Neuro sweet syndrome: a systematic review. A rare complication of Sweet syndrome

Francesco Drago, Giulia Ciccarese, Arianna Fay Agnoletti, Francesca Sarocchi, Aurora Parodi

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Sweet’s syndrome (SS) is an inflammatory disease characterized by fever, leucocytosis and distinctive skin lesions that histologically consist of a dermal infiltrate of neutrophils with nuclear fragmentation. Aseptic neutrophilic inflammation may occur also in other organs. Central nervous system involvement in SS, Neuro-Sweet’s syndrome (NSS), is rare and reported especially among Asian patients. A systematic review of the literature has been performed to find articles reporting cases of SS with neurological involvement. The search terms: “Sweet’s syndrome/disease with neurological involvement, Neuro Sweet Syndrome/Disease” were used in the Pubmed Database. Sixty-nine NSS patients including 46 males and 23 females, more Asian than Caucasian, have been described from 1983 to date. The average age was 48.7 year-old. The most representative neurologic symptom was the altered state of consciousness, followed by headache and memory disorders. Differently from SS with skin or other district involvement, NSS appears to be more common in Asian patients than in Caucasian ones and affects mainly the male sex in the third or fourth decade of life. A very wide range of symptoms and signs can occur, depending on which part of the nervous system is affected. Initial presentation is usually with the SS typical skin lesions followed by neurological involvement. However, also an opposite presentation or a simultaneous skin and nervous involvement may happen. Awareness of the possible neurological complications in SS is important to avoid unnecessary therapies for other forms of meningoencephalitis and lead to successful treatment with systemic corticosteroids.

Original languageEnglish
Pages (from-to)33-42
Number of pages10
JournalActa Neurologica Belgica
Volume117
Issue number1
DOIs
Publication statusPublished - Mar 1 2017

Fingerprint

Sweet Syndrome
Skin
Headache Disorders
Meningoencephalitis
Leukocytosis
Memory Disorders
Neurologic Manifestations
Consciousness
PubMed
Nervous System
Signs and Symptoms
Adrenal Cortex Hormones
Neutrophils
Fever
Central Nervous System

Keywords

  • Encephalitis
  • Meningitis
  • Neuro-Bechet disease
  • Neuro-sweet syndrome
  • Sweet syndrome

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Neuro sweet syndrome : a systematic review. A rare complication of Sweet syndrome. / Drago, Francesco; Ciccarese, Giulia; Agnoletti, Arianna Fay; Sarocchi, Francesca; Parodi, Aurora.

In: Acta Neurologica Belgica, Vol. 117, No. 1, 01.03.2017, p. 33-42.

Research output: Contribution to journalArticle

Drago, Francesco ; Ciccarese, Giulia ; Agnoletti, Arianna Fay ; Sarocchi, Francesca ; Parodi, Aurora. / Neuro sweet syndrome : a systematic review. A rare complication of Sweet syndrome. In: Acta Neurologica Belgica. 2017 ; Vol. 117, No. 1. pp. 33-42.
@article{9301fc9eaa134354ac9678c18df70eba,
title = "Neuro sweet syndrome: a systematic review. A rare complication of Sweet syndrome",
abstract = "Sweet’s syndrome (SS) is an inflammatory disease characterized by fever, leucocytosis and distinctive skin lesions that histologically consist of a dermal infiltrate of neutrophils with nuclear fragmentation. Aseptic neutrophilic inflammation may occur also in other organs. Central nervous system involvement in SS, Neuro-Sweet’s syndrome (NSS), is rare and reported especially among Asian patients. A systematic review of the literature has been performed to find articles reporting cases of SS with neurological involvement. The search terms: “Sweet’s syndrome/disease with neurological involvement, Neuro Sweet Syndrome/Disease” were used in the Pubmed Database. Sixty-nine NSS patients including 46 males and 23 females, more Asian than Caucasian, have been described from 1983 to date. The average age was 48.7 year-old. The most representative neurologic symptom was the altered state of consciousness, followed by headache and memory disorders. Differently from SS with skin or other district involvement, NSS appears to be more common in Asian patients than in Caucasian ones and affects mainly the male sex in the third or fourth decade of life. A very wide range of symptoms and signs can occur, depending on which part of the nervous system is affected. Initial presentation is usually with the SS typical skin lesions followed by neurological involvement. However, also an opposite presentation or a simultaneous skin and nervous involvement may happen. Awareness of the possible neurological complications in SS is important to avoid unnecessary therapies for other forms of meningoencephalitis and lead to successful treatment with systemic corticosteroids.",
keywords = "Encephalitis, Meningitis, Neuro-Bechet disease, Neuro-sweet syndrome, Sweet syndrome",
author = "Francesco Drago and Giulia Ciccarese and Agnoletti, {Arianna Fay} and Francesca Sarocchi and Aurora Parodi",
year = "2017",
month = "3",
day = "1",
doi = "10.1007/s13760-016-0695-1",
language = "English",
volume = "117",
pages = "33--42",
journal = "Acta Neurologica Belgica",
issn = "0300-9108",
publisher = "Springer-Verlag Italia",
number = "1",

