Vascular collateralization along ventriculoperitoneal shunt catheters in moyamoya disease

2013 ◽  
Vol 11 (6) ◽  
pp. 710-712 ◽  
Author(s):  
Amit Singla ◽  
Ning Lin ◽  
Allen L. Ho ◽  
R. Michael Scott ◽  
Edward R. Smith
Keyword(s):  
Blood Supply ◽  
Moyamoya Disease ◽  
Shunt Malfunction ◽  
Shunt Revision ◽  
Ventricular Catheter ◽  
Increased Risk ◽  
Diagnostic Angiography ◽  
Subsequent Revision ◽  
Collateral Vessels ◽  
Clinical Records

Surgically created openings such as bur holes can serve as avenues for the development of collateral blood supply to the brain in patients with moyamoya disease. When such collateralization occurs through preexisting shunt catheter sites, the potential exists for perioperative stroke if these vessels are damaged during revision of a ventricular catheter for shunt malfunction. In this paper the authors report on a series of patients with a history of ventriculoperitoneal (VP) shunts who later developed moyamoya disease and were found to have spontaneous transdural collateral vessels at ventricular catheter sites readily visualized on diagnostic angiography. A consecutive surgical series of 412 patients with moyamoya disease treated at Boston Children's Hospital from 1990 to 2010 were reviewed to identify patients with concomitant moyamoya and a VP shunt. The clinical records and angiograms of these patients were reviewed to determine the extent of bur hole collaterals through the shunt site. Three patients were identified who had VP shunts placed for hydrocephalus and subsequently developed moyamoya disease. All 3 patients demonstrated spontaneous transdural collaterals at the ventricular catheter bur hole, as confirmed by angiography during the workup for moyamoya disease. No patients required subsequent revision of their ventricular catheters following the diagnosis of moyamoya. All patients have remained stroke free and clinically stable following pial synangiosis. Although the association of moyamoya and shunted hydrocephalus is rare, it may present a significant potential problem for the neurosurgeon treating a shunt malfunction in this patient population, because shunt bur holes may become entry sites for the ingrowth of significant cortical transdural collateral blood supply to the underlying brain. Shunt revision might therefore be associated with an increased risk of postoperative stroke or operative-site hemorrhage in this population if this vascularization is interrupted when shunt catheters are removed and replaced. A knowledge of the existence of shunt-related collaterals in patients with moyamoya may aid the surgeon in planning shunt revisions and considering, for example, a new entry point for a ventricular catheter, rather than replacing an existing one, to minimize the risk of jeopardizing existing collaterals.

Radiographic assessment of snap-shunt failure

2010 ◽  
Vol 6 (3) ◽  
pp. 299-302 ◽  
Author(s):  
Anand I. Rughani ◽  
Bruce I. Tranmer ◽  
Jeffrey E. Florman ◽  
James T. Wilson
Keyword(s):  
Normal Values ◽  
Clinical Symptoms ◽  
Type System ◽  
Shunt Malfunction ◽  
Ct Scans ◽  
Shunt Revision ◽  
Ventricular Catheter ◽  
Shunt Failure ◽  
Shunt System ◽  
Ct Finding

Accurate assessment of imaging studies in patients with ventriculoperitoneal shunts can be aided by empirical findings. The authors characterize an objective measurement easily performed on head CT scans with the goal of producing clear evidence of shunt fracture or disconnection in patients with a snap shunt–type system. The authors describe 2 cases of ventriculoperitoneal shunt failure involving a fracture and a disconnection associated with a snap-shunt assembly. In both cases the initial clinical symptoms were not convincing for shunt malfunction, and the interpretation of the CT finding failed to immediately identify the abnormality. As the clinical picture became more convincing for shunt malfunction, each patient subsequently underwent successful shunt revision. The authors reviewed the CT scans of 10 patients with an intact and functioning snap-shunt system to characterize the normal appearance of the snap-shunt connection. On CT scans the distance between the radiopaque portion of the ventricular catheter and the radiopaque portion of the reservoir dome measures an average of 4.72 mm (range 4.6–4.9 mm, 95% CI 4.63–4.81 mm). In the authors' patient with a fractured ventricular catheter, this interval measured 7.8 mm, and in the patient with a disconnection it measured 7.7 mm. In comparison with the range of normal values, a radiolucent interval significantly greater than 4.9 mm should promptly raise concern for a disconnected or fractured shunt in this system. This measurement may prove particularly useful when serial imaging is not readily available.

