Aged ; Ethmoid Sinus ; pathology ; Female ; Humans ; Nasal Polyps ; complications ; Oculomotor Nerve Diseases ; complications ; Sphenoid Sinus ; pathology

Oculomotor cistern is normal anatomic structure that is like an arachnoid-lined cerebrospinal fluid-filled sleeve, containing oculomotor nerve. We report a case of arachnoid cyst in oculomotor cistern, manifesting as oculomotor nerve palsy. The oblique sagittal MRI, parallel to the oculomotor nerve, showed well-defined and enlarged subarachnoid spaces along the course of oculomotor nerve. Simple fenestