Symptomatic intracranial hypertension in an adult patient with spinal muscular atrophy and arachnoid cysts receiving nusinersen.

 In patients with spinal muscular atrophy (SMA) headache after intrathecal administration of nusinersen is usually attributed to post-lumbar puncture syndrome. However, lumbar puncture opening pressure (LOP) has also been reported to be increased in children with SMA, both before and after treatment with nusinersen, although symptoms associated with increased LOP were not observed. We report to our knowledge the first case of symptomatic intracranial hypertension in an adult SMA patient. This 21-year-old man suffered from headache and vomiting followed by visual disturbances after the 12th injection of nusinersen. Bilateral papilledema was recognized ophthalmologically. MRI of the head showed signs of intracranial hypertension and additionally arachnoid cysts but not hydrocephalus. Symptoms resolved after 8 weeks of treatment with repeated lumbar punctures and acetazolamide. This case raises the possibility of intracranial hypertension as a complication of nusinersen therapy although arachnoid cysts represent another risk factor for intracranial hypertension. We recommend that patients suffering from headache after nusinersen injections should not only be questioned and examined for symptoms suggestive of post-lumbar puncture syndrome, but also intracranial hypertension.

Surgical techniques used in the management of intra-arachnoid diverticula in dogs across four referral centres and their immediate outcome.

OBJECTIVES: To report the surgical techniques being used to treat single focal spinal intra-arachnoid diverticula in dogs, their complications and immediate postoperative outcomes. MATERIALS AND METHODS: Retrospective multi-centre case series across four referral centres. RESULTS: Fifty-seven dogs were included in the study. The most common type of surgery was durectomy (28 dogs) followed by marsupialisation (11 dogs), durotomy alone (seven dogs), shunt placement (six dogs) and stabilisation (five dogs). A higher proportion of intra-arachnoid shunt dogs became unable to walk in the immediate postoperative period (24 hours postsurgery) (4/6, 66%) compared to all dogs five of 57, 9% (2/7 durotomy alone, 3/28 durectomy alone). Of the nine dogs with immediate postoperative deterioration, seven had improved, walking without assistance, by 3 to 5 weeks postoperatively. CLINICAL SIGNIFICANCE: This study does not identify an influence of surgical technique on short-term outcome. Dogs with a thoracolumbar intra-arachnoid diverticulum that undergo a shunt placement are likely to deteriorate neurologically in the immediate 24-hour postoperative period but appear to improve by 3 to 5 weeks after surgery. Further work is required to evaluate whether one surgical technique is superior for preventing or reducing long-term relapse.

Suprasellar Cysticercosis Cyst with Optic Nerve Compression Masquerading as an Arachnoid Cyst.

Cysticercosis is a parasitic infection caused by the larvae of the cestode Taenia solium. Ocular parasitosis in humans is well recognized; however, cysticercosis of the optic nerve is rare. Here, we report a case of an adult male who presented with right-sided headache and a gradual loss of vision in the right eye. Optical coherence tomography indicated severe loss of ganglion cells in the right eye. Magnetic resonance imaging showed a predominantly suprasellar cystic lesion thought to represent an arachnoid cyst. We performed a craniotomy to excise the cyst. Histopathological examination of the excised cyst revealed internal living larvae of T. soilum. After co-administration of praziquantel and albendazole, vision was restored, and the headaches ceased. Vision has since been restored in both eyes. A higher degree of neurocysticercosis suspicion should be maintained for patients living in endemic areas who present with ophthalmic symptoms where the brain scans show cystic lesions.

Thoracic Arachnoid Cyst Made Symptomatic by Demyelination.

Spinal arachnoid cysts are uncommon distinct pouches of cerebrospinal fluid (CSF) or CSF-like fluid found adjacent to normal CSF spaces commonly extradural and rarely intradural. They are usually asymptomatic and discovered incidentally. We present a patient with rapid upper motor neuron neurologic deterioration over the course of 1 week. Findings on magnetic resonance imaging revealed various central nervous system demyelination lesions and thoracic arachnoid cyst with cord compression. This acute presentation, in the absence of trauma, is not in favor of the natural history of the intradural thoracic arachnoid cysts. The patient's sensory, gait, and lower limb neurologic deficits improved after surgery with residual mild but stable upper limb deficits. Our patient likely had a static compensated cyst made symptomatic by demyelination as an additional central nervous system lesion.

Spinal Arachnoid Diverticula: Outcome in 96 Medically or Surgically Treated Dogs.

BACKGROUND: Little is reported about the role of medical management in the treatment of spinal arachnoid diverticula (SAD) in dogs. OBJECTIVES: To describe the outcome of 96 dogs treated medically or surgically for SAD. ANIMALS: Ninety-six dogs with SAD. METHODS: Retrospective case series. Medical records were searched for spinal arachnoid diverticula and all dogs with information on treatment were included. Outcome was assessed with a standardized questionnaire. RESULTS: Fifty dogs were managed medically and 46 dogs were treated surgically. Dogs that underwent surgery were significantly younger than dogs that received medical management. No other variables, related to clinical presentation, were significantly different between both groups of dogs. The median follow-up time was 16 months (1-90 months) in the medically treated and 23 months (1-94 months) in the surgically treated group. Of the 38 dogs treated surgically with available long-term follow-up, 82% (n = 31) improved, 3% (n = 1) remained stable and 16% (n = 6) deteriorated after surgery. Of the 37 dogs treated medically with available long-term follow-up, 30% (n = 11) improved, 30% (n = 11) remained stable, and 40% (n = 15) deteriorated. Surgical treatment was more often associated with clinical improvement compared to medical management (P = .0002). CONCLUSIONS AND CLINICAL IMPORTANCE: The results of this study suggest that surgical treatment might be superior to medical treatment in the management of SAD in dogs. Further studies with standardized patient care are warranted.

Arachnoid cyst: a further anomaly associated with Kallmann syndrome?

BACKGROUND: Kallmann syndrome (KS) is defined by the association of hypogonadotropic hypogonadism and anosmia. It is characterized by a significant clinical and genetic heterogeneity; actually, it may present several non-reproductive non-olfactory anomalies, and all the ways of genetic transmission can be involved in the inheritance of the disease. Although six pathogenesis-related genes have been identified so far, KS remains sporadic in 70 % of the cases, and the genetic diagnosis is not available for all of them. The purpose of this paper is to present a further disease that can enrich the wide spectrum of KS variability, that is cerebral arachnoid cyst. CASE DESCRIPTION: This 11-year-old boy presented with the typical characteristics of KS together with those related to a sylvian arachnoid cyst. He was admitted because of worsening headache. At the admission, the physical examination revealed eunuchoid aspect, micropenis, previous cryptorchidism, and anosmia. MRI pointed out a large, left sylvian arachnoid cyst, agenesia of the olfactory bulbs/tracts complex, and hypoplasia of the left olfactory sulcus. The child was operated on by endoscopic fenestration of the cyst, followed by transient external drainage for subdural hygroma and microscopic fenestration for recurrence of the cyst. His statural growth is normal but the sexual development still delayed in spite of hormone replacement therapy. CONCLUSION: According to the present and the other four cases in the literature, arachnoid cyst should be included among the anomalies possibly accompanying KS date although this association seems to be occasional as far as embryogenesis and physiopathology are concerned.

Quiste aracnoideo bilateral del ángulo pontocerebeloso / Bilateral cerebellopontine arachnoid cyst

Introducción. Los quistes aracnoideos bilaterales del ángulo pontocerebeloso son excepcionales, y únicamente existen tres casos publicados en la bibliografía. Caso clínico. Niña de 14 años, previamente sana, que acude a consultas por presentar cefalea bifrontal de seis semanas de evolución. La exploración clínica era normal y la resonancia magnética craneal mostraba dos lesiones extraaxiales localizadas en ambos ángulos pontocerebelosos, siendo ligeramente mayor la izquierda. Las lesiones se comportaban como homogéneamente intensas en T1 e hiperintensas en T2, no captaban contraste y no existía restricción en las secuencias de difusión. No se indicó tratamiento quirúrgico. Conclusiones. Los quistes aracnoideos bilaterales situados en el ángulo pontocerebeloso son excepcionales. La principal indicación para el tratamiento quirúrgico es la presencia de síntomas o signos neurológicos coincidentes con la localización de los quistes (AU)

