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Pediatrics and Neonatology Jan 2024
Topics: Humans; Abducens Nerve Diseases; Spasm; Reflex; Diagnostic Errors
PubMed: 37684162
DOI: 10.1016/j.pedneo.2023.05.006 -
Journal of Neuro-ophthalmology : the... Mar 2024Duane retraction syndrome (DRS) is known to relate to the absence of the abducens nucleus, with abnormal innervation of the lateral rectus (LR) muscle by branchesof the...
BACKGROUND
Duane retraction syndrome (DRS) is known to relate to the absence of the abducens nucleus, with abnormal innervation of the lateral rectus (LR) muscle by branchesof the oculomotor nerve (CN III). The purposes of this study were to investigate the morphological characteristics of the oculomotor nerve (CN III), the abducens nerve (CN VI), and the extraocular muscles in patients with clinically diagnosed Duane retraction syndrome (DRS) using MRI. In addition, we assessed the association between ocular motility, horizontal rectus muscle volumes, and CN III/VI in patients with Duane retraction syndrome (DRS).
METHODS
The study comprised 20 orthotropic control subjects (40 eyes) and 42 patients with Duane syndrome (48 eyes), including 20 patients with DRS Type I (24 eyes), 5 patients with DRS Type II (6 eyes), and 17 patients with DRS Type III (18 eyes). Three-dimensional (3D) T1/2 images of the brainstem and orbit were obtained to visualize the cranial nerves, especially the abducens (VI) and oculomotor (III) nerves, as well as extraocular muscles.
RESULTS
Based on the clinical classification, among 42 patients, MRI showed that the abducens nerves (CN VI) on the affected side were absent in 24 of 24 eyes (100%; 20 patients) with Type I DRS and in 16 of 18 eyes (88%; 16 patients) with Type III DRS. However, CN VI was observed in 6 of 6 eyes (100%; 5 patients) with Type II DRS and in 2 of 18 eyes (11%) with Type III DRS. CN III was observed in all patients. The oculomotor nerves on the affected side were thicker than those on the nonaffected contralateral side in DRS Type I ( P < 0.05) and Type III ( P < 0.05), but not in DRS Type II. Smaller LR and larger MR volumes were shown in the affected eye than that in the nonaffected eye in DRS Types I and III. Based on the presence or absence of CN VI, there was a tendency for thicker oculomotor nerves in the affected eye than in the nonaffected eye in the absence groups ( P < 0.05). However, no significant difference was found in the present group. In the CN VI absence groups, similar results were found in the affected eyes than in the nonaffected eyes as in DRS Types I and III. In addition, the presence of CN VI was correlated with better abduction ( P = 0.008). The LR and MR volumes have positive correlations with the oculomotor nerve diameter in the affected eye. However, there was no correlation between the range of adduction/abduction and the LR/MR ratio in patients with or without an abducens nerve.
CONCLUSIONS
Different types of DRS have different characteristic appearances of CN VI and CN III on MRI. Horizontal rectus muscles have morphological changes to adapt to dysinnervation of CN VI and aberrant innervation of CN III. Thus, these neuroimaging findings may provide a new diagnostic criterion for the classification of DRS, improving the comprehension of the physiopathogenics of this disease.
Topics: Humans; Duane Retraction Syndrome; Abducens Nerve; Oculomotor Muscles; Orbit; Magnetic Resonance Imaging
PubMed: 37682628
DOI: 10.1097/WNO.0000000000001909 -
Iranian Journal of Child Neurology 2023Cavernous hemangiomas (CHs) are vascular structures comprising abnormally dilated blood vessel clusters. This anomaly is estimated to occur in approximately one out of...
Cavernous hemangiomas (CHs) are vascular structures comprising abnormally dilated blood vessel clusters. This anomaly is estimated to occur in approximately one out of every 500-600 people. Individuals often show the first sign of cavernous hemangiomas in their second or third decade. Therefore, the presentation of this disorder is not common in children. This study presents a five year-old-girl who developed abruptly nearly fixed right-sided eye deviation and incoordination after a short course of viral infection. In physical examination, she had left peripheral facial, right eye oculomotor, and left abducens nerve palsy. Her brain's computed tomography (CT) scan revealed hemorrhages in the posterior aspect of the pons and some areas of the hemispheres in different stages. Following brain magnetic resonance imaging (MRI), multiple popcorn ball low-signal T2* lesions with both supra- and infratentorial locations with marked peripheral hypo intensities were seen. These findings were in favor of multiple cavernous hemangiomas of the brain. The novelty of this case was due to observing cavernous angiomas in both supra- and infratentorial spaces in pediatric age.
PubMed: 37637788
DOI: 10.22037/ijcn.v17i2.37749 -
European Heart Journal. Case Reports Aug 2023Orthotopic heart transplant (OHT) recipients are at increased risk for varicella zoster reactivation, and severe complications may arise due to their immunosuppressive...
BACKGROUND
Orthotopic heart transplant (OHT) recipients are at increased risk for varicella zoster reactivation, and severe complications may arise due to their immunosuppressive regimens. Managing immunosuppression in acute infection is difficult, and specific guideline recommendations or evidence from the literature are lacking. However, patient care must involve weighing the risk of transplant rejection with the consequences of worsening infection.
CASE SUMMARY
An OHT patient with a history of multiple episodes of acute rejection, latent varicella zoster virus (VZV) infection, and recent completion of anti-viral prophylaxis presented with unilateral facial droop and pain, abducens nerve palsy, crusting facial rash, and ear swelling. Imaging revealed necrotizing otitis externa, with associated otitis media, and petrous apicitis concerning for Gradenigo syndrome. A VZV-positive viral panel confirmed our suspicion for Ramsay Hunt syndrome (RHS). The patient's mentation continued to decline, and subsequent lumbar puncture also revealed VZV meningoencephalitis. The patient's mycophenolate mofetil (MMF) was suspended, with continuation of tacrolimus, and initiation of intravenous acyclovir. The patient demonstrated gradual resolution of his infection, without developing any signs of acute rejection.
DISCUSSION
Varicella zoster virus reactivation is common in OHT patients, particularly when viral prophylaxis is discontinued; however, cardiologists should be aware of the rarer manifestations that can manifest in these immunocompromised patients. This is the first documented case of simultaneous RHS, Gradenigo syndrome, and VZV meningoencephalitis in any patient, regardless of transplant status. We demonstrate that even in patients at very high risk of rejection, MMF can be safely discontinued and host immunity maintained with temporary tacrolimus monotherapy.
PubMed: 37575545
DOI: 10.1093/ehjcr/ytad373 -
Cureus Jul 2023Cerebral venous thrombosis (CVT) is a rare condition characterized by the obstruction of cerebral venous sinuses or cortical veins, leading to stroke-like symptoms. This...
Cerebral venous thrombosis (CVT) is a rare condition characterized by the obstruction of cerebral venous sinuses or cortical veins, leading to stroke-like symptoms. This case report presents a case of a 74-year-old male with isolated unilateral abducens nerve palsy as the sole sign of CVT, without accompanying symptoms or focal deficits. Neuroimaging, including CT and magnetic resonance venography, confirmed the diagnosis of CVT with a thrombus in the right transverse sinus. The patient was co-managed with the medical team and initiated on anticoagulation therapy. Follow-up showed resolution of diplopia and improvement in extraocular muscle movements. Unilateral abducens nerve palsy in CVT is rare, with most cases presenting as bilateral palsy. The case report emphasizes the importance of considering CVT in the differential diagnosis of isolated abducens nerve palsy and highlights the role of neuroimaging in early detection. Timely diagnosis and appropriate management are crucial for favorable outcomes in CVT cases. Further research is needed to enhance understanding of the pathophysiology, prognosis, and optimal management of this uncommon presentation.
PubMed: 37546125
DOI: 10.7759/cureus.41350 -
Cureus Jul 2023A healthy 28-year-old lady, para 1, presented to the emergency department with persistent frontal headache, nausea, and vomiting following an emergency cesarean...
