children

Case Report A Rare Case of Spontaneous Arachnoid Rupture Presenting as Right Hemiplegia and Expressive Aphasia in a Pediatric Patient

Anne Bryden 1,*, Natalie Majors 2, Vinay Puri 3 and Thomas Moriarty 4

1 Department of Neurology and Pediatrics, University of Louisville SOM, Louisville, KY 40202, USA 2 Department of Neurology and Pediatrics, Vanderbilt University, Nashville, TN 37420, USA; [email protected] 3 Department of Neurology and Pediatrics, University of Louisville, Louisville, KY 40202, USA; [email protected] 4 Department of Neurological and Pediatrics, University of Louisville, Louisville, KY 40202, USA; [email protected] * Correspondence: [email protected]; Tel.: +1-(570)-951-2998

Abstract: This study examines an 11-year-old boy with a known history of a large previously asymptomatic (AC) presenting with acute onset of right facial droop, hemiplegia, and expressive aphasia. Shortly after arrival to the emergency department, the patient exhibited complete resolution of right-sided hemiplegia but developed and had persistent word- finding difficulties. Prior to symptom onset while in class at school, there was an absence of reported jerking movements, headache, , fever, or trauma. At the time of neurology consultation,

 the physical exam showed mildly delayed cognitive processing but was otherwise unremarkable. The  patient underwent MRI scanning of the , which revealed left convexity subdural hematohygroma

Citation: Bryden, A.; Majors, N.; and perirolandic cortex edema resulting from ruptured left frontoparietal AC. He was evaluated by Puri, V.; Moriarty, T. A Rare Case of and managed expectantly. He recovered uneventfully and was discharged two days Spontaneous Arachnoid Cyst after presentation remaining asymptomatic on subsequent outpatient visits. The family express Rupture Presenting as Right concerns regarding increased anxiety and mild memory loss since hospitalization. Hemiplegia and Expressive Aphasia in a Pediatric Patient. Children 2021, 8, Keywords: pediatrics; arachnoid ; spontaneous rupture; aphasia; neurological deficits 78. https://doi.org/10.3390/ children8020078

Academic Editor: Marco Carotenuto 1. Introduction Received: 15 November 2020 Arachnoid cysts (ACs) are cerebral spinal fluid (CSF) collections between two layers Accepted: 22 January 2021 Published: 24 January 2021 of arachnoid membrane that are primarily congenital in origin or acquired secondary to trauma, , or -based surgery. ACs are a relatively common developmental anomaly that are frequently diagnosed incidentally by intracranial imaging in pediatric Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in patients [1–3]. They represent 1% of all intracranial masses [4] with a prevalence of around published maps and institutional affil- 2.6% in children [1] and male predominance [1,5–7]. In general, arachnoid cysts have a iations. benign course with a large proportion of patients remaining asymptomatic. However, they can be associated with clinical symptoms, such as chronic headache, , asymmetric macrocrania, or [8,9]. Arachnoid cysts may also present with symptoms as a consequence of cyst rupture causing acute subdural hygroma, subdural , and/or intracystic hemorrhage [2]. Copyright: © 2021 by the authors. Larger arachnoid cyst size, a median size of 2.2 × 2.6 × 2.5 cm [5], and recent head trauma Licensee MDPI, Basel, Switzerland. This article is an open access article are risk factors for symptomatic rupture [10]. Spontaneous or nontraumatic rupture appear distributed under the terms and as a rare event, with a total of only 57 reported cases of spontaneous AC rupture [11]. conditions of the Creative Commons The most frequently reported symptom following spontaneous rupture is headache (82%) Attribution (CC BY) license (https:// and / (35.3%). Neurological deficits and altered consciousness are rarely creativecommons.org/licenses/by/ reported [11]. 4.0/).

Children 2021, 8, 78. https://doi.org/10.3390/children8020078 https://www.mdpi.com/journal/children Children 2021, 8, x FOR PEER REVIEW 2 of 6

