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ENeurologicalSci Jun 2024Waldenstroms macroglobulinemia (WM) is a low-grade B cell neoplasm. Bing Neel syndrome is a rare manifestation of WM characterized by infiltrative involvement of the...
BACKGROUND AND AIMS
Waldenstroms macroglobulinemia (WM) is a low-grade B cell neoplasm. Bing Neel syndrome is a rare manifestation of WM characterized by infiltrative involvement of the central nervous system.
CASE REPORT
64-year-old man, presented with 4 years history of slowly progressive diplopia and ptosis of eyes. Examination showed left oculomotor (internal and external ophthalmoplegia), with trochlear, abducens, and right partial oculomotor and abducens nerve involvement. Evaluation showed anemia of hemoglobin 10.7 g/dL, raised erythrocyte sedimentation rate of 120 mm/h and plasma albumin:globulin reversal. Serum protein electrophoresis showed a paraprotein peak in the early gamma region with elevated IgM level (3810 mg/dL) and elevated free kappa light chain level (70.1 mg/L). Bone marrow aspiration from posterior iliac crest revealed mature small lymphocytes with positive immunohistochemical markers of CD5, CD10 negativity and MYD88 mutation positivity suggestive of WM. Patient was treated with bendamustine and rituximab regimen, with no neurological improvement at the end of one year.
CONCLUSION
This case expands spectrum of paraproteinemic neuropathy to include cranial nerve palsy. Thus, plasma cell dyscrasias have to be considered in patients with isolated ophthalmoparesis especially in elderly patients, even with other comorbidities such as diabetes mellitus.
PubMed: 38784860
DOI: 10.1016/j.ensci.2024.100505 -
Diabetes Therapy : Research, Treatment... May 2024Glucagon-like peptide-1 receptor agonists (GLP-1RAs) have attracted much attention because of their significant hypoglycemic and weight-loss effects. Previous...
INTRODUCTION
Glucagon-like peptide-1 receptor agonists (GLP-1RAs) have attracted much attention because of their significant hypoglycemic and weight-loss effects. Previous preparations can only be subcutaneously injected. Oral administration of GLP-1RAs semaglutide helps to broaden treatment options, but its safety in the real world still needs to be observed. This study is based on FDA adverse event reporting system (FAERS) database to mine adverse drug events (ADE) of oral semaglutide, and provide references for the clinical safe use of this drug.
METHODS
To analyze the signal quality of oral semaglutide, which is a drug used in the FAERS database from the third quarter of 2019 to the third quarter of 2023, we collected ADE data and performed data mining by using disproportionate analysis. Then, we standardized the data and used a variety of signal-quantification techniques, including reported odds ratio (ROR), proportional reporting ratio (PRR), Bayesian belief propagation neural network (BCPNN), and multiple empirical Bayesian gamma Poisson contractions (MGPS), for further analysis.
RESULTS
We screened 2398 reports on the use of semaglutide tablets, involving a total of 5653 ADE. These reports were mainly submitted by consumers, and the reporting country was mainly the United States. A total of 23 system organ classes (SOC) and 93 preferred terms (PT) were mined for the signals of semaglutide tablets. The three most common SOC were gastrointestinal disorders, general disorders and administration site conditions, and investigations. At the PT level, metabolism and nutrition disorders exhibit the highest number of signals, with the top three being thyroid cyst, acute cholecystitis, and ketosis. Gastrointestinal disorders rank second, primarily involving eructation, pancreatitis, impaired gastric emptying, and regurgitation. In addition, vith nerve paralysis occurs and the signal intensity is high.
CONCLUSIONS
Our study provides a deeper and broader understanding of the safety of oral semaglutide. The results of the ROR, PRR, BCPNN, and MGPS algorithms exhibit high consistency, with metabolism and nutrition-related disorders having the highest number of signals. The conclusions align with the technical specifications of the product. Notably, other unexpected effects are reported, including acute cholecystitis, paralysis of the abducens nerve, and positional vertigo.
PubMed: 38776037
DOI: 10.1007/s13300-024-01594-7 -
Neurology Jun 2024
Topics: Humans; Synkinesis; Pons; Male; Demyelinating Diseases; Magnetic Resonance Imaging; Female; Abducens Nerve Diseases; Middle Aged
PubMed: 38771991
DOI: 10.1212/WNL.0000000000209542 -
Brain Tumor Research and Treatment Apr 2024Giant cell tumors (GCTs) are locally aggressive primary bone tumors of osteoclast-like cells. Most GCTs occur within the long bones, and primary GCTs involving the...