scholarly journals Shunt malfunction and endoscopy

2021 ◽  
Vol 3 (1(January-April)) ◽  
pp. e792021
Author(s):  
Bermans Iskandar ◽  
Ricardo de Amoreira Gepp
Keyword(s):  
Obstructive Hydrocephalus ◽  
Pediatric Neurosurgery ◽  
Shunt Malfunction ◽  
Surrounding Tissue ◽  
Shunt Revision ◽  
Third Ventriculostomy ◽  
Ventricular Catheter ◽  
Ventricular Wall ◽  
Optimal Position ◽  
Slit Ventricle Syndrome

Objective:   Hydrocephalus is the most common neurological disease in pediatric neurosurgery.(1) The CSF shunts remains as the most common treatment choice for nonobstructive hydrocephalus worldwide, but shunt complications still the most common neurosurgical problem, especially in pediatric neurosurgery. Endoscopy and shunts are the way to treat hydrocephalus. Especially third ventriculostomy is the most effective treatment to obstructive hydrocephalus but shunt still the most important way to treat.(2, 3) Shunt malfunction is frequent and after so many years this is very important problem to the patients. Ventricular problem due to obstruction is responsible up to 72% of shunt problems.(4) The Shunt Trial Study showed that the overall shunt survival was 62% at 1 year, 52% at 2 years, 46% at 3 years, 41% at 4 years. The survival curves for the 3 differents valves were similar to those from the original trial and did not show a survival advantage for any particular valve.(5, 6) We still don´t have one perfect solution to hydrocephalus and shunt malfunction. The major author described his experience in use endoscopy to evaluate and treat shunt malfunction and one new approach and way to evaluate this problem.   Results/Discussion: The literature review was performed, and we found 84 articles when we used the keywords. Endoscopy has been one important way to treat and solve shunt problems. In obstructive hydrocephalus third ventriculostomy is the best way to treat these patients.(1-3) The major author first described goals of endoscopy. First goal is safe catheter removal in surgical review, avoiding bleeding when removing catheter addressing all the adhesions on catheter. Second goal is put in optimal position the new catheter with pure endoscopy view or using neuronavigation systems that could help the endoscope system.(7, 8)   Optimal new catheter placement and optimal long-term catheter survival are especially important because most of the problems are due to ventricular problems. These good placements could avoid loculations and ventricular collapse with ependymal problems. Avoid new catheter malpositiitioning, you can use the endoscope to follow the old tract to insert the new catheter in one good position avoiding choroid plexus. Another situation is when you have small ventricles especially in slit ventricle syndrome.   The major author has been studied some causes to ventricular catheter obstruction. He noticed after some surgical reviews some ventricular ependymal inside catheter. Ventricular ependymal protrusions inside the catheter could cause intermittent occlusion.(8) Some endoscope views showed these protrusion and ependymal changes after intermittent increase and decrease of ventricular pressure. These protrusions correspond to catheter holes a secondary to suction. These protrusions could stuck in the holes in chronicle suction.(8) The major author reported one endoscopic evidence of overdrainage-related ventricular tissue protrusions that cause partial or complete obstruction of the ventricular catheter. He did a retrospective review in fifty patients underwent 83 endoscopic shunt revision procedures that revealed in-growth of ventricular wall tissue into the catheter tip orifices (ependymal bands), producing partial, complete, or intermittent shunt obstructions. Endoscopic ventricular explorations revealed ependymal bands at various stages of development, which appear to form secondarily to siphoning.(8) How to minimize this overshunting? Anti siphon systems could help and decrease proximal shunt malfunction in some complex patients. The other problem is ventricular bleeding. The use of endoscope has been important tool to remove ventricular catheters, when you could see the adhesions.(9) The use the endoscope could be particularly important to open loculations and cysts avoiding ventricular entrapment. Patients with ventricular cysts could need more than one catheter. The use of endoscopy to fenestrate the cyst could keep the patient with one catheter or without any shunt system.(10, 11)   Conclusion: Shunt malfunction has a lot of possible causes, but a probably ventricular catheter problem is the most common situation. Choose appropriate endoscope rigid or flexible for each case could help to treat and avoid some of ventricular. Endoscopy could be one important tool to help the surgeon to understand and solve this dangerous situation to the patient. Ventricular wall protrusions are a significant cause of proximal shunt obstruction, and they appear to be caused by siphoning of surrounding tissue into the ventricular catheter orifices.

