Coexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update

Central precocious puberty (CPP) is a rare disease. The mean annual incidence in girls is 0.8-1.1/100,000 and in boys 0-0.1/100,000. Intracranial arachnoid cysts (ICACs) are usually congenital and represent 1% of intracranial masses in newborns. Intraventricular location is rare. The objective of this work is to carry out a literature updated review of the coexistence of CPP and intraventricular arachnoid cyst (IVAC). ICACs are usually asymptomatic but can present with CPP in 10-40% of patients. IVACs represents only 0.3-1.4% of ICACs, and most seemed originate from the velum interpositum cistern. CPP in girls is usually idiopathic, while in 30-70% of boys are due to an intracranial lesion. Therefore, the coexistence of PPC and IVAC is very rare in boys and exceptional in girls. The exact mechanism of a cyst´s influence on the hypothalamic-pituitary axis is not completely understood. Theories include increased ventricular volume, associated mass effect on the hypothalamus, and direct compression of portions of the hypothalamic-pituitary axis. Analysis of LH peaks after GnRH testing is the gold standard for the diagnosis of CPP. Brain MRI should be part of the assessment in boys and also in girls since clinical features, including age and sex, are not helpful in predicting those with underlying brain pathology. In cases of CPP with IVAC, surgery does not have any effect on the course of pubertal development. The indication for surgery is the onset of neurological symptoms. The medical treatment selected, safe and effective, is GnRH analog depot preparations. In conclusion, there seems to be a consensus for the diagnosis and management of the coexistence of CPP and IVAC, but the etiopathogenesis is not yet well recognized (AU)

A Case of Miyazaki Syndrome Caused by Arachnoid Cyst-Peritoneal Shunt.

BACKGROUND: Miyazaki syndrome is overshunting-associated myelopathy, which is a rare complication of ventriculoperitoneal shunt. We present the first case of Miyazaki syndrome caused by cystoperitoneal (CP) shunt for an arachnoid cyst (AC) in this report. CASE DESCRIPTION: We report a case of a 42-year-old man with 12-year progressive spastic paraplegia, who underwent CP shunt for an AC at the age of 15 years. Although few findings suggested overshunting on symptoms and head computed tomography, contrast-enhanced magnetic resonance imaging revealed the engorgement of the cervical spinal epidural venous plexus compressing the spinal cord. Shunt valve replacement with a pressure-adjustable valve was performed. Postoperatively, the cervical cord compression by the enlarged spinal epidural venous plexus was completely improved, but, possibly due to delayed diagnosis and treatment, the patient's symptoms only partially improved. CONCLUSIONS: When patients with a history of any kind of shunt surgery develop myelopathy, Miyazaki syndrome should be suspected and, for early diagnosis, cervical and/or contrast-enhanced magnetic resonance imaging should be performed.

Spontaneous Resolution of Asymptomatic Pediatric Suprasellar Arachnoid Cysts: Report of 2 Cases and Review of the Literature.

INTRODUCTION: Sellar/suprasellar arachnoid cysts are infrequent nonneoplastic cystic lesions that account for approximately 1% of all arachnoid cysts. While various surgical treatment options have been proposed for symptomatic lesions, treatment guidelines regarding the management of asymptomatic sellar/suprasellar arachnoid cysts remain to be elucidated. This case series aims to provide a better insight into the management and outcomes of such lesions. CASE PRESENTATION: We discuss 2 pediatric patients diagnosed with sellar/suprasellar arachnoid cysts who remained asymptomatic and demonstrated spontaneous gradual resolution of their arachnoid cysts on subsequent imaging studies. CONCLUSION: Review of the literature identified only a handful of previously reported cases of spontaneously disappearing suprasellar arachnoid cysts. There might be a role for conservative management of sellar/suprasellar arachnoid cysts in certain patients who remain asymptomatic.

Does subjective improvement in adults with intracranial arachnoid cysts justify surgical treatment?

