scholarly journals Gasperini Syndrome: A Case Report and Systematic Review and Proposing a New Definition

2020 ◽  
Vol 12 (3) ◽  
pp. 433-439
Author(s):  
Riwaj Bhagat ◽  
Siddharth Narayanan ◽  
Marwa Elnazeir ◽  
Thong Diep Pham ◽  
Robert Paul Friedland ◽  
...  

Gasperini syndrome (GS), a rare brainstem syndrome, is featured by ipsilateral cranial nerves (CN) V–VIII dysfunction with contralateral hemibody hypoesthesia. While there have been 18 reported cases, the GS definition remains ambiguous. We report a new case and reviewed the clinical features of this syndrome from all published reports to propose a new definition. A 57-year-old man with acute brainstem stroke had right CN V–VIII and XII palsies, left body hypoesthesia and ataxia. Brain MRI showed an acute stroke in the right caudal pons and bilateral cerebellum. After a systematic review, we classified the clinical manifestations into core and associate features based on the frequencies of occurring neurological deficits. We propose that a definitive GS requires the presence of ipsilateral CN VI and VII palsies, plus one or more of the other three core features (ipsilateral CN V, VIII palsies and contralateral hemibody hemihypalgesia). Additionally, GS, similar to Wallenberg’s syndrome, represents a spectrum that can have other associated neurological features. The revised definition presented in this study may enlighten physicians with the immediate recognition of the syndrome and help improve clinical localization of the lesions and its management.

2014 ◽  
Vol 21 (4) ◽  
pp. 471-476
Author(s):  
Danil Adam ◽  
Toma Papacocea ◽  
Ioana Hornea ◽  
Cristiana Moisescu

Abstract Meningioma is in most cases a benign tumor of the central nervous system with two growth patterns: en masse and en plaque. Hyperostosis is associated in 13 - 49 % of the cases with en plaque meningioma. We describe the case of a 47 years old woman with meningotelial sphenoid ridge meningioma which was totally removed. At the first admission she presented with no neurological deficits, seizures and a mild right exophthalmos. This had an indolent growth. After 10 years, the patient was readmitted for headache, blurred vision and right exophthalmos. Skull X-rays and brain MRI revealed an important thickening of the right superior orbit wall and sphenoid ridge. She underwent a new surgery. There was no intradural tumor found. Instead, bones of the superior and lateral right orbit walls were very hiperostotic. A hole of 3/2 cm in the right superior orbital wall was drilled and the orbital cavity was decompressed. In the postoperative period, the symptoms were remitted and the exophthalmos reduced. We discuss the causes and management of hyperostosis associated with meningiomas.


2019 ◽  
Vol 80 (S 03) ◽  
pp. S310-S310
Author(s):  
Frederick Luke Hitti ◽  
John Y.K. Lee

A variety of lesions may arise within the cerebellopontine angle (CPA). Schwannomas and meningiomas are most commonly found in this location. Imaging characteristics of meningiomas include hyperdensity on head computed tomography (CT) and avid contrast enhancement on T1-weighted postcontrast magnetic resonance imaging (MRI). Here, we present the case of a 49-year-old woman with enlarging right CPA meningioma. The patient reported mild hearing loss on the right but her neurological exam was otherwise benign. Since the lesion was enlarging and symptomatic, the patient was offered resection of the mass for diagnosis and treatment via an endoscopic retrosigmoid approach. We provide a video that illustrates the steps taken to resect this mass endoscopically. After cerebrospinal fluid (CSF) was drained to achieve brain relaxation, the tumor was visualized. The tumor had a rich vascular supply and had the appearance of a typical meningioma. The bipolar was used to cauterize the tumor's vascular supply. The tumor capsule was then opened with the microscissors. The round knife, suction, and ultrasonic tissue debrider were used to debulk the tumor. After internal debulking of the tumor, the capsule was dissected off the cerebellum and mobilized. A combination of blunt and sharp dissection was done to free the tumor capsule from the adjacent structures. Inferiorly, the lower cranial nerves were visualized. Tissue pathology confirmed a diagnosis of grade I meningioma. A gross total resection was achieved and the patient remained neurologically stable, postoperatively. Furthermore, T1-weighted postcontrast brain MRI, 1 year after surgery, showed no residual.The link to the video can be found at: https://youtu.be/X9c_inLp-So.


