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Cureus May 2024Moebius syndrome is a rare disease characterized by unilateral or bilateral facial nerve palsies with/without other cranial nerve palsy. It manifests clinically with...
Moebius syndrome is a rare disease characterized by unilateral or bilateral facial nerve palsies with/without other cranial nerve palsy. It manifests clinically with facial muscle weakness and/or ophthalmoplegia and can be associated with other physical anomalies such as various limb deformities and orofacial malformation. Herein, we have described the clinical and radiological features of Moebius syndrome in a 9-year-old female child who presented with left-side facial palsy and bilateral complete horizontal gaze palsy.
PubMed: 38910704
DOI: 10.7759/cureus.60887 -
Journal of Radiology Case Reports 2024Multiple myeloma is a plasma cell neoplasm, which may present as a solitary plasmacytoma and, uncommonly, as an extramedullary plasmacytoma. Intracranial plasmacytomas...
Multiple myeloma is a plasma cell neoplasm, which may present as a solitary plasmacytoma and, uncommonly, as an extramedullary plasmacytoma. Intracranial plasmacytomas may manifest in central nervous system involvement as cranial nerve palsies. Cranial nerve six palsy is the most common in cases of malignancy. However, isolated abducens palsy presenting as multiple myeloma recurrence is very uncommon. Here, we detail two cases in which intracranial plasmacytoma lesions were present within the region of the Dorello canal, resulting in acute isolated unilateral diplopia from disease recurrence in the absence of systemic marrow involvement.
Topics: Humans; Abducens Nerve Diseases; Multiple Myeloma; Neoplasm Recurrence, Local; Male; Magnetic Resonance Imaging; Middle Aged; Aged; Diagnosis, Differential; Plasmacytoma; Female; Diplopia; Brain Neoplasms
PubMed: 38910589
DOI: 10.3941/jrcr.v18i1.5240 -
Cureus Jun 2024Plasmacytomas rarely affect the skull base and may be found as an isolated lesion or as a part of multiple myeloma. The typical feature of plasmacytomas is aggressive...
Plasmacytomas rarely affect the skull base and may be found as an isolated lesion or as a part of multiple myeloma. The typical feature of plasmacytomas is aggressive bone destruction in the skull. It is often confused with the chordoma of the clivus. The most common location for skull-base plasmacytomas is the nasopharynx. The most commonly affected cranial nerve in clivus tumors is the abducens nerve. In our 64-year-old male case, a plasmacytoma was detected in the clivus. There was ptosis and decreased vision due to optic nerve and oculomotor nerve involvement due to the plasmacytoma. Radiotherapy was applied for the treatment.
PubMed: 38882228
DOI: 10.7759/cureus.62447 -
Sisli Etfal Hastanesi Tip Bulteni 2024Numerous neurological manifestations associated with COVID-19 have been reported. However, abducens nerve palsy (ANP) associated with COVID-19 is very rare and mostly...
Numerous neurological manifestations associated with COVID-19 have been reported. However, abducens nerve palsy (ANP) associated with COVID-19 is very rare and mostly related to accompanying respiratory symptoms. Here we present a 29-year-old woman with unilateral ANP manifesting with diplopia and positive SARS-CoV-2 S antibodies, which were checked later. On admission, she had signs of viral pneumonia in thorax CT without any respiratory symptoms. Her cranial neuroimaging revealed no abnormality. Following treatment with favipiravir 2x1600 mg loading dose and then 2x600mg daily maintenance, dexamethasone 8 mg/day and enoxaparin 6000 IU/day, her CT findings recovered completely whereas her ANP only partially resolved. One week after the end of COVID-19 treatment, she also developed Herpes simplex keratitis which was successfully treated with valacyclovir. It should be kept in mind that isolated abducens nerve palsy may be the only finding of COVID-19 cases without any respiratory symptoms.
PubMed: 38808042
DOI: 10.14744/SEMB.2023.55491 -
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 -
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