Introduction. Bilateral cerebellopontine arachnoid cysts are very rare, and only three cases have been previously reported. Case report. A 14-year-old previously healthy girl presented to our outpatient clinic with a 6-weeks history of frontal headache. They typically would start in the occipital region and then radiate bifrontally. The neurological examination was unremarkable. Magnetic resonance imaging revealed an extra-axial bilateral lesion in bilateral cerebellopontine angle, larger on left side. The lesions were homogeneously hypointenese on T1 -weighted imaging and hyperintense on T2- weighted imaging without evidence of contrast enhancement and without evidence of restriction on diffusion-weighted imaging. No surgical treatment was indicated. Conclusions. Bilateral arachnoid cysts of the cerebellopontine angle are very infrequent and the main indication for surgery is the existence of clinical symptoms or neurological deficit coincident with the locations of the cysts (AU)

Adhesive arachnoiditis after percutaneous fibrin glue treatment of a sacral meningeal cyst.

The authors present the case of a 64-year-old woman who was referred for severe sacral pain. She reported that her pain had been longstanding, and had greatly increased after percutaneous fibrin glue placement therapy for a sacral meningeal cyst 2 months earlier at a different hospital. An MRI scan obtained immediately after fibrin glue placement at that hospital suggested that fibrin glue had migrated superiorly into the subarachnoid space from the sacral cyst to the level of L-4. On admission to the authors' institution, physical examination demonstrated no abnormal findings except for perianal hypesthesia. An MRI study obtained at admission demonstrated a cystic lesion in the peridural space from the level of S-2 to S-4. Inhomogeneous intensity was identified in this region on T2-weighted images. Because the cauda equina and nerve roots appeared to be compressed by the lesion, total cyst excision was performed. The cyst cavity was filled with fluid that resembled CSF, plus gelatinous material. Histopathological examination revealed that the cyst wall was composed of hyaline connective tissue with some calcification. No nervous tissue or ganglion cells were found in the tissue. The gelatinous material was acellular, and appeared to be degenerated fibrin glue. Sacral pain persisted to some extent after surgery. The authors presumed that migrated fibrin glue caused the development of adhesive arachnoiditis. The risk of adhesive arachnoiditis should be considered when this therapy is planned. Communication between a cyst and the subarachnoid space should be confirmed to be sufficiently narrow to prevent the migration of injected fibrin glue.

Thoracolumbar spinal arachnoid diverticula in 5 pug dogs.

Clinical features, myelography, and computed tomography imaging findings as well as neurological outcome with and without surgery in 5 pug dogs with thoracolumbar arachnoid diverticula are described. Short-term prognosis after surgical therapy may not be as good as reported for other canine breeds, since immediate postsurgical deterioration is possible. Improvement of neurological deficits beyond the presurgical status may take several months.

Diverticules arachnoïdes spinales thoraco-lombaires chez 5 chiens Carlin. Les caractéristiques cliniques, la myélographie et l'imagerie par tomodensitométrie ainsi que les résultats neurologiques avec et sans chirurgie chez 5 chiens Carlin atteints de diverticules arachnoïdes thoraco-lombaires sont décrits. Le pronostic à court terme après la thérapie chirurgicale peut ne pas être aussi bon que signalé pour d'autres races canines, vu qu'une détérioration postchirurgicale immédiate est possible. L'amélioration des déficits neurologiques par rapport à l'état avant la chirurgie peut prendre plusieurs mois.(Traduit par Isabelle Vallières).

Homicide and subsequent catatonia associated with a large arachnoid cyst: case report.

The existence of a focal brain lesion that might be the crucial cause for the development of diverse psychiatric phenomena and certain characteristics of personality is often a controversial issue. The patient was a 29-year-old male when he killed his father with a single knock with the blunt side of an axe. Subsequently to the act, the patient developed a 10-month-long catatonic stupor during which he experienced intensive fear, delusions, and affective symptoms. He was an emotionally blunted person with no medical record and without prior history of aggressive behavior. Magnetic resonance image revealed a large, right-sided arachnoid cyst that was associated with right temporal and frontal lobe hypoplasia and bilateral changes of perfusion in peri-insular regions. The treatment with clozapine and diazepam showed to be therapeutic. This could be the second case of homicide committed by a person with arachnoid cyst and without past history of aggression, and the second description of an adult patient with cyst who developed catatonic stupor. This is the first description of long-lasting organic catatonic stupor treated with clozapine and diazepam. Relevant literature is reviewed and some controversial issues are discussed.

