yuri Teramachi (Y)

Department of Nephrology, Toyota Memorial Hospital, Toyota, Aichi, Japan.

Kato Tomonori (K)

Department of Hematology, Toyota Memorial Hospital, Toyota, Aichi, Japan.

Ichiro Akiguchi (I)

Center of Neurological and Cerebrovascular Diseases, Takeda Hospital, Kyoto, Japan.

Yasuhiro Ito (Y)

Department of Neurology, Toyota Memorial Hospital, Toyota, Aichi, Japan.

Classifications MeSH