scholarly journals A Case of Neurosarcoidosis with Labyrinthine Involvement

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Peter B. Johnson ◽  
Roxanne Melbourne-Chambers ◽  
Amit Manohar Saindane ◽  
Nilesh Desai ◽  
Myrton Smith

Sarcoidosis is a chronic granulomatous disease of unknown aetiology, which may involve any organ system. It most commonly occurs in adults with childhood involvement being rare. Central nervous system involvement is seen in up to 25% and typically involves meningeal disease resulting in multiple cranial neuropathies. Other common clinical findings include seizures, headache, dementia, and pituitary dysfunction. Imaging plays a central role in the diagnosis with typical findings including pachymeningeal and leptomeningeal enhancing lesions. Other imaging findings include lacunar and major territory infarcts, hypothalamic and infundibular thickening, hydrocephalus, and cranial nerve enhancement. We present a case of an eight-year-old male patient with progressive headache, visual disturbance, unilateral sensory hearing loss, and multiple cranial neuropathies. Imaging findings demonstrated the classic pachymeningeal and leptomeningeal enhancement along much of the skull base, as well as enhancement of the right and left second and eighth cranial nerves. Extensive inflammatory changes were noted in the temporal bones and paranasal sinuses. There was also enhancement of the right and left labyrinths. Sinus biopsy confirmed sarcoidosis. We present the first case to our knowledge of sarcoid labyrinthitis.

2020 ◽  
Vol 11 (2) ◽  
pp. 315-321
Author(s):  
Renata García Franco ◽  
Alejandro Arias Gómez ◽  
Juvenal Guzman Cerda ◽  
Marlon García Roa ◽  
Paulina Ramirez Neria

Appropriate medical management can be an alternative in those patients with submacular cysticercosis in whom achieving good visual outcome with vitreoretinal surgery is not possible. We report the case of a 25-year-old female who presented complaining of blurred vision in her left eye associated with photopsias and metamorphopsias of 3 months duration. Initial visual acuity in the right eye was 20/20 and 20/100 in the left eye. Upon indirect ophthalmoscopy in the left eye, a yellow-white, dome-shaped, elevated lesion with foveal involvement was observed. The rest of the ophthalmological examination proved normal. With clinical findings and images, submacular cysticercosis was diagnosed, and vitreoretinal surgery was suggested. Nevertheless, the patient did not accept the treatment; therefore, medical management was initiated. Central nervous system involvement was ruled out, and treatment with praziquantel and systemic prednisolone was initiated. Cysticercosis was resolved with significant improvement of her symptoms and visual acuity.


2021 ◽  
Vol 2 (6) ◽  
Author(s):  
Renato J. Galzio ◽  
Mattia Del Maestro ◽  
Diamantoula Pagkou ◽  
Massimo Caulo ◽  
Sofia Asioli ◽  
...  

BACKGROUND The authors reported the first documented case of intracranial extraaxial nonneurofibromatosis type 1–related nontriton malignant peripheral nerve sheath tumor (MPNST) originating from the falx cerebri. OBSERVATIONS A 34-year-old man with headache, short-term memory deficit, postural instability, and blurred vision presented with a large heterogenous contrast-enhanced intraventricular cystic lesion originating from the free margin of the falx cerebri. The patient received surgery using the right posterior interhemispheric approach. Gross total resection was performed, and the inferior border of the falx cerebri was resected. The postoperative course was uneventful. Histological examination revealed hypercellular foci of neoplastic spindle cells with hyperchromatic and wavy nuclei. Hence, a diagnosis of MPNST was made based on concomitant immunochemistry findings, including mouse double minute 2 homolog focal positivity and geographic loss of H3K27me3. The patient received adjuvant radiotherapy, and recurrence was not observed. LESSONS Intracranial MPNSTs are extremely rare tumors, typically originating from the cranial nerves in the posterior cranial fossa. An even rarer variant of these tumors, referred to as malignant intracerebral nerve sheath tumors, may directly arise from the brain parenchyma. The authors reported the first case of an intracranial MPNST originating from the dura mater of the falx cerebri, acting as an extraaxial lesion with prevalent expansion in the right ventricle.