Giant cell tumors (GCTs) are locally aggressive primary bone tumors of osteoclast-like cells. Most GCTs occur within the long bones, and primary GCTs involving the clivus are extremely rare. We present the case of an 18-year-old boy with binocular horizontal diplopia with an insidious onset who was found to have a hypointense enhancing mass involving the clivus and left side dorsum sellae on magnetic resonance images. The tumor was completely resected via an endoscopic endonasal transclival approach, and histopathologic examination via immunohistochemistry indicated a GCT. The patient's left abducens nerve palsy improved slightly after surgery. Because of the rarity of GCTs, there is no consensus about the definitive treatment protocol. However, we suggest that gross total resection is the treatment of choice, and denosumab plays a critical role in patients with subtotal resection.
PubMed: 38742263
DOI: 10.14791/btrt.2024.0010 -
Endoscopic transsphenoidal resection of parasellar abducens nerve schwannoma: A video demonstration.Surgical Neurology International 2024The abducens nerve schwannoma (ANS) in the sellar and parasellar region are extremely rare. Only around two dozen of ANS have been described in the world literature....
BACKGROUND
The abducens nerve schwannoma (ANS) in the sellar and parasellar region are extremely rare. Only around two dozen of ANS have been described in the world literature. These cases were, however, operated through the transcranial approach. We demonstrate, with the help of an edited video, that ANS located in the sellar and parasellar region can be safely and effectively operated through a transsphenoidal approach under endoscopic visualization.
CASE DESCRIPTION
Here, we present a case of a 30-year-old male who presented with a nine-month history of diplopia, weight gain, and loss of sexual functions. On neuro-opthalmological examination, a mild abducens palsy on the left side. Other cranial nerves were intact. On endocrinological testing, mild hypopituitarism on gonadal and thyroid axes. Magnetic resonance imaging (MRI) scan showed a contrast-enhanced cystic lesion in the sellar and parasellar region extending into the left temporal fossa. The patient underwent endonasal transsphenoidal endoscopic resection. A binostril standard approach was used, the left middle concha resected, and the nasoseptal flap was raised [Video 1]. The tumor was relatively soft and avascular yet invasive and could be removed with straight and curved suctions and gentle curettage. Subcapsular dissection was the key to saving the sixth nerve. Only minimal remnant posterior to the left internal carotid artery was assumed to be left behind. No cerebrospinal fluid (CSF) leakage was noted during the surgery. The skull base defect was reconstructed with the left-sided nasoseptal flap [Video 1]. Postoperatively, no new cranial nerve deficits. Diplopia is preoperative. Endocrine functions were unchanged. No CSF leak was observed. Postoperative MRI scan showed a near total resection. There was no operation-relevant complication. Diplopia resolved completely in a follow-up period of 6 months.
CONCLUSION
The endoscopic transsphenoidal route is safe and effective for the resection of parasellar ANS. Subcapsular dissection is key to keep the sixth nerve intact.
PubMed: 38741979
DOI: 10.25259/SNI_609_2023 -
Frontiers in Neuroanatomy 2024The topographic anatomy of the abducens nerve has been the subject of research for more than 150 years. Although its vulnerability was initially attributed to its...
The topographic anatomy of the abducens nerve has been the subject of research for more than 150 years. Although its vulnerability was initially attributed to its length, this hypothesis has largely lost prominence. Instead, attention has shifted toward its intricate anatomical relations along the cranial base. Contrary to the extensive anatomical and neurosurgical literature on abducens nerve anatomy in humans, its complex anatomy in other species has received less emphasis. The main question addressed here is why the human abducens nerve is predisposed to injury. Specifically, we aim to perform a comparative analysis of the basicranial pathway of the abducens nerve in mammals and primates. Our hypothesis links its vulnerability to cranial base flexion, particularly around the sphenooccipital synchondrosis. We examined the abducens nerve pathway in various mammals, including primates, humans ( = 40; 60% males; 40% females), and human fetuses ( = 5; 60% males; 40% females). The findings are presented at both the macroscopic and histological levels. To associate our findings with basicranial flexion, we measured the cranial base angles in the species included in this study and compared them to data in the available literature. Our findings show that the primitive state of the abducens nerve pathway follows a nearly flat (unflexed) cranial base from the pontomedullary sulcus to the superior orbital fissure. Only the gulfar segment, where the nerve passes through Dorello's canal, demonstrates some degree of variation. We present evidence indicating that the derived state of the abducens pathway, which is most pronounced in humans from an early stage of development, is characterized by following the significantly more flexed basicranium. Overall, the present study elucidates the evolutionary basis for the vulnerability of the abducens nerve, especially within its gulfar and cavernous segments, which are situated at the main synchondroses between the anterior, middle, and posterior cranial fossae-a unique anatomical relation exclusive to the abducens nerve. The principal differences between the pathways of this nerve and those of other cranial nerves are discussed. The findings suggest that the highly flexed human cranial base plays a pivotal role in the intricate anatomical relations and resulting vulnerability of the abducens nerve.