OBJECTIVE Subjective improvement of patients who have undergone surgery for intracranial arachnoid cysts has justified surgical treatment. The current study aimed to evaluate the outcome of surgical treatment for arachnoid cysts using standardized interviews and assessments of neuropsychological function and balance. The relationship between arachnoid cyst location, postoperative improvement, and arachnoid cyst volume was also examined. METHODS The authors performed a prospective, population-based study. One hundred nine patients underwent neurological, neuropsychological, and physiotherapeutic examinations. The arachnoid cysts were considered symptomatic in 75 patients, 53 of whom agreed to undergo surgery. In 32 patients, results of the differential diagnosis revealed that the symptoms were due to a different underlying condition and were unrelated to an arachnoid cyst. Neuropsychological testing included target reaction time, Grooved Pegboard, Rey Auditory Verbal Learning, Rey Osterrieth complex figure, and Stroop tests. Balance tests included the extended Falls Efficacy Scale, Romberg, and sharpened Romberg with open and closed eyes. The tests were repeated 5 months postoperatively. Cyst volume was pre- and postoperatively measured using OsiriX software. RESULTS Patients who underwent surgery did not have results on balance and neuropsychological tests that were different from patients who declined or had symptoms unrelated to the arachnoid cyst. Patients with a temporal arachnoid cyst performed within the normal range on the neuropsychological tests. Seventy-seven percent of the patients who underwent surgery reported improvement, yet there were no differences in test results before and after surgery. Arachnoid cysts in the temporal region and posterior fossa did not influence the preoperative results of neuropsychological and motor tests. The arachnoid cyst volume decreased postoperatively (p < 0.0001), but there was no relationship between volume reduction and clinical improvement. CONCLUSIONS The results of this study speak against objectively verifiable improvement following surgical treatment in adults with intracranial arachnoid cysts.

Trigeminal neuropathy associated with an enlarging arachnoid cyst in Meckel's cave: case report, management strategy and review of the literature.

We describe a rare case and novel management strategy of painful trigeminal neuropathy caused by an arachnoid cyst confined to Meckel's cave. A 57-year-old female presented with several years of progressive trigeminal pain and signs of trigeminal deafferentation, including sensory loss, corneal anesthesia and mastication muscle atrophy. Medical treatment with carbamazepine provided partial and temporary pain control. Surgical treatment was eventually performed by aspiration of the arachnoid cyst through the foramen ovale using a percutaneous approach. The patient experienced relief of pain and improvement of numbness and muscle strength. To our knowledge, this is the first case description of a percutaneous drainage of a Meckel's cave arachnoid cyst.

Long-term endocrine outcome of suprasellar arachnoid cysts.

OBJECTIVE Due to their distinct location, suprasellar arachnoid cysts are known to cause a wide variety of problems, such as hydrocephalus, endocrine symptoms, and visual abnormalities. The long-term outcome of these cysts has not been elucidated. To find out the long-term outcome of suprasellar arachnoid cysts, a retrospective review of the patients was performed. The neurological and endocrine symptoms were thoroughly reviewed. METHODS Forty-five patients with suprasellar arachnoid cysts, with an average follow-up duration of 9.7 years, were enrolled in the study. A comprehensive review was performed of the results of follow-up regarding not only neurological symptoms but also endocrine status. The outcomes of 8 patients who did not undergo operations and were asymptomatic or had symptoms unrelated to the cyst were included in the series. RESULTS Surgery was most effective for the symptoms related to hydrocephalus (improvement in 32 of 32), but endocrine symptoms persisted after surgery (4 of 4) and required further medical management. More surprisingly, a fairly large number of patients (14 of 40; 1 was excluded because no pre- or postoperative endocrine evaluation was available) who had not shown endocrine symptoms at the time of the initial diagnosis and treatment later developed endocrine abnormalities such as precocious puberty and growth hormone deficiency. The patients with endocrine symptoms detected during the follow-up included those in both the operated (n = 12 of 32) and nonoperated (n = 2 of 8) groups who had been stable during follow-up since the initial diagnosis. CONCLUSIONS This study implies that patients with suprasellar arachnoid cysts can develop late endocrine problems during follow-up, even if other symptoms related to the cyst have been successfully treated. Hence, patients with these cysts need long-term follow-up for not only neurological symptoms but also endocrine abnormalities.