2011 ◽  
Vol 145 (6) ◽  
pp. 992-998 ◽  
Author(s):  
Nilesh K. Desai ◽  
Lindsay Young ◽  
Mario A. Miranda ◽  
Joe Walter Kutz ◽  
Peter S. Roland ◽  
...  

Objectives. Pontine tegmental cap dysplasia (PTCD) is a rare congenital malformation. Clinical and imaging findings in 3 patients and the authors’ experience with bilateral cochlear implantation in 1 patient are described. Study Design. Retrospective review. Setting. Two tertiary medical centers. Subjects and Methods. Three patients were evaluated by an otolaryngologist and underwent magnetic resonance imaging (MRI) of the temporal bones and brain. High-resolution computed tomography (CT) scanning of the temporal bones was performed in 2 patients. Imaging findings of the brain, the presence and course of resolvable cranial nerves, the membranous labyrinth, and internal auditory canals were reviewed. Clinical data were reviewed. Results. All patients demonstrated typical brain characteristics of PTCD. Mild, bilateral cochlear dysplasia was noted in 2, and all had a normal vestibular labyrinth. The cochleovestibular nerves were universally absent bilaterally. The facial nerves were subjectively deficient bilaterally in 1 patient, unilaterally in the second patient, and normal in the third. An accessory canal for the seventh cranial nerve, referred to as a duplicated internal auditory canal, was present in all patients. Auditory brainstem response testing revealed profound bilateral sensorineural hearing loss in all of the patients; none suffered facial weakness. A single patient underwent bilateral cochlear implantation with only minimal response. Conclusion. The authors report 3 cases of PTCD with emphasis on imaging of the seventh and eighth cranial nerves and clinical neurotologic findings. All patients manifested duplicated internal auditory canals, a previously unreported finding in PTCD. Bilateral profound sensorineural hearing loss is due to absence of the cochleovestibular nerve. Prognosis for cochlear implantation is poor.


Lupus ◽  
2021 ◽  
pp. 096120332098401
Author(s):  
Esra Baglan ◽  
Semanur Ozdel ◽  
Mutlu Uysal Yazıcı ◽  
Ebru Azapağası ◽  
Halil Çelik ◽  
...  

Pediatric-onset systemic lupus erythematosus is among the prototypic systemic autoimmune diseases seen in children. Although the neuropsychiatric involvement rate varies during the course of the disease, it is an important cause of morbidity and mortality. The clinical picture of neuropsychiatric SLE (NPSLE) is highly variable, and neurological features can precede systemic findings, leading to some diagnostic difficulties. NPSLE requires early and aggressive immunosuppressive therapy. Some patients can be resistant to immunosuppressive therapy. Chorea is a rare manifestation that occurs in 1.2%–2% of SLE patients and can result from an immunologically mediated mechanism, antiphospholipid autoantibodies or ischemia. Herein we present the first case of pediatric-onset SLE diagnosed with central nervous system involvement and treated with Zipper method. The Zipper method is a new immunomodulation treatment. The clinical findings of the patient, which were resistant to corticosteroids and cyclophosphamide, resolved by this novel treatment.


2020 ◽  
Vol 11 ◽  
pp. 282
Author(s):  
Shodai Yamada ◽  
Kenji Yagi ◽  
Kazuhiro Hirano ◽  
Masaaki Uno

Background: In patients with secondary empty sella syndrome (ESS), optic nerve herniation into the sella turcica is caused by shrinkage of the mass lesion at the sella turcica, resulting in visual disturbance. ESS is often surgically treated using chiasmapexy. Here, we report the first case of spontaneous improvement in a patient with ESS. Case Description: A 69-year-old woman presented with a month-long history of visual disturbance in the right eye, poor visual acuity, and quadrantanopia in her upper temporal visual field. Magnetic resonance (MR) imaging showed herniation of her right optic nerve and gyrus rectus into the sella turcica. The visual disturbance gradually improved, and the patient’s vision became almost normal after a month without any treatment. On repeated MR imaging, it was observed that the herniation of the right optic nerve and gyrus rectus disappeared due to an intrasellar cyst re-expansion. The secondary ESS caused by the shrinkage of the intrasellar cyst resulted in the visual disturbance and re-expansion of the cyst resulted in spontaneous improvement of symptoms. The visual disturbance did not recur for a year. Conclusion: Patients with secondary ESS without severe symptoms may be followed up conservatively. However, surgical treatment should be applied if symptoms deteriorate or do not improve.


Author(s):  
Shalini Mahana Valecha ◽  
Dolly Bashani ◽  
Jaya Gedam ◽  
Pandeeswari .

The first case of primary ovarian ectopic pregnancy was reported by St. Maurice in 1689. Primary ovarian ectopic pregnancy is rare entity, with incidence of around 3% of all ectopic pregnancies. The aim of present case report is to study the role of different modalities like clinical findings, biochemistry, sonography, surgery and histopathology in diagnosis of primary ovarian ectopic pregnancy. Authors present a case of a 24 years old female with history of 2 months amenorrhoea, per vaginal spotting and mild intermittent abdominal pain in RIF. Examination was unremarkable and serum βHCG was 2007.5IU/ml. An ultrasound scan showed an ectopic mass in the right adnexa suggestive of a tubal ectopic pregnancy and she underwent surgical management at our institute.  At laparoscopy, both fallopian tubes were noted to be normal with an ectopic mass attached to the right ovary with a pedicle. With these unusual laparoscopic findings, possibilities of tubal miscarriage, ovarian pregnancy, or abdominal pregnancy were suspected. Ectopic mass was coagulated and cut through the pedicle with bipolar cautery and specimen was sent for HPE. Our patient made an uneventful recovery and no further medical management was indicated. The diagnosis of right ovarian ectopic pregnancy was finally confirmed by histopathological evidence of the presence of chorionic villi in a background of ovarian stromal tissue, in consideration with Spigelberg’s criteria. Present case highlights the importance of considering non-tubal ectopic pregnancies when making a diagnosis based on ultrasound scan. Pre-operative diagnosis of ovarian ectopic pregnancy still remains a challenge in spite of current medical advances. Despite the benefits and reliability of ultrasound scanning, there will still be situations where the definitive diagnosis can only be made at surgery. However, histopathological examination is confirmatory and always mandatory.


Author(s):  
Cigdem Ozer Gokaslan ◽  
Ugur Toprak ◽  
Emin Demirel ◽  
Cagri Erdim ◽  
Aytul Hande Yardimci ◽  
...  

Background: Schwannomas are benign slow-growing tumors most often associated with the cranial nerves. Schwannomas often originate from the eighth cranial nerve. They may also originate from the peripheral nervous system of the neck and extremities. However extracranial peripheral schwannomas are considered a rare entity. Objective: The knowledge of rare localizations and typical imaging findings will lead to a successfulradiological diagnosis. Therefore, in this study, we present the clinical findings and MRI characteristics of schwannomas with a rare localization involving the peripheral, lower and upper extremity and intramuscular regions. Materials and Methods: The hospital database was screened for patients with an extracranial soft tissue mass. Twenty-one cases of schwannomas were found in rare localization. We analyzed the MR images of these patients retrospectively. The MR images were evaluated in terms of tumor location, signal intensity, and enhancement pattern. The histological examination of all the patients confirmed the diagnosis of schwannoma. Results: In 21 patients, the schwannomas were peripheral, localized to upper (n = 6) and lower extremities (n = 11). The remaining four patients had intramuscular schwannomas. : The patients diagnosed with intramuscular schwannomas had schwannomas in sternocleidomastoid, gastrocnemius, triceps muscle and lateral wall of the abdomen. The average long-axis diameter of the tumor was 27.7 mm and the average short-axis diameter was 16.4 mm. The contrast pattern was diffused in eight tumors and peripheral in 13. Conclusion: In this study, we present clinical findings and MRI characteristics of schwannomas with a rare localization involving the peripheral, lower and upper extremity and intramuscular regions.