Increased risk of distal ventriculoperitoneal shunt obstruction associated with slit valves or distal slits in the peritoneal catheter

1997 ◽  
Vol 87 (5) ◽  
pp. 682-686 ◽  
Author(s):  
Jeffrey W. Cozzens ◽  
James P. Chandler
Keyword(s):  
Shunt Revision ◽  
Ventricular Catheter ◽  
Peritoneal Catheter ◽  
Distal Catheter ◽  
Content Type ◽  
Vp Shunt ◽  
Increased Risk ◽  
Catheter Obstruction ◽  
Shunt Obstruction ◽  
Fine Print

✓ The authors describe a relationship between the presence of distal shunt catheter side-wall slits and distal catheter obstruction in a single-surgeon series of ventriculoperitoneal (VP) shunt revisions. Between 1985 and 1996, 168 operations for VP shunt revision were performed by the senior author (J.W.C.) in 71 patients. Indications for shunt revision included obstruction in 140 operations; overdrainage or underdrainage requiring a change of valve in 17 operations; inadequate length of distal shunt tubing resulting in the distal end no longer reaching the peritoneum in five operations; the ventricular catheter in the wrong ventricle or space, requiring repositioning in five operations; and a disconnected or broken shunt in one operation. Of the 140 instances of shunt obstruction, the blockage occurred at the ventricular end in 108 instances (77.1%), the peritoneal end in 17 (12.1%), the ventricular and the peritoneal end in 14 (10%), and in the valve mechanism (not including distal slit valves) in one (0.8%). Thus, the peritoneal end was obstructed in 31 (22.1%) of 140 cases of shunt malfunction. In every case in which the peritoneal end was obstructed, some form of distal slit was found: either a distal slit valve in an otherwise closed catheter or slits in the side of an open catheter. No instances were found of distal peritoneal catheter obstruction when the peritoneal catheter was a simple open-ended tube with no accompanying side slits (0 of 55). It is concluded that side slits in the distal peritoneal catheters of VP shunts are associated with a greater incidence of distal shunt obstruction.

scholarly journals Suprahepatic space as an alternative site for distal catheter insertion in pseudocyst-associated ventriculoperitoneal shunt malfunction

2020 ◽  
Vol 26 (3) ◽  
pp. 247-254
Author(s):  
Zohreh Habibi ◽  
Mehdi Golpayegani ◽  
Bahar Ashjaei ◽  
Keyvan Tayebi Meybodi ◽  
Farideh Nejat
Keyword(s):  
Catheter Insertion ◽  
Shunt Malfunction ◽  
Shunt Revision ◽  
Ventricular Catheter ◽  
Distal Catheter ◽  
Safe Place ◽  
Catheter Malfunction ◽  
Shunt Removal ◽  
Pseudocyst Formation

OBJECTIVEDistal catheter malfunction due to pseudocyst formation or intraabdominal adhesion is a significant problem, especially in pediatric patients who have limited sites for distal catheter insertion. In this study, the authors present a series of 12 patients with intractable distal shunt malfunctions due to peritoneal pseudocyst formation who underwent distal catheter replacement in the suprahepatic space to reduce the risk of distal catheter malfunction.METHODSTwelve consecutive patients with shunt malfunction due to pseudocyst formation who had undergone ventriculosuprahepatic shunting from 2014 to 2019 were identified. According to medical records, after primary evaluations, shunt removal, and antibiotic therapy, they underwent revision surgeries with placement of a distal catheter into the suprahepatic space.RESULTSNine boys and 3 girls, ranging in age from 5 months to 14 years, with one or more episodes of pseudocyst formation, underwent shunt revision with placement of a distal catheter into the suprahepatic space. After a median follow-up of 31 months, none of the patients experienced further distal malfunction.CONCLUSIONSThe suprahepatic space appears to be a safe place to secure the distal end of a ventricular catheter following pseudocyst formation, with less risk of re-adhesion. This lower risk might be attributable to the lack of omentum in the suprahepatic space.

scholarly journals Intradural spine surgery may not carry an increased risk of shunt revision compared with extradural spine surgery in pediatric patients with myelomeningocele

2018 ◽  
Vol 21 (1) ◽  
pp. 11-15 ◽  
Author(s):  
Elizabeth N. Kuhn ◽  
Betsy Hopson ◽  
Michael J. Conklin ◽  
Jeffrey P. Blount
Keyword(s):  
Spinal Fusion ◽  
Spine Surgery ◽  
Pediatric Patients ◽  
Tethered Cord ◽  
Shunt Malfunction ◽  
Shunt Revision ◽  
Rank Test ◽  
Kaplan Meier ◽  
Increased Risk ◽  
Significant Difference