A healthy 28-year-old lady, para 1, presented to the emergency department with persistent frontal headache, nausea, and vomiting following an emergency cesarean section four days ago. She experienced difficulties with six failed attempts of spinal anesthesia intrapartum before conversion to general anesthesia. A 25-gauge Whitacre needle was utilized for administering spinal anesthesia under a sitting position. The anesthetist noticed a loss of resistance upon needle insertion, but only a negligible amount of cerebrospinal fluid was obtained upon removing the stylet. The patient underwent an emergency cesarean section due to fetal distress, and she was not in labor during the attempts of spinal anesthesia. Otherwise, the cesarean section lasted for an hour and was uneventful. No intrapartum eclampsia or pre-eclampsia. She was diagnosed with post-dural puncture headache, and her symptoms improved after receiving intravenous hydration, oral caffeine, and non-steroidal anti-inflammatory drug (NSAIDs). However, on the sixth day after the spinal anesthesia, she suddenly developed double vision. Examination showed bilateral visual acuity was measured at 6/7.5. No proptosis or ptosis was noted. The relative afferent pupillary defect was negative with no anisocoria. Both eyes were orthophoria with normal head posture. Extraocular muscles revealed a right abduction restriction of -1 with the patient complaining of binocular horizontal diplopia at the right gaze, consistent with right abducens nerve palsy. Systemic neurological findings were normal, and imaging results were unremarkable. Diagnosis of right abducens nerve palsy post-dural puncture was made clinically. The patient was keen on conservative management instead of blood patch therapy. Hence, she was treated supportively via uni-ocular patching to relieve diplopia. Spontaneous complete recovery of the right abducens nerve palsy was observed after three weeks. Cranial nerve palsy is a rare complication reported following spinal anesthesia, with the abducens nerve being the commonest nerve involved. Although it is not always benign, the presented case showed spontaneous complete recovery of the right abducens nerve palsy after three weeks. Awareness of this uncommon complication will avoid unnecessary distress and investigative burden to both the patient and the doctor.
PubMed: 37539413
DOI: 10.7759/cureus.41298 -
Cureus Jun 2023We reported two cases of the central nervous system (CNS) multiple myeloma (MM) with unusual presentation of sixth nerve palsy. The first patient developed in the...
We reported two cases of the central nervous system (CNS) multiple myeloma (MM) with unusual presentation of sixth nerve palsy. The first patient developed in the setting of newly diagnosed MM and the second patient in the relapsed refractory setting. One underwent surgery, and the other received radiation. Both patients received systemic chemotherapy and noted improvement. We also performed a comprehensive literature review of previously published cases of sixth nerve palsy from MM. This review highlights the importance of recognizing this presentation of CNS multiple myeloma to avoid delays in diagnosis and to get appropriate management in time.
PubMed: 37503472
DOI: 10.7759/cureus.40998 -
Case Reports in Women's Health Sep 2023Isolated abducens nerve palsy is a rare presentation in women during pregnancy. When an abducens nerve palsy is elicited in a pregnant woman, work-up should start with...
Isolated abducens nerve palsy is a rare presentation in women during pregnancy. When an abducens nerve palsy is elicited in a pregnant woman, work-up should start with labs and neuroimaging to rule out mechanical and organic causes such as tumors, preeclampsia, and multiple sclerosis. This case report highlights a 35-year-old woman, gravida 1, para 0, who was sent to the local medical center by her ophthalmologist at 37 weeks of gestation due to a left-sided headache and blurry vision. Upon admission, work-up was negative for preeclampsia. Tick-borne disease panel and lumbar puncture were unrevealing. No other mechanical or lab abnormalities were elicited. Magnetic resonance venography revealed a diminutive left transverse sinus, left sigmoid sinus, and left internal jugular vein in comparison with the right, indicating a possible congenital variant. Labor was induced to see if this would alleviate the patient's abducens nerve palsy. After induction of labor and initiation of dexamethasone, the patient's sixth cranial nerve palsy began to improve.
PubMed: 37485440
DOI: 10.1016/j.crwh.2023.e00527