Children 2021, 8, 78 and nausea/vomiting (35.3%). Neurological deficits and altered consciousness are rarely2 of 5 reported [11]. This case describes a rare spontaneous AC rupture, and, to our knowledge, this is the firstThis spontaneous case describes AC rupture a rare spontaneous that presents AC with rupture, acute and,onset to focal our knowledge,neurological this signs, is thenamely first spontaneousfacial nerve injury, AC rupture expressive that presents aphasia, withand hemiplegia. acute onset focal neurological signs, namely facial nerve injury, expressive aphasia, and hemiplegia. 2. Patient Description 2. PatientThe Descriptionpatient, an 11-year-old male with known history of a previously asymptomatic, left frontalThe patient, arachnoid an 11-year-old cyst, found male incidentally with known on imaging history after of a previouslypersistent asymptomatic, at age left4, was frontal in arachnoidhis normal cyst, state found of health incidentally until experiencing on imaging aftersudden-onset persistent headaches while at age in 4,class. was inShortly his normal after, statehe developed of health until a right-si experiencingded lower sudden-onset facial droop, dizziness right hemiplegia, while in class. and Shortlyexpressive after, aphasia. he developed There awas right-sided no history lower of trauma, facial droop, , right or hemiplegia, other neurological and expres- con- siveditions, aphasia. and the There patient was nowas history otherwise of trauma, in good seizures, health. These or other symptoms neurological began conditions, to resolve anden route the patient to a pediatric was otherwise tertiary in goodcare center health. vi Thesea a medical symptoms stat beganflight. toUpon resolve arrival, en route the tore- amaining pediatric symptoms tertiary care included center some via a medicalright arm stat we flight.akness, Upon mild-to-modera arrival, the remainingte dysarthria, symp- flat- tomstened included nasolabial some fold, right and arm facial , asymmetry mild-to-moderate on smiling. There dysarthria, was near-complete flattened nasolabial symp- fold,tom andresolution facial asymmetryat the time onof neurology smiling. There consultation was near-complete 5 min after symptominitial exam resolution and 70 min at thefollowing time of neurologysymptom onset. consultation The neurological 5 min after exam initial showed exam and mildly 70 min delayed following cognitive symptom pro- onset.cessing, The but neurological the cranial exam nerve, showed motor, mildly sensory, delayed coordination, cognitive and processing, gait exams but thewere cranial unre- nerve,markable. motor, There sensory, was coordination,an absence of and witnesse gait examsd/reported were unremarkable., abnormal There was move- an absencements, loss of witnessed/reported of consciousness, headache, convulsions, photop abnormalhobia, movements,or fever. There loss was of consciousness, no evidence of headache,external trauma photophobia, on physical or fever. exam There or report was no of evidence trauma preceding of external and/or trauma during on physical symp- examtoms. or report of trauma preceding and/or during symptoms. FiguresFigures1 1and and2 reveal2 reveal a changea change in signalin signal of arachnoidof arachnoid cyst cyst suggestive suggestive of intervalof interval hemorrhagehemorrhage with with a a thin thin subdural hematoma along along its its inferior inferior aspect. aspect. Minimal Minimal edema edema in in thethe subjacent subjacent Rolandic Rolandic cortex cortex was was present. present. Given Given the the patient’s patient’s -like stroke-like presentation, presentation, an an MRAMRA of of head head and and neck neck was was administered, administered, which which was was found found to to be be normal. normal. The The findings, findings, withwith aa comparisoncomparison of the previous previous MRI MRI of of th thee patient patient at atage age 4 (Figures 4 (Figures 3 and3 and 4),4 ),are are con- consistentsistent with with a ruptured a ruptured arachnoid arachnoid cyst. cyst. No Noacute acute neurosurgical neurosurgical intervention intervention was was per- performedformed due due to to the the absence absence of of raised raised intracranial and reductionreduction ofof neurological neurological symptoms. A routine EEG performed in the emergency department exhibited interictal symptoms. A routine EEG performed in the emergency department exhibited interictal epileptiform discharges over the left and right central–temporal region during drowsy and epileptiform discharges over the left and right central–temporal region during drowsy sleep states, suggestive of increased risk for partial seizures from these regions. and sleep states, suggestive of increased risk for partial seizures from these regions.

FigureFigure 1. 1.MRI MRI T2WI, T2WI, sagittal sagittal section section in in 2019 2019 (11 (11 years years of of age). age).

The patient received 500 mg levetiracetam twice daily for seizure prophylaxis. On day 2 of admission, a rapid-sequence MRI brain scan showed a stable thin subdural hematoma and a ruptured arachnoid cyst at the left frontoparietal vertex with the interval resolution of edema in the subjacent cortex. At discharge on day 3, the patient’s neurological exam remained normal without development of symptoms or signs related to acute intracranial hypertension. The patient maintained on 500 mg levetiracetam BID for seizure prophylaxis for three months. A rapid-sequence MRI brain scan at 2-week outpatient follow-up revealed a mild interval decrease in the size of left convexity subdural hematohygroma. He has had no return of rupture symptoms or concerns for seizures, including nocturnal seizures.