A patient with myasthenia gravis and a large arachnoid cyst - report of a case.

Myasthenia gravis is a chronic autoimmune disease characterized by weakening of voluntary muscles during the day and a marked restitution of function during the night and after rest. The symptoms may worsen over days or weeks, sometimes even in a few hours, and are usually well controlled by appropriate therapy. Arachnoid cysts are congenital or acquired deformities of the arachnoid membrane and are usually too small to cause distinct clinical symptomatology. We describe a case of a 76-year-old myasthenia gravis patient with an arachnoid cyst. To the best of our knowledge this is the first reported case of these two comorbidities together.

[Left temporal arachnoid cyst and specific learning disorders associated with Pervasive Developmental Disorders - Not Otherwise Specified (PDD-NOS): contributions of an integrative neuropsychomotor, neuropsychological, psychopathological and neurosurgical approach about a case report in a child (François)]. / Kyste arachnoïdien temporal gauche et troubles spécifiques des apprentissages associés à un trouble envahissant du développement non spécifié (TED-NoS) : apports d'une approche intégrative neuro-psychomotrice neuropsychologique, psychopathologique et neurochirurgicale à propos d'une observation chez un enfant (le cas François).

Left temporal arachnoid cyst and specific learning disorders associated with pervasive developmental disorders - not otherwise specified (PDD-NOS): contributions of an integrative neuro-psychomotor, neuropsychological, psychopathological and neurosurgical approach about a case report in a child (François). With DSM-IV and DSM-IV-TR, the terminology of pervasive developmental disorders (PDD) covers two main categories of infantile disorders: disorders of "strictly" autistic nature and pervasive developmental disorders - not otherwise specified (PDD-NOS). Under the terminology of multiple complex developmental disorder (MCDD), it is proposed to classify children presenting symptoms approaching the psychotic disharmonies and usually diagnosed as PDD-NOS. Such a category of developmental disorders is now included without nosographic distinction in the autistic spectrum in the Diagnostic and Statistical Manual of mental disorders (DSM-V). CASE REPORT: We are reporting a case report of a 6-year-old boy which shows a PDD-NoS/MCDD complex symptomatology type. This child presents multiple disorders: minor neurological signs (soft signs), neuro-psychomotor disorders, developmental coordination disorder (DCD), communication, thought, and regulation of emotions disorders, attention deficit disorders (ADD); in the presence of a high verbal intellectual potential, which makes it difficult to establish a clear diagnosis. A cerebral magnetic resonance imaging (MRI) was carried out due to the presence of minor neurological signs (soft signs) and of neurodevelopmental multiple disorders. The MRI revealed a voluminous arachnoid temporo-polar left cyst with a marked mass effect on the left temporal lobe. DISCUSSION: A neurosurgical intervention allowed to observe the gradual disappearance of the specific symptomatology (in particular soft signs, neuro-psychomotor functions and autistic symptoms) secondary to the interference of the cyst's pressure with intracranial areas involving neurological and psychopathological abnormalities, underlying at the same time the reversibility of the disorders after decompression as demonstrated in some studies. There are always, with a quantitative and qualitative decrease, an emotional dysregulation, a DCD, an ADD as well as impairments in the executive functions. CONCLUSION: This clinical case underlines the necessity of an evaluation in a transdisciplinary way and to follow the developmental evolution of the child in order to focus adapted therapeutics. Furthermore, with neurodevelopmental disorders not specified, it is important to examine the presence of soft signs with standardized neuro-psychomotor assessment, and then, to propose an MRI investigation. To our knowledge, this is the first report in the literature with a school age child of an unusual association between a temporal arachnoid cyst associated with PDD-NOS/MCDD.