PubMed: 38741761
DOI: 10.3389/fnana.2024.1383126 -
Diagnostics (Basel, Switzerland) Apr 2024A 77-year-old-man with arterial hypertension, diabetes mellitus type II presented at our clinic for a routine ophthalmological exam. He complained of intermittent double...
A 77-year-old-man with arterial hypertension, diabetes mellitus type II presented at our clinic for a routine ophthalmological exam. He complained of intermittent double vision. The ophthalmic examination revealed paralysis of III (n. oculomotorius) and VI (n. abducens) cranial nerves with ptosis, deficit in elevation and abduction of the left eye. The patient underwent urgent MRI imaging of the brain/orbits and paranasal sinuses, and urgent neurological assessment. MRI revealed a volume-occupying process, starting from the posterior wall of the left maxillary sinus with perineural diffusion and involvement of the homolateral trigeminal nerve, intracranial spread in the medial cranial fossa and involvement of the cavernous, sphenoidal sinuses and the orbital apex on the left side. Biopsy was performed, and the histology resulted in sinonasal squamous cell carcinoma with intracranial spread.
PubMed: 38732347
DOI: 10.3390/diagnostics14090932 -
Journal of Neurosurgery. Case Lessons May 2024Unilateral cranial nerve (CN) VI, or abducens nerve, palsy is rare in children and has not been reported in association with Chiari malformation type 1 (CM1) in the...
BACKGROUND
Unilateral cranial nerve (CN) VI, or abducens nerve, palsy is rare in children and has not been reported in association with Chiari malformation type 1 (CM1) in the absence of other classic CM1 symptoms.
OBSERVATIONS
A 3-year-old male presented with acute incomitant esotropia consistent with a unilateral, left CN VI palsy and no additional neurological symptoms. Imaging demonstrated CM1 without hydrocephalus or papilledema, as well as an anterior inferior cerebellar artery (AICA) vessel loop in the immediate vicinity of the left abducens nerve. Given the high risk of a skull base approach for direct microvascular decompression of the abducens nerve and the absence of other classic Chiari symptoms, the patient was initially observed. However, as his palsy progressed, he underwent posterior fossa decompression with duraplasty (PFDD), with the aim of restoring global cerebrospinal fluid dynamics and decreasing possible AICA compression of the left abducens nerve. Postoperatively, his symptoms completely resolved.
LESSONS
In this first reported case of CM1 presenting as a unilateral abducens palsy in a young child, possibly caused by neurovascular compression, the patient's symptoms resolved after indirect surgical decompression via PFDD.
PubMed: 38710112
DOI: 10.3171/CASE23539 -
Cureus Apr 2024Herpes zoster ophthalmicus (HZO) is a condition resulting from the reactivation of dormant varicella zoster virus within the sensory nerve ganglion in the ophthalmic...
Herpes zoster ophthalmicus (HZO) is a condition resulting from the reactivation of dormant varicella zoster virus within the sensory nerve ganglion in the ophthalmic branch of the trigeminal nerve. The tell-tale rash along one side of the nerve tract accompanied by pain, a burning sensation, and itching alerts health practitioners on the right path to diagnosis. Conversely, HZO can present with other rarer complications such as intraocular and extraocular manifestations. This case report deals with a seemingly healthy 45-year-old female who developed left abducens nerve palsy within one week of developing a vesicular rash on the same side. Curiously, those afflicted are usually of an advanced age or suffer from an immunocompromised state; this patient however suffered from no other comorbidities nor did she report having been in contact with anyone of a similar affliction. In this case, the classical treatment regime of antivirals and corticosteroids resulted in the complete resolution of the infection and the return of full ocular function. Being able to recognize and appreciate these typical and atypical signs and symptoms of HZO can aid in the further propagation of good outcomes and timely resolutions.
PubMed: 38707108
DOI: 10.7759/cureus.57506 -
Cureus Apr 2024Myasthenia gravis is an autoimmune disease of the neuromuscular junction caused by autoantibodies directed against the acetylcholine receptors. It presents with...
Myasthenia gravis is an autoimmune disease of the neuromuscular junction caused by autoantibodies directed against the acetylcholine receptors. It presents with skeletal muscle weakness, often initially presenting with ocular symptoms such as ptosis and diplopia. When myasthenia gravis is isolated to only ocular symptoms, it is referred to as ocular myasthenia gravis (OMG). Here, we present an atypical initial presentation of OMG in a 68-year-old male patient presenting with isolated abducens nerve palsy at the initial onset. With this case report, we highlight the importance of a thorough history and clinical assessment necessary for a timely diagnosis of OMG in patients who present with isolated abducens nerve palsy.
PubMed: 38707069
DOI: 10.7759/cureus.57501