}

TY - JOUR

T1 - Neuro sweet syndrome

T2 - a systematic review. A rare complication of Sweet syndrome

AU - Drago, Francesco

AU - Ciccarese, Giulia

AU - Agnoletti, Arianna Fay

AU - Sarocchi, Francesca

AU - Parodi, Aurora

PY - 2017/3/1

Y1 - 2017/3/1

N2 - Sweet’s syndrome (SS) is an inflammatory disease characterized by fever, leucocytosis and distinctive skin lesions that histologically consist of a dermal infiltrate of neutrophils with nuclear fragmentation. Aseptic neutrophilic inflammation may occur also in other organs. Central nervous system involvement in SS, Neuro-Sweet’s syndrome (NSS), is rare and reported especially among Asian patients. A systematic review of the literature has been performed to find articles reporting cases of SS with neurological involvement. The search terms: “Sweet’s syndrome/disease with neurological involvement, Neuro Sweet Syndrome/Disease” were used in the Pubmed Database. Sixty-nine NSS patients including 46 males and 23 females, more Asian than Caucasian, have been described from 1983 to date. The average age was 48.7 year-old. The most representative neurologic symptom was the altered state of consciousness, followed by headache and memory disorders. Differently from SS with skin or other district involvement, NSS appears to be more common in Asian patients than in Caucasian ones and affects mainly the male sex in the third or fourth decade of life. A very wide range of symptoms and signs can occur, depending on which part of the nervous system is affected. Initial presentation is usually with the SS typical skin lesions followed by neurological involvement. However, also an opposite presentation or a simultaneous skin and nervous involvement may happen. Awareness of the possible neurological complications in SS is important to avoid unnecessary therapies for other forms of meningoencephalitis and lead to successful treatment with systemic corticosteroids.

AB - Sweet’s syndrome (SS) is an inflammatory disease characterized by fever, leucocytosis and distinctive skin lesions that histologically consist of a dermal infiltrate of neutrophils with nuclear fragmentation. Aseptic neutrophilic inflammation may occur also in other organs. Central nervous system involvement in SS, Neuro-Sweet’s syndrome (NSS), is rare and reported especially among Asian patients. A systematic review of the literature has been performed to find articles reporting cases of SS with neurological involvement. The search terms: “Sweet’s syndrome/disease with neurological involvement, Neuro Sweet Syndrome/Disease” were used in the Pubmed Database. Sixty-nine NSS patients including 46 males and 23 females, more Asian than Caucasian, have been described from 1983 to date. The average age was 48.7 year-old. The most representative neurologic symptom was the altered state of consciousness, followed by headache and memory disorders. Differently from SS with skin or other district involvement, NSS appears to be more common in Asian patients than in Caucasian ones and affects mainly the male sex in the third or fourth decade of life. A very wide range of symptoms and signs can occur, depending on which part of the nervous system is affected. Initial presentation is usually with the SS typical skin lesions followed by neurological involvement. However, also an opposite presentation or a simultaneous skin and nervous involvement may happen. Awareness of the possible neurological complications in SS is important to avoid unnecessary therapies for other forms of meningoencephalitis and lead to successful treatment with systemic corticosteroids.

KW - Encephalitis

KW - Meningitis

KW - Neuro-Bechet disease

KW - Neuro-sweet syndrome

KW - Sweet syndrome

UR - http://www.scopus.com/inward/record.url?scp=84988693273&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84988693273&partnerID=8YFLogxK

U2 - 10.1007/s13760-016-0695-1

DO - 10.1007/s13760-016-0695-1

M3 - Article

C2 - 27659797

AN - SCOPUS:84988693273

VL - 117

SP - 33

EP - 42

JO - Acta Neurologica Belgica

JF - Acta Neurologica Belgica

SN - 0300-9108

IS - 1

ER -