2020 ◽  
Vol 101 (2) ◽  
pp. 121-125 ◽  
Author(s):  
A. V. Titov ◽  
O. E. Mozgovaya

The artery of Percheron originates from the right or left posterior cerebral artery and is one of the options for normal blood supply to the paramedian portions of the thalamus. Stroke in this area has specific MRI changes, the most informative ones in diffusion-weighted imaging mode. With the involvement of the midbrain, its pial surface can display an additional pathognomonic V-shaped sign in 67%. The clinical manifestations are non-specific. In some cases, there is a triad of symptoms, which is distinguished into paramedian thalamic syndrome (vertical gaze palsy, neuropsychological disorders, and depressed level of consciousness up to coma). The symptoms of a midbrain lesion as oculomotor nerve group diseases, contralateral hemiparesis, hemiataxia or hemianesthesia, in combination with a triad, develop a thalamopeduncular or mesencephalon thalamic syndrome. The paper presents a clinical case of a 59-year-old man who was hospitalized with acute unconsciousness, a Glasgow coma scale of 13 scores, and disorientation. A neurological examination revealed vertical gaze palsy and upper limb ataxia. Brain MRI revealed characteristic bithalamic paramedian stroke. After completing the treatment, the patient was discharged showing incomplete clinical recovery.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Adeyinka Taiwo ◽  
Marcelo Correia ◽  
Nicole Peyton

Abstract BACKGROUND: Crooke’s cell adenoma is a rare variant of ACTH-secreting corticotroph adenomas (<1%). These are aggressive macroadenomas and usually invade the cavernous sinus producing visual symptoms. Clinical Case: A 48-year old male presented with acute right eye and right temple pain, and diplopia that started 6 days prior. Past medical history included hypertension, type 2 diabetes, hyperlipidemia, traumatic brain injury, CSF leak with bacterial meningitis s/p sphenoid repair and palsy of the right III, V and VI cranial nerves. Head CT showed a 1.9 x 2.1 x 1.5 cm soft tissue-density mass medial to the right cavernous carotid artery extending into the sphenoid sinus. There was expansile bony change to the floor and right dorsum of the sella. The mass showed mild contrast enhancement and abutted the superior orbital fissure. A brain MRI was not obtained due to history of a left cochlear implant. He did not have any cushingoid features on exam. Neuro-ophthalmology exam revealed, severe sixth cranial nerve palsy, bilateral optic disc pallor and a mild right superotemporal visual field defect. Pre-operative pituitary function tests revealed ACTH 103 pg/ml (7–63), cortisol 14 ug/dl (6.0–18.4), FSH 2.7 mIU/ml (1.5–12.4), LH 3.8 mIU/ml (1.7–8.6), prolactin 17.6 ng/ml (4–15.2), total testosterone 175 ng/dl (249–836), TSH 1.10 uIU/ml (0.27–4.20), free T4 0.67ng/dl (0.8–1.8) and IGF-1 208 ng/ml (69–224). He underwent successful endoscopic trans-sphenoidal resection of a 4.2 x 2.5 x 0.5 cm mass. Pathology revealed hyaline deposits in the cytoplasm and perinuclear cytokeratin in more than 50% of the adrenocorticotroph cells consistent with Crooke’s cell adenoma. Post-operatively, his right eye pain and abduction deficit improved, and diplopia returned to baseline. He was placed on a short hydrocortisone taper. Due to high risk of recurrence, he received 54Gy in 30 fractions to the sella. Six and 12-month follow-up head CTs revealed stable residual enhancing tissue. Pituitary function tests done 9 months post-surgery were normal and no hormone replacement was needed. He was clinically stable 12 months after surgery. Conclusion: We described a rare case of invasive Crooke’s cell pituitary adenoma with visual disturbances that was managed with resection and radiotherapy. The patient remained free of recurrence 1 year later. However, lifetime surveillance is necessary due to high incidence of recurrence. Reference: 1. Crooke’s Cell Tumors of the Pituitary. Neurosurgery, Volume 76, Issue 5, May 2015, Pages 616–22