2020 ◽  
Vol 66 (5) ◽  
pp. 549-552
Author(s):  
Zafer Bağcı

Abstract Introduction Tetanus is a preventable infectious disease with vaccination. Cephalic tetanus is the rarest form in which local tetanus can involve the cranial nerves. Case Herein, we report a case of cephalic tetanus in a 16-month-old girl who had never been vaccinated. The patient, who had a complaint of a wound on the cheek mucosa for 2 weeks, was seen playing with the soil in the garden 1 week ago and was found to have abundant soil removed by her mother. The patient was diagnosed as cephalic tetanus according to her complaints and clinical findings. Discussion We believe that, this is the first case reported in the literature of cephalic tetanus in such a young child wherein the disease focus of infection from a wound on the cheek mucosa. The symptom we defined as ‘a child who cries when she smiles’ presented in this case could only be associated with this disease. Conclusion In addition to detailed anamnesis and meticulous physical examination, the clinical symptoms that we have described for the first time in a child with cephalic tetanus should also be considered for early and accurate diagnosis.


2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Nikita Chapurin ◽  
Cynthia Wang ◽  
David M. Steinberg ◽  
David W. Jang

Objective. We aim to describe the first case in the literature of allergic fungal sinusitis (AFS) presenting with hyperprolactinemia due to compression of the pituitary gland.Case Presentation. A 37-year-old female presented with bilateral galactorrhea and occipital headaches of several weeks. Workup revealed elevated prolactin of 94.4, negative pregnancy test, and normal thyroid function. MRI and CT demonstrated a 5.0 × 2.7 × 2.5 cm heterogeneous expansile mass in the right sphenoid sinus with no pituitary adenoma as originally suspected. Patient was placed on cabergoline for symptomatic control until definitive treatment.Results. The patient underwent right endoscopic sphenoidotomy, which revealed nasal polyps and fungal debris in the sphenoid sinus, consistent with AFS. There was bony erosion of the sella and clivus. Pathology and microbiology were consistent with allergic fungal sinusitis caused byCurvulariaspecies. Prolactin levels normalized four weeks after surgery with resolution of symptoms.Conclusion. Functional endoscopic sinus surgery alone was able to reverse the patient’s pituitary dysfunction. To our knowledge, this is the first case of AFS presenting as hyperprolactinemia due to pituitary compression.


2020 ◽  
Vol 11 ◽  
pp. 35
Author(s):  
Nicollas Rabelo ◽  
Vinicius Trindade Gomes da Silva ◽  
Marcelo Prudente do Espírito Santo ◽  
Davi Solla ◽  
Dan Zimelewicz Oberman ◽  
...  

Background: Ossifying fibroma (OF) is benign bone lesions, most frequent in young children, more common in the maxillary sinus and mandible (75–89%), the pathogenesis of the tumor is not clear, there are many subtypes of OF. This paper aims to report an OF a case and literature review. Case Description: Male, 19 years old, with a progressive history proptosis since 2012, diagnosed as a right supraorbital lesion at an external service and assigned to conservative management. Then, he evolved with double vision, which worsened in February of 2018, associated with a moderate headache. On admission: proptosis and downward deviation of the right orbit was noticed on the physical exam and with exception of limited right upgaze, external ocular movements were maintained. Head computed tomography showed a multiloculate expansive osteolytic lesion at the right orbital roof. On magnetic resonance imaging, the lesion had an inner content with septations, T1-weighted imaging heterogeneous signal, T2-weighted imaging high signal intensity, and peripheral contrast enhancement. The patient underwent a right frontal craniotomy with a gross total resection and the postoperative follow-up was uneventful. Menzel reported the first case in 1782. The clinical findings depend on localization. There are five subtypes. In general, the lesions have a radiological appearance with hyperdense boundary and cause deformity and destruction in bones with high recurrence risk. Radical resection is curative. Conclusion: As a result, the correlation of clinical, radiologic, and pathologic data is significant while going for a specific diagnosis in cases of craniofacial fibrous lesions. Total excision is the best treatment, but it can recur.


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