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Children 2021, 8, x FOR PEER REVIEWHe has occasional headaches, not sustained or requiring medication. The family express3 of 6

Children 2021, 8, x FOR PEER REVIEWconcerns regarding occasional anxiety and mild memory loss since hospitalization. 3 of 6

Children 2021, 8, x FOR PEER REVIEW 3 of 6

FigureFigure 2. 2.MRI MRI T2WI,T2WI, axialaxial sectionsection inin 2019.2019. A very thin subdural hematoma is is identified identified along along the the left convexity with mild edema noted in the subjacent perirolandic cortex. Decreased T2 signal is leftFigure convexity 2. MRI with T2WI, mild axial edema section noted in 2019. in the A subjacentvery thin subdural perirolandic hematoma cortex. is Decreased identified T2 along signal the is leftnow convexity identified with within mild the edema cyst when noted compared in the subjac to Figuresent perirolandic 3 and 4, whichcortex. is Decreased suggestive T2 of signal interval is nowFigure identified 2. MRI T2WI, within axial the cystsection when in 2019. compared A very to thin Figures subdural3 and hematoma4, which isis suggestiveidentified along of interval the nowhemorrhage. identified These within findings the cyst are when consiste comparednt with to a rupturedFigures 3 arachnoidand 4, which cyst. is suggestive of interval hemorrhage.left convexity These with mild findings edema are noted consistent in the with subjac a rupturedent perirolandic arachnoid cortex. cyst. Decreased T2 signal is hemorrhage. These findings are consistent with a ruptured arachnoid cyst. now identified within the cyst when compared to Figures 3 and 4, which is suggestive of interval hemorrhage. These findings are consistent with a ruptured arachnoid cyst.

Figure 3. MRI T2WI, sagittal section in 2012 (4 years of age).

FigureFigure 3.3.MRI MRI T2WI,T2WI, sagittalsagittal sectionsection inin 20122012 (4 (4 years years of of age). age). Figure 3. MRI T2WI, sagittal section in 2012 (4 years of age).

Figure 4. MRI T2WI, axial section in 2012. An extra-axial, well defined CSF structure lesion is iden- Figuretified in 4. the MRI left T2WI, vertex axial with section the following in 2012. measAn extra-axial,urements: wellanteroposterior, defined CSF 64 structure mm; oblique lesion cranio- is iden- Figure 4. MRI T2WI, axial section in 2012. An extra-axial, well defined CSF structure lesion is iden- Figuretifiedcaudal, in 4. 53theMRI mm; left T2WI, vertextransverseaxial with dimensions, sectionthe following in 2012. 23 meas mm. Anurements: This extra-axial, lesion anteroposterior, causes well defineda moderate 64 CSF mm; compression structure oblique lesion cranio- effect is tified in the left vertex with the following measurements: anteroposterior, 64 mm; oblique cranio- identifiedcaudal,of the underlying 53 in mm; the transverse left frontal vertex and dimensions, with pari theetal following gyri 23 mm. at the This measurements: vertex lesion with causes scalloping anteroposterior, a moderate of the compressioninner 64 table. mm; These oblique effect caudal, 53 mm; transverse dimensions, 23 mm. This lesion causes a moderate compression effect offindings the underlying are consistent frontal with and a parimoderate-sizedetal gyri at the arachnoid vertex with cyst scalloping at the left frontoparietalof the inner table. vertex. These craniocaudal,of the underlying 53 mm; frontal transverse and pari dimensions,etal gyri at the 23 vertex mm. with This scalloping lesion causes of the a moderate inner table. compression These findings are consistent with a moderate-sized arachnoid cyst at the left frontoparietal vertex. effectfindings of the are underlying consistent frontalwith a mo andderate-sized parietal gyri arachnoid at the vertex cyst withat the scalloping left frontoparietal of the inner vertex. table. These findingsThe are patient consistent received with a moderate-sized500 mg levetiracetam arachnoid twice cyst at daily the left for frontoparietal seizure prophylaxis. vertex. On day 2The of admission,patient receivedreceived a rapid-sequence 500500 mg mg levetiracetam levetiracetam MRI brain twice twice scan daily showeddaily for for seizurea seizurestable prophylaxis. thin prophylaxis. subdural On Onhe- daymatoma 2 of admission,and a ruptured aa rapid-sequencerapid-sequence arachnoid cyst MRI MRI at brain thebrain left scan scan frontoparietal showed showed a stablea stablevertex thin thin with subdural subdural the interval he- he- matomaresolution and of aedemaa rupturedruptured in the arachnoidarachnoid subjacent cyst cyst cortex. at at the the At left leftdischarge frontoparietal frontoparietal on day vertex 3, vertex the withpatient’s with the the intervalneurolog- interval resolutionical exam remainedofof edemaedema ininnormal the the subjacent subjacent without cortex. cortex. developm At At discharge dischargeent of symptoms on on day day 3, 3, theor the signspatient’s patient’s related neurolog- neurolog- to acute icalical exam remainedremained normalnormal without without developm developmentent of of symptoms symptoms or or signs signs related related to toacute acute