Multifocal intraparenchymal Langerhans' cell histiocytosis concomitant with an arachnoid cyst in a child: case report and review of the literature.

Langerhans' cell histiocytosis is a disease usually found in children and characterized by idiopathic proliferation of histiocytes in the reticuloendothelial system. Intracranial Langerhans' cell histiocytosis presenting as multifocal intraparenchymal lesions is very rare. In this article, the authors report on a 4-year-old boy diagnosed with multifocal intraparenchymal Langerhans' cell histiocytosis concomitant with an arachnoid cyst. After a series of laboratory examinations, the right frontal mass was surgically excised. Histological examinations confirmed the diagnosis of intracranial Langerhans' cell histiocytosis. The patient's intracranial hypertension symptoms were alleviated, and the remaining foci were treated by Langerhans' cell histiocytosis-directed standard chemotherapy. At the 8-month follow-up visit, no recurrence of the excised lesion was found, and no change in the size of other lesions was seen. Supratentorial intracerebral lesions with mass effect and enhancement have rarely been described; in this report, the histological features of and therapeutic options for such a case are discussed.

Intracranial arachnoid cysts in dogs.

Intracranial arachnoid cyst (IAC) is an infrequently reported developmental disorder seen primarily in small-breed dogs. It usually occurs in the caudal fossa, in the region of the quadrigeminal cistern. Although still considered uncommon, IAC is being recognized more frequently in veterinary medicine, coinciding with the increased availability of magnetic resonance imaging. In this article, clinical information from previously reported cases of canine IAC is combined with additional case information from our hospitals. Similar to IAC in people, it is thought that canine IAC is often an incidental finding. When IAC is responsible for neurologic disease in dogs, generalized seizures and cerebellovestibular dysfunction are the most common clinical presentations. Medical therapy of IAC focuses on management of increased intracranial pressure and seizures, if the latter are part of the clinical complaints. Surgical therapy of IAC involves either cyst fenestration or shunting the excess fluid to the peritoneal cavity

Spontaneous reduction of intracranial arachnoid cysts: a complete review.

The aetiopathology, clinical features and treatment of arachnoid cysts (AC) are still extremely controversial topics. The posterior fossa is the second most common site of these lesions, since they are often detected in the cerebellar or cerebellar-cistern region. Despite this, almost all the cases of non-surgical reduction of an AC reported in literature concern cysts of the middle fossa, whereas only two cases of spontaneous reduction of a posterior fossa AC has been described in literature. A complete review of the literature regarding this topic has been collected and discussed. The authors present a case of spontaneous reduction of an arachnoid cyst situated along the midline of the posterior cranial fossa with regression of all neurological symptoms. This 43-year-old man presented increasing nuchal headache with vomiting and nausea, simulating subarachnoid haemorrhage. CT and MRI documented an arachnoid cyst along the midline of the posterior fossa compressing the mesencephalon. The patient did not present any family history of this pathology nor had suffered head trauma. The patient was only given analgesics to relieve painful symptoms. After 30 days, spontaneous resolution of all the neurological symptoms took place. A 2 months, MRI control documented marked reduction of the cyst without any evident brain compression. The feasibility of a wait and see policy in such cases is discussed.

Coexistence of epileptic nocturnal wanderings and an arachnoid cyst.

Episodic nocturnal wanderings (ENWs) have rarely been associated with gross abnormalities of brain structures. We describe the case of a patient with ENWs in coexistence with an arachnoid cyst (AC). The patient was a 15-year-old boy who presented with nocturnal attacks characterized by complex motor behaviors. An MRI revealed a left temporal cyst and a SPECT Tc99 scan showed left temporal hypoperfusion and bilateral frontal hyperperfusion, more evident on the right side. During an all-night polysomnographic recording with audiovisual monitoring, dystonic posture followed by sleepwalking-like behavior was documented. The sleepwalking-like behavior was preceded by a spike discharge over the left frontocentral region with contralateral projection and secondary generalization during stage 2 sleep. Treatment with levetiracetam produced a striking remission of seizures. This supports a conservative management of an AC, considering that it may be an incidental finding. In epileptic patients, an AC may not necessarily be related to the location of the seizure focus.