2020 ◽  
Vol 2 (2) ◽  
pp. V13
Author(s):  
Satoshi Kiyofuji ◽  
Masahiro Shin ◽  
Kenji Kondo ◽  
Tsukasa Koike ◽  
Taichi Kin ◽  
...  

Cerebellopontine (CP) angle tumors are often resected via retrosigmoid craniotomy; however, sometimes cranial nerves (CNs) make their resection more complex. In such cases, the endoscopic transnasal approach can avoid such manipulations as delivering surgical instruments over CNs or peeling off CNs from the tumor, minimizing the risk of postoperative deficits. A 35-year-old man presented with a 37-mm cystic tumor in the right CP angle, and preoperative 3D fusion images revealed that multiple CNs (VII, VIII, and lower CNs) were running on the tumor posteriorly. The endoscopic transnasal approach enabled safe subtotal resection without causing neurological deficits, and the patient underwent stereotactic radiosurgery for the residual schwannoma.The video can be found here: https://youtu.be/xKLwdDsLpWA.


Author(s):  
Beatrice Khater ◽  
Vicky Kassouf ◽  
Georges Haddad ◽  
Roula Hourany

Obstructive sleep apnoea (OSA) is a common condition usually treated with continuous positive airway pressure (CPAP). No reports have linked it to an acute subdural haematoma. A 54-year-old white man who had hypertension well controlled with an angiotensin II receptor blocker, presented with a 2-week history of occipital headache with no other focal neurological symptoms. The headache began 12 days after he had started using CPAP for OSA. A brain MRI performed 2 weeks later showed bilateral subdural haematomas which were chronic on the left and sub-acute/acute on the right. Since the patient was clinically stable with no focal neurological deficits, he received prednisone for 3 weeks and was followed up with consecutive CT scans demonstrating gradual regression of the haematomas. This is the first report showing that subdural haematomas could be linked to CPAP use.


2021 ◽  
Vol 12 ◽  
Author(s):  
Bing-Yan Ren ◽  
Yi Guo ◽  
Jing Han ◽  
Qian Wang ◽  
Zai-Wang Li

Introduction: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, a serious neurological autoimmune disorder caused by autoantibodies with diverse clinical manifestations, may simultaneously onset with antimyelin oligodendrocyte glycoprotein (MOG) demyelination after recurrent central nervous system (CNS) demyelination.Case Report: We present a case of anti-NMDAR encephalitis combining with anti-MOG CNS demyelination following recurrent CNS demyelination. A 38-year-old man admitted to hospital developed epileptic seizures following recurrent episodes of cross-sensory disturbance and dizziness. Magnetic resonance imaging (MRI) showed a demyelinating lesion in the right brainstem initially. Despite a good response to methylprednisolone pulse therapy at the beginning, the patient still had relapses and progression after corticosteroid reduction or withdrawal. Then brain MRI discovered new serpentine lesions involving extensive cerebral cortex on his second relapse. Repeat autoantibodies test indicated cerebrospinal fluid (CSF) NMDAR antibodies coexisted with MOG-Abs simultaneously, suggesting the diagnosis of anti-NMDAR encephalitis with anti-MOG CNS demyelination.Results: After a definite diagnosis, the patient was treated with mycophenolate mofetil (MMF) and corticosteroid. He was discharged after his symptoms ameliorated. No neurological sequels remained, and there were no effects on his activities of daily living after 6 months of immunoregulatory therapy of MMF and corticosteroid.Conclusion: For individuals with recurrent CNS demyelination, especially combining with cortical encephalitis, repeated detection of autoantibodies against AE, and demyelination in CSF/serum can be helpful to enable a definite early diagnosis. For patients who suffer from anti-NMDAR encephalitis combining with anti-MOG CNS demyelination, second-line immunotherapy is recommended when first-line treatment such as steroids, intravenous immunoglobulin G (IVIG) and plasma exchange has been proven ineffective to prevent the relapse of disease.