OBJECTIVEPatients with myelomeningocele are often affected by scoliosis and tethered cord syndrome, and frequently require spine surgery. Intradural spine surgeries may carry an inherently higher risk of inducing shunt malfunction due to entry into the subarachnoid space. In this study, the authors sought to compare rates of shunt malfunction after intradural and extradural spine surgeries among pediatric patients with myelomeningocele.METHODSThe authors reviewed records of the National Spina Bifida Program Registry for Children’s Hospital of Alabama. The Exago reporting function was used to identify patients who had received at least one of the following procedures: shunt revision, tethered cord release (TCR), or spinal fusion for deformity. The registry records were reviewed for all identified patients to determine if a shunt revision was performed within the 1st year after TCR or spinal fusion.RESULTSFinal analyses included 117 patients, of whom 39 underwent spinal fusion and 78 underwent TCR. Among patients who underwent spinal fusion, shunt revision was performed within 30 days in 2 patients (5.1%), within 60 days in 2 (5.1%), within 90 days in 4 (10.3%), and within 1 year in 5 (12.8%). Among patients who underwent TCR, shunt revision was performed within 30 days in 7 patients (9.0%), within 60 days in 10 (12.8%), within 90 days in 11 (14.1%), and within 1 year in 17 (21.8%). Using the log-rank test, there was no significant difference in Kaplan-Meier curves between intradural and extradural groups (p = 0.59).CONCLUSIONSIn a review of single-institution registry data, the authors found no statistically significant difference in the risk of shunt malfunction after intradural and extradural spine surgeries.

Reliability of the radiopharmaceutical shunt flow study for the detection of a CSF shunt malfunction in the presence of stable ventricular size

2020 ◽  
Vol 26 (4) ◽  
pp. 364-370
Author(s):  
Jeffrey J. Quezada ◽  
J. Gordon McComb
Keyword(s):  
Ct Scan ◽  
Operative Intervention ◽  
Shunt Malfunction ◽  
Shunt Revision ◽  
Csf Shunt ◽  
Technical Failure ◽  
Ventricular Size ◽  
Shunt Flow ◽  
Programmable Valve ◽  
Flow Study

OBJECTIVEThe authors sought to determine the reliability of a radiopharmaceutical (RP) shunt flow study for the detection of a CSF-diverting shunt malfunction in the presence of stable ventricular size.METHODSAfter the authors obtained IRB approval, all CSF RP shunt flow studies done between January 1, 2014, and January 1, 2019, in pediatric patients at Children’s Hospital Los Angeles were identified. Included in the study were only those patients in whom an MRI or CT scan was done during the hospital admission for shunt malfunction and showed no increase in ventricular size compared with the most recent prior MRI or CT scan when the patient was asymptomatic. Data recorded for analysis were patient age and sex, etiology of the hydrocephalus, shunt distal site, nonprogrammable versus programmable valve, operative findings if the shunt was revised, and follow-up findings for a minimum of 90 days after admission. The RP shunt flow study consisted of tapping the reservoir and injecting technetium-99m DTPA according to a set protocol.RESULTSThe authors identified 146 RP flow studies performed in 119 patients meeting the above criteria. Four of the 146 RP studies (3%) were nondiagnostic secondary to technical failure and were excluded from statistical analysis. Of the 112 normal flow studies, operative intervention was not undertaken in 102 (91%). The 10 (9%) remaining normal studies were performed in patients who underwent operative intervention, in which 8 patients had a proximal obstruction, 1 had a distal obstruction, and 1 patient had no obstruction. Of the 30 patients with abnormal flow studies, symptoms of shunt malfunction subsided in 9 (30%) patients and these patients did not undergo operative intervention. Of the 21 (70%) operated patients, obstruction was proximal in 9 patients and distal in 5, and for 7 patients the shunt tubing was either fractured or disconnected. Regression analysis indicated a significant association between the flow study interpretation and the odds for shunt revision (OR 27, 95% CI 10–75, p < 0.0001). No other clinical variables were significant. The sensitivity of a shunt flow study alone for detection of shunt malfunction in cases with stable ventricular size was the same as a shunt flow study plus an MRI or CT (70% vs 70%), but performing a shunt flow in addition to MRI or CT did increase the specificity from 92% to 100% and the accuracy from 87% to 94%.CONCLUSIONSRP shunt flow studies were of definite value in deciding whether to operatively intervene in patients with symptoms of shunt malfunction in whom no change in ventricular size was detected on current MRI or CT scans compared to scans obtained when the patients were asymptomatic.