Quistes aracnoideos de localización infrecuente. Quistes extratemporales / Rare location of arachnoid cysts. Extratemporal cysts

El manejo terapéutico de los quistes aracnoideos depende, en gran medida, de su localización. Casi el 50% de los quistes aracnoideos se localizan en la fosa temporal-cisura de Silvio. En cambio, la otra mitad se reparte en localizaciones diversas, a veces excepcionales. En este trabajo describimos, bajo la denominación de «quistes aracnoideos de localización infrecuente», aquellos compuestos por las 2 hojas de membrana aracnoidea que no se encuentran localizados en la fosa temporal y que son primarios o de origen congénito

The therapeutic management of arachnoid cysts depends largely on its location. Almost 50% of arachnoid cysts are located in the temporal fossa-Sylvian fissure, whereas the other half is distributed in different locations, sometimes exceptional. Under the name of infrequent location arachnoid cysts, a description is presented of those composed of 2 sheets of arachnoid membrane, which are not located in the temporal fossa, and are primary or congenital

[Arachnoid cysts of the central nervous system. Algorithms and recommendations for management]. / Quistes aracnoideos del sistema nervioso central. Algoritmos y recomendaciones generales de manejo.

The symptoms related to the presence of arachnoid cysts in the Central Nervous System depend on the size of the cyst and its growth rate, its location and, in some cases, the associated CSF dynamic disorder. Sometimes there is acute clinical presentation due to cyst rupture or acute bleeding. Although it is generally accepted that asymptomatic or paucisymptomatic cysts do not require surgical treatment, there is no consensus on the therapeutic approach of choice in symptomatic cases. The aim of this paper is to review the literature, analyzing the pros and cons of the three main surgical options (microsurgery, neuroendoscopy, and CSF shunt) based primarily on the location of the cyst. Although treatment must be always individualized, basic management recommendations may be offered.

Spontaneous Shrinkage of a Suprasellar Arachnoid Cyst Diagnosed with Prenatal Sonography and Fetal Magnetic Resonance Imaging: Case Report and Review of the Literature.

Arachnoid cysts (AC's) are congenital anomalies that occur within the cerebrospinal fluid (CSF) cisterns and major cerebral fissures. Suprasellar AC's comprise 5-12.5% of all the lesions. Despite being commonly reported, their etiology and pathogenesis still remain unclear. In this report, we presented an unusual case of spontaneous shrinkage of a suprasellar AC that had been incidentally diagnosed during a routine prenatal sonographic examination. To our knowledge, only three cases of spontaneous shrinkage of suprasellar AC have been reported in the literature. In detail of the manuscript, fetal and postnatal radiological images were presented and the possible mechanisms were discussed with a review of the literature.

Petrous and sphenoid arachnoid cysts: diagnosis and management.

BACKGROUND: Intraosseous arachnoid cysts are rare and difficult to diagnose. The purpose of this study was to describe the clinical and radiological semiology of petrous and sphenoid arachnoid cysts and to propose a specific management strategy. METHODS: This was a retrospective, descriptive study of patients with arachnoid cysts, which utilized CT, MRI, and the patients' medical histories. RESULTS: Ten patients were included in this study. On CT, the lesions were lytic with bony delineation. On MRI, the lesions exhibited the same signals as cerebrospinal fluid and were not enhanced after contrast. On fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted sequences, the arachnoid cysts' signal disappeared, which is a key feature for eliminating the diagnosis of cholesteatoma. Two patients underwent surgery because of misdiagnosis, either with a meningocele or a cholesteatoma. CONCLUSION: MRI FLAIR and diffusion-weighted sequences, together with osseous CT scans, help to distinguish arachnoid cysts from meningoceles and avoid unnecessary surgeries with potential complications.

Leptomeningeal rheumatoid nodules: diagnosis and failed therapeutics.

A 67-year-old woman presented with recurrent transient ischaemic attack-like episodes over a 2 year period. Nodular enhancing leptomeningeal changes were detected on MRI and were consistent with meningeal rheumatoid nodules on biopsy. The patient's nodular disease continued to progress and regress clinically and radiologically irrespective of disease modifying agents and peripheral and serological rheumatoid arthritis control. This patient's unique presentation and diagnostic work-up is discussed alongside the dilemma of therapeutic management of meningeal rheumatoid nodules.