Percutaneous fibrin glue therapy for meningeal cysts of the sacral spine with or without aspiration of the cerebrospinal fluid.

OBJECT: The authors assessed the efficacy of computed tomography (CT)-guided percutaneous injection of fibrin glue to treat meningeal cysts of the sacral spine in patients with back pain, and evaluated the necessity for cerebrospinal fluid (CSF) aspiration before glue injection. METHODS: Of the 31 patients in this study, 15 underwent injection of fibrin glue under CT guidance after aspiration of more than 15 ml of CSF (Group A), and 16 patients were treated with the glue but without CSF aspiration (Group B). Clinical results were evaluated after an average of 23 months of follow-up, and changes on the imaging studies were also evaluated. The clinical outcome and postoperative complications were analyzed. RESULTS: All 31 patients experienced resolution or marked improvement of symptoms for as long as 28 months after fibrin glue therapy. No patient experienced recurrence of symptoms during the follow-up interval. The postoperative pain relief was statistically significant (p < 0.001) according to evaluations in which a 100-mm visual analog pain scale was used. There were no statistical differences between the two groups (p > 0.05). CONCLUSIONS: Percutaneous CT-guided fibrin glue therapy for sacral arachnoid cysts may be a definitive therapy. It is unnecessary to aspirate the CSF before injection of the fibrin glue.

Acetazolamide and corticosteroid therapy in complicated arachnoid cyst.

CASE REPORT: We report on the successful conservative treatment of a child harbouring a right-temporal arachnoid cyst associated with bilateral hygroma. TREATMENT: Medical treatment was by corticosteroids for several days and acetazolamide (15 mg/kg/die) for 10 months. OUTCOME: Clinical symptoms and signs disappeared completely with partial resolution of the bilateral hygroma at MRI follow-up.

Management of leptomeningeal malignancy.

Leptomeningeal carcinomatosis is defined as malignant infiltration of the pia matter and arachnoid membrane. Leukaemias and lymphomas, lung, breast cancer and melanoma are the primary tumours commonly associated with leptomeningeal carcinomatosis. Diagnosis is based on compatible symptoms and signs, cytological evidence of malignancy in the cerebrospinal fluid, and neuroimaging studies. Treatment is largely palliative (median survival 2-4 months). Patients with lympomatous or leukaemic meningitis, chemosensitive tumours such as breast cancer, low tumour burden, minimal neurological deficits, good performance status and controllable systemic disease survive longer with occasional long-term responses. Available treatment options include focal radiation therapy to CNS sites of bulky, symptomatic or obstructive meningeal deposits, intrathecal cytotoxic therapy and systemic chemotherapy. No evidence of superiority of intrathecal treatment compared with best palliative care (including radiation therapy and systemic treatment) is available from clinical trials. Novel treatment approaches include intrathecal liposomal Ara-C, the development of new cytotoxic compounds, signal transduction inhibitors and monoclonal antibodies for intrathecal or systemic use. Until data from multi-centre randomised trials are available, rationalisation of therapy should be done by stratifying patients to prognostic groups. High-risk patients will only survive for a few weeks and are better managed with supportive measures, whereas low-risk patients justify vigorous cerebrospinal fluid-directed treatment combined with radiation therapy and systemic chemotherapy.

Arachnoid cyst with GnRH-dependent sexual precocity and growth hormone deficiency.

The coexistence of gonadotropin-releasing hormone (GnRH)-dependent sexual precocity and growth hormone deficiency in patients with arachnoid cysts is rarely reported, and its pathogenesis is not well recognized. This report describes an 11-year-old female who had a huge intracranial arachnoid cyst with initial symptoms and signs of sexual precocity. Her brain magnetic resonance imaging revealed distorted hypothalamus with a thin and stretched pituitary stalk. After treatment with cysto-peritoneal shunting and gonadotropin-releasing hormone analogue, her puberty was arrested and subnormal growth rate was observed. Catch-up growth was detected after growth hormone therapy. Hence, coexistence of gonadotropin-releasing hormone-dependent sexual precocity and growth hormone deficiency in this patient was confirmed.