scholarly journals Causes of death and comorbidities in hospitalized patients with COVID-19

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Sefer Elezkurtaj ◽  
Selina Greuel ◽  
Jana Ihlow ◽  
Edward Georg Michaelis ◽  
Philip Bischoff ◽  
...  
Keyword(s):  
Cause Of Death ◽  
Causes Of Death ◽  
Pulmonary Infection ◽  
Diffuse Alveolar Damage ◽  
University Hospital ◽  
Health Conditions ◽  
Teaching Hospitals ◽  
Increased Risk ◽  
Clinical Records ◽  
Full Body

AbstractInfection by the new corona virus strain SARS-CoV-2 and its related syndrome COVID-19 has been associated with more than two million deaths worldwide. Patients of higher age and with preexisting chronic health conditions are at an increased risk of fatal disease outcome. However, detailed information on causes of death and the contribution of pre-existing health conditions to death yet is missing, which can be reliably established by autopsy only. We performed full body autopsies on 26 patients that had died after SARS-CoV-2 infection and COVID-19 at the Charité University Hospital Berlin, Germany, or at associated teaching hospitals. We systematically evaluated causes of death and pre-existing health conditions. Additionally, clinical records and death certificates were evaluated. We report findings on causes of death and comorbidities of 26 decedents that had clinically presented with severe COVID-19. We found that septic shock and multi organ failure was the most common immediate cause of death, often due to suppurative pulmonary infection. Respiratory failure due to diffuse alveolar damage presented as immediate cause of death in fewer cases. Several comorbidities, such as hypertension, ischemic heart disease, and obesity were present in the vast majority of patients. Our findings reveal that causes of death were directly related to COVID-19 in the majority of decedents, while they appear not to be an immediate result of preexisting health conditions and comorbidities. We therefore suggest that the majority of patients had died of COVID-19 with only contributory implications of preexisting health conditions to the mechanism of death.

scholarly journals Patterns of multimorbidity and their effects on adverse outcomes in rheumatoid arthritis: a study of 5658 UK Biobank participants

BMJ Open ◽  
2020 ◽  
Vol 10 (11) ◽  
pp. e038829
Author(s):  
Ross McQueenie ◽  
Barbara I Nicholl ◽  
Bhautesh D Jani ◽  
Jordan Canning ◽  
Sara Macdonald ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Outcome Measures ◽  
Primary Outcome ◽  
Adverse Outcomes ◽  
Major Adverse Cardiovascular Events ◽  
Uk Biobank ◽  
Long Term Conditions ◽  
Increased Risk ◽  
All Cause Mortality ◽  
Clinical Records

ObjectiveTo investigate how the type and number of long-term conditions (LTCs) impact on all-cause mortality and major adverse cardiovascular events (MACE) in people with rheumatoid arthritis (RA).DesignPopulation-based longitudinal cohort study.SettingUK Biobank.ParticipantsUK Biobank participants (n=502 533) aged between 37 and 73 years old.Primary outcome measuresPrimary outcome measures were risk of all-cause mortality and MACE.MethodsWe examined the relationship between LTC count and individual comorbid LTCs (n=42) on adverse clinical outcomes in participants with self-reported RA (n=5658). Risk of all-cause mortality and MACE were compared using Cox’s proportional hazard models adjusted for lifestyle factors (smoking, alcohol intake, physical activity), demographic factors (sex, age, socioeconomic status) and rheumatoid factor.Results75.7% of participants with RA had multimorbidity and these individuals were at increased risk of all-cause mortality and MACE. RA and >4 LTCs showed a threefold increased risk of all-cause mortality (HR 3.30, 95% CI 2.61 to 4.16), and MACE (HR 3.45, 95% CI 2.66 to 4.49) compared with those without LTCs. Of the comorbid LTCs studied, osteoporosis was most strongly associated with adverse outcomes in participants with RA compared with those without RA or LTCs: twofold increased risk of all-cause mortality (HR 2.20, 95% CI 1.55 to 3.12) and threefold increased risk of MACE (HR 3.17, 95% CI 2.27 to 4.64). These findings remained in a subset (n=3683) with RA diagnosis validated from clinical records or medication reports.ConclusionThose with RA and other LTCs, particularly comorbid osteoporosis, are at increased risk of adverse outcomes, although the role of corticosteroids could not be evaluated in this study. These results are clinically relevant for the monitoring and management of RA across the healthcare system, and future clinical guidelines for RA should acknowledge the importance of multimorbidity.