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3. Discussion ACs are CSF collections between layers of arachnoid membrane that typically present as asymptomatic and represent 1% of all intracranial masses [4]. In general, arachnoid cysts have a benign course with a large proportion of patients remaining asymptomatic. However, they can be associated with clinical symptoms, such as chronic headache, seizure, asymmetric macrocrania, or hydrocephalus. from recent trauma is the most common cause of AC rupture; rarely do they rupture spontaneously, reflected by the fact that there are only 57 reported cases of spontaneous AC rupture. In the case of traumatic rupture, these lesions are more associated with subdural hygroma, subdural hematoma, and/or intracystic hemorrhage, across all ages [2]. Different treatment options for rupture of ACs are very controversial. Immediate operative intervention, either through microsurgical fenestration or CSF derivation, is warranted by raised intracranial pressure and progressive neurological deterioration [11–13]. Conservative treatment has also been proposed depending on a patient’s intracranial pressure and neurological condition [9]. In the case of our patient, conservative treatment was favored due to only mild , no significant rise in intracranial pressure, and the reduction—and eventual cessation—of neurological symptoms. Our patient was diagnosed with non-traumatic rupture of left frontoparietal arachnoid cyst with associated subdural hematohygroma. This resulted in a transient right hemiplegia, right facial droop, and expressive aphasia. Presumably, these symptoms are secondary to the cyst rupture, release of proteinaceous fluid, and hemorrhage with subsequent inflammation. ACs have a prevalence of 2.6% in children [1], with a higher preponderance in males [1,5–7]. As reported in many studies, the main symptoms following spontaneous AC rupture are headache and nausea/vomiting [11,12], whereas focal neurological deficits are rare [2], this being the first case to our knowledge with hemiplegia and expressive aphasia. Our patient did not experience prolonged headaches or nausea/vomiting; instead, he presented with acute onset focal neurological symptoms. Additionally, our patient did not suffer from a traumatic event, and his AC ruptured spontaneously, which is extremely rare. The family concerns regarding increased anxiety and mild memory loss since hospitalization may be attributed to the possible side effect of levetiracetam [14]. Anticipatory guidance in pediatric patients diagnosed with arachnoid cysts is disputed among pediatric neurosurgeons. ACs are a relatively common developmental anomaly, which are frequently diagnosed incidentally by intracranial imaging in pediatric patients, leading most physicians to recommend conservative observational treatment [1–3]. When the patient is asymptomatic with a small AC, no treatment is the preferred course of action with periodic imaging to monitor the cyst’s growth. However, if symptoms arise or the cyst ruptures, the cyst will be re-evaluated and surgical treatment, via open fenes- tration, endoscopic cyst fenestration, or cysto-peritoneal shunting [11], is recommended [1]. Larger arachnoid cyst size and recent head trauma are risk factors for symptomatic rupture. Mild head trauma is extremely common in children’s daily lives, and while the trauma can potentially lead to rupture, physicians believe that parents should be aware of this risk but not restrict sports or other activities for most children with ACs [10]. Discussion of risk factors, size of AC, and mild head trauma during follow up with families and caretakers of patients with ACs is very important. Additionally, these groups need to receive extra guidance on the potential consequences of rupture, ranging from benign symptoms to subdural hygroma, subdural hematoma, intracystic hemorrhage, and edema with the potential for neurosurgical intervention. Furthermore, the potential for acute neurological symptoms that mimic or needs to be included in the potential consequences of AC rupture, spontaneous or traumatic.

4. Conclusions Rupture of an arachnoid cyst must be in the differential diagnosis of acute onset of focal neurological symptoms. The symptoms can mimic a stroke or a complex . Patients with arachnoid cysts must be educated about these consequences from rupture Children 2021, 8, 78 5 of 5

of a cyst, and, thus, the potential for stroke-like symptoms should be part of anticipatory counselling in patients with arachnoid cysts.

Author Contributions: A.B. researched the literature and reviewed the patient’s chart, assisted in the initial manuscript, and revised the initial and final manuscript. N.M. researched the literature, assisted in the initial manuscript, and revised the initial manuscript. V.P. examined the patient, reviewed the initial and final manuscript, and approved the final manuscript. T.M. reviewed and approved the final manuscript. All authors have read and agreed to the published version of the manuscript. Funding: This research received no external funding. Informed Consent Statement: Informed consent was obtained from all subjects involved in the study. Data Availability Statement: The data presented in this study is available upon request from the corresponding author. The data are not publicly available due to privacy of the patient. Conflicts of Interest: The authors declare no conflict of interest.

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