Characteristics and management of arachnoid cyst in the pediatric headache clinic setting.

OBJECTIVE/BACKGROUND: Arachnoid cysts are generally identified incidentally on brain imaging, although they occasionally cause symptoms because of expansion or bleeding. This study aims to describe patients in whom an arachnoid cyst was identified on magnetic resonance imaging (MRI) study performed for the evaluation of headache in a pediatric headache clinic and to highlight the clinical dilemma posed by this finding. METHODS: A retrospective descriptive study design was used. The electronic database of a tertiary pediatric headache clinic was searched for all newly admitted patients with headache who underwent MRI evaluation in 2008-2013. The indications for imaging were based on clinical practice parameters recommended by the Subcommittee of the American Academy of Neurology. Clinical and imaging parameters were collected from the files. Findings were compared between patients with and without an arachnoid cyst. RESULTS: Of the 250 (31%) of 800 patients who met the inclusion criteria, 11 (4.4%) had an arachnoid cyst. Two patients had a ruptured cyst with midline shifting and a large subdural collection. Both presented with headache, vomiting, phonophobia, and photophobia. In the other 9 asymptomtic patients with an arachnoid cyst, imaging showed only a mild mass effect without midline shifting; their symptoms were considered unrelated to the cyst. The patients with a symptomatic arachnoid cyst were referred for surgery, with good outcome. CONCLUSIONS: Arachnoid cysts are found in a small percentage of brain scans performed for evaluation of headache in the setting of a hospital-based pediatric headache clinic. For the long run in these clinical settings, most of the cysts are asymptomatic. Precise anamnesis, neurologic examination, and imaging performed according to accepted practice guidelines may help clinicians determine if the headache and symptoms are caused by the cyst or if they should seek primary headache diagnosis with overlapping symptoms. The clinical distinction between symptomatic and asymptomatic patients (symptoms that are directly related to the arachnoid cyst) may be difficult. Family history of migraine may help in the diagnosis of asymptomatic patients.

Extensive middle cranial fossa arachnoid cysts and different clinical presentation in two patients.

Arachnoid cysts (ACs), particularly suprasellar cysts, cause a wide spectrum of endocrine disorders. Herein, we report two patients diagnosed with an extensive AC in the middle cranial fossa while being investigated for etiologies of precocious puberty and short stature. One of them required surgery due to his pubertal disorders associated with compression effects of cyst. After surgery, his puberty progression was regressed within one year. On the other hand, surgery was not planned for the second patient considering of his cranial imaging findings and the extremely low incidence of growth hormone (GH) deficiency caused by middle fossa AC (MFAC). We started treatment with recombinant human GH and no complication was found during treatment follow-up. Endocrine disorders associated with MFACs are extremely rare. By presenting with these two cases, we aimed to remain our fellow physcians that ACs can be possibly cause of endocrine disorders. Clinicians should be careful evaluating endocrine disorders because real cause may not be cyst itself but masked by it.

Management of subdural hygromas associated with arachnoid cysts.

OBJECT: Arachnoid cysts may occasionally be associated with subdural hygromas. The management of these concurrent findings is controversial. METHODS: The authors reviewed their experience with arachnoid cysts and identified 8 patients with intracranial arachnoid cysts and an associated subdural hygroma. The medical records and images for these patients were also examined. RESULTS: In total, 8 patients presented with concurrent subdural hygroma and arachnoid cyst. Of these 8 patients, 6 presented with headaches and 4 had nausea and vomiting. Six patients had a history of trauma. One patient was treated surgically at the time of initial presentation, and 7 patients were managed without surgery. All patients experienced complete resolution of their presenting signs and symptoms. CONCLUSIONS: Subdural hygroma may lead to symptomatic presentation for otherwise asymptomatic arachnoid cysts. The natural course of cyst-associated subdural hygromas, even when symptomatic, is generally benign, and symptom resolution can be expected in most cases. The authors suggest that symptomatic hygroma is not an absolute indication for surgical treatment and that expectant management can result in good outcomes in many cases.