Effects of Surgical Revascularization on Peripheral Artery Aneurysms in Moyamoya Disease: Report of Three Cases

Neurosurgery ◽  
2001 ◽  
Vol 49 (2) ◽  
pp. 463-468 ◽  
Author(s):  
Satoshi Kuroda ◽  
Kiyohiro Houkin ◽  
Hiroyasu Kamiyama ◽  
Hiroshi Abe
Keyword(s):  
Moyamoya Disease ◽  
Superficial Temporal Artery ◽  
Temporal Artery ◽  
Intracranial Bleeding ◽  
Peripheral Artery ◽  
Surgical Revascularization ◽  
Beneficial Effects ◽  
Collateral Arteries ◽  
Lenticulostriate Artery ◽  
Collateral Vessels

Abstract OBJECTIVE AND IMPORTANCE The beneficial effects of surgical revascularization on rebleeding in moyamoya disease remain unclear. This report is intended to clarify the effects of surgical revascularization on peripheral artery aneurysms, which represent one of the causes of intracranial bleeding in moyamoya disease. CLINICAL PRESENTATION Findings for three female patients who experienced intracranial bleeding are presented. Cerebral angiography revealed that intracranial bleeding resulted from the rupture of peripheral artery aneurysms arising from dilated collateral vessels such as the lenticulostriate artery. INTERVENTION The patients successfully underwent superficial temporal artery-middle cerebral artery anastomosis combined with encephaloduromyoarteriosynangiosis. Angiography demonstrated obliteration of the peripheral artery aneurysms, together with the disappearance or decrease in caliber of the parent collateral arteries, after surgery. None of the patients experienced rebleeding during the follow-up period (up to 52 mo). CONCLUSION The results strongly suggest that surgical revascularization potentially improves cerebral circulation and decreases hemodynamic stress on collateral vessels, obliterating peripheral artery aneurysms.

scholarly journals Atypical Posterior Reversible Encephalopathy Syndrome in a Postpartum Woman With Moyamoya Disease: A Case Report and Literature Review

2021 ◽  
Vol 12 ◽  
Author(s):  
Ning Zou ◽  
Guixiang Guo ◽  
Fangchao Wan ◽  
Xin Li
Keyword(s):  
Glasgow Coma Scale ◽  
Moyamoya Disease ◽  
Posterior Reversible Encephalopathy Syndrome ◽  
Reversible Posterior Leukoencephalopathy Syndrome ◽  
Postpartum Woman ◽  
Posterior Reversible Encephalopathy ◽  
Coma Scale ◽  
Posterior Leukoencephalopathy ◽  
Collateral Vessels ◽  
Reversible Encephalopathy

Background: Moyamoya disease is a rare cerebrovascular occlusive disease, which is characterized by stenosis and gradual occlusion of the internal carotid arteries, causing the progression of characteristic collateral vessels. To date, most studies investigating moyamoya disease have focused on medical implications, and the potential implications for neurocognitive and/or neuropsychiatric functioning were inconclusive.Case Presentation: we present a case of a 26-year-old Chinese postpartum woman who presented to the emergency department with a 19-h history of cognitive decline, vomiting, and convulsions. Blood pressure, heart rate, and respiration rate were 200/120 mmHg, 115 beats/minute, and 30 breaths/minute, respectively, on arrival. The Glasgow Coma Scale, modified RANKIN scale (mRS), and National Institute of Health stroke scale (NIHSS) scores were 3, 5, and 18, respectively. Moyamoya disease was diagnosed using cerebral angiography and digital subtraction angiography. The cognitive functions of orientation, use of language, ability to calculate, and memory significantly improved after 11 days of treatment (Glasgow Coma Scale: 15; mRS: 0; NIHSS: 0).Conclusions:This patient was diagnosed with reversible posterior leukoencephalopathy syndrome related to moyamoya disease. This case highlights that atypical posterior reversible encephalopathy syndrome can occur in patients with moyamoya disease, and should be considered for the differential diagnosis of cerebral infarcts and hemorrhage in a postpartum female.

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