PMID- 30175267
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220331
IS  - 2451-9936 (Electronic)
IS  - 2451-9936 (Linking)
VI  - 12
DP  - 2018 Dec
TI  - Vasculitis with superior ophthalmic vein thrombosis compatible with 
      neuro-neutrophilic disease.
PG  - 39-44
LID - 10.1016/j.ajoc.2018.08.005 [doi]
AB  - PURPOSE: To present a unique case of neuro-neutrophilic disease with inflammation 
      and thrombosis of the superior ophthalmic vein (SOV). OBSERVATIONS: A 43-year-old 
      Japanese man with past histories of oculomotor paralysis, auditory disorder, 
      ischemic enteritis, and recurrent oral ulceration was referred to our hospital 
      because of blurred vision in his right eye. Ophthalmic examination revealed 
      decreased best corrected visual acuity and central scotoma in his right eye. 
      Orbit magnetic resonance imaging (MRI) revealed an enlarged SOV in the right eye, 
      with Gadolinium (Gd) enhancement in the wall of the vein but not inside the vein, 
      indicating thrombosis. Multiple Gd-enhanced hyperintense lesions were also 
      observed in the juxtacortical area of the brain. We diagnosed the patient with 
      vasculitis in the right SOV that was adversely affecting the optic nerve. We 
      ruled out systemic thrombophilia, infections, and malignancy by systemic 
      examinations. The human leukocyte antigen (HLA) typing was Cw1-, B54-, B61-, A2-, 
      A24-, and DR4-positive and B51-negative. We treated the patient with systemic 
      steroid and anticoagulant therapy. After three courses of steroid pulse therapy, 
      his symptoms and the MRI findings of the right SOV and brain improved; therefore, 
      we decided to discontinue the anticoagulant therapy. One month after 
      anticoagulant cessation, MRI revealed recurrence of the thrombus and enlargement 
      of the right SOV despite the lack of vision worsening. We restarted the 
      anticoagulant therapy while continuing the oral prednisolone treatment. At the 
      final visit, 14 months after the onset of the disease, the patient was still 
      receiving oral anticoagulation with warfarin potassium and prednisolone (5 
      mg/day). His symptoms and the right eye's visual function remained normal with a 
      mildly enlarged SOV; there was less Gd enhancement and no brain lesions on MRI. 
      CONCLUSIONS AND IMPORTANCE: We treated a unique case of possible 
      neuro-neutrophilic disease that presented visual disturbances due to right SOV 
      inflammation and thrombosis. Anticoagulation and systemic steroid therapies were 
      required to reduce the inflammation and to prevent the recurrence of thrombosis.
FAU - Sakamoto, Mari
AU  - Sakamoto M
AD  - Division of Ophthalmology, Department of Surgery, Kobe University Graduate, 
      School of Medicine, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan.
FAU - Kurimoto, Takuji
AU  - Kurimoto T
AD  - Division of Ophthalmology, Department of Surgery, Kobe University Graduate, 
      School of Medicine, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan.
FAU - Mori, Sotaro
AU  - Mori S
AD  - Division of Ophthalmology, Department of Surgery, Kobe University Graduate, 
      School of Medicine, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan.
FAU - Ueda, Kaori
AU  - Ueda K
AD  - Division of Ophthalmology, Department of Surgery, Kobe University Graduate, 
      School of Medicine, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan.
FAU - Keshi, Yukako
AU  - Keshi Y
AD  - Division of Ophthalmology, Department of Surgery, Kobe University Graduate, 
      School of Medicine, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan.
FAU - Yamada, Yuko
AU  - Yamada Y
AD  - Division of Ophthalmology, Department of Surgery, Kobe University Graduate, 
      School of Medicine, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan.
FAU - Azumi, Atsushi
AU  - Azumi A
AD  - Kobe Kaisei Hospital, 3-11-15 Shiohara Kitamachi, Nada-ku, Kobe, 657-0068, Japan.
FAU - Shimono, Taro
AU  - Shimono T
AD  - Department of Diagnostic and Interventional Radiology, Osaka City University 
      Graduate School of Medicine, 1-4-3 Asahi-machi, Abeno-ku, Osaka, 545-8585, Japan.
FAU - Nakamura, Makoto
AU  - Nakamura M
AD  - Division of Ophthalmology, Department of Surgery, Kobe University Graduate, 
      School of Medicine, 7-5-2 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan.
LA  - eng
PT  - Case Reports
DEP - 20180825
PL  - United States
TA  - Am J Ophthalmol Case Rep
JT  - American journal of ophthalmology case reports
JID - 101679941
PMC - PMC6115536
OTO - NOTNLM
OT  - Human leukocyte antigen
OT  - Neuro-Behcet disease
OT  - Neuro-Sweet disease
OT  - Neuro-neutrophilic disease
OT  - Thrombosis
EDAT- 2018/09/04 06:00
MHDA- 2018/09/04 06:01
PMCR- 2018/08/25
CRDT- 2018/09/04 06:00
PHST- 2018/04/06 00:00 [received]
PHST- 2018/08/02 00:00 [revised]
PHST- 2018/08/22 00:00 [accepted]
PHST- 2018/09/04 06:00 [entrez]
PHST- 2018/09/04 06:00 [pubmed]
PHST- 2018/09/04 06:01 [medline]
PHST- 2018/08/25 00:00 [pmc-release]
AID - S2451-9936(18)30135-X [pii]
AID - 10.1016/j.ajoc.2018.08.005 [doi]
PST - epublish
SO  - Am J Ophthalmol Case Rep. 2018 Aug 25;12:39-44. doi: 10.1016/j.ajoc.2018.08.005. 
      eCollection 2018 Dec.