2021 ◽  
Vol 12 ◽  
pp. 563
Author(s):  
Mohammad Samadian ◽  
Seyed Farzad Maroufi ◽  
Mehrdad Hosseinzadeh Bakhtevari ◽  
Hamid Borghei-Razavi

Background: Isolated cavernous malformation (CM) of the abducens nerve has not been reported in the literature. Herein, the authors address the clinical importance of these lesions and review the reported cases of CM from 2014 to 2020. Case Description: A 21-year-old man presented with binocular diplopia and headache from 2 months before his admission. The neurological examination revealed right-sided abducens nerve palsy. The brain MRI revealed an extra-axial pontomedullary lesion suggestive of a CM. The lesion was surgically removed. During the operation, the abducens nerve was resected considering the lesion could not be separated from the nerve and an anastomosis was performed using an interposition nerve graft and fibrin glue. Pathological examination of the resected lesion revealed that it was originated from within the nerve. The patient’s condition improved in postoperative follow-ups. Conclusion: Surgical resection of the cranial nerves CMs is appropriate when progressive neurological deficits are present. If the lesion is originated from within the nerve, we suggest resection of the involved nerve and performing anastomosis. Novel MRI sequences might help surgeons to be prepared for such cases and fibrin glue can serve as an appropriate tool to perform anastomosis when end-to-end sutures are impossible to perform.


2022 ◽  
Vol 15 ◽  
Author(s):  
Hang Shu ◽  
Manqiu Ding ◽  
Pei Shang ◽  
Jia Song ◽  
Yue Lang ◽  
...  

Myelin oligodendrocyte glycoprotein antibody-associated disease is an immune-mediated demyelinating disease of the central nervous system that is present in both adults and children. The most common clinical manifestations are optic neuritis, myelitis, acute disseminated encephalomyelitis, and brainstem syndrome. Cerebral cortical encephalitis (CCE) is a rare clinical phenotype of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), which usually begins with seizures, headaches, and fever, and may be misdiagnosed as viral encephalitis in the early stages. Herein, we report two typical MOG antibody (MOG-Ab)-positive patients presenting with CCE, both of whom presented with headache, fever, seizures, and who recovered completely after immunotherapy. In addition, we performed a systematic review of the present literature from the perspectives of population characteristics, clinical symptoms, MRI abnormalities, treatments, and prognosis. Among the patients reported in 25 articles, 33 met our inclusion criteria, with the age of onset ranging from 4 to 52 years. Most of the patients had seizures, headache, fever, and unilateral cortical lesions on brain MRI. For acute CCE, 30 patients were treated with high-dose intravenous methylprednisolone, and the symptoms of most patients were completely relieved after immunotherapy. This study reported our experience and lessons learned in the diagnosis and treatment of MOG-Ab-positive CCE and provides a systematic review of the literature to analyse this rare clinical phenotype.


2015 ◽  
Vol 2015 ◽  
pp. 1-2 ◽  
Author(s):  
Demet Menekse Gerede ◽  
Irem Muge Akbulut ◽  
Sadık Ersoz ◽  
Mustafa Kilıckap

Myxomas are benign and the most common tumors of the cardiac muscle (Reynen, 1995). They are predominantly located in the left atrium. Clinical manifestations may vary according to the localization and the size of the myxoma. On the other hand, imaging of a myxoma by contrast dye during coronary angiography is a rare sign, which displays the vascular supply of the tumor. Here, we report the case of a 51-year-old man presenting with presyncope and palpitations due to a giant left atrial myxoma having its vascular supply from the right coronary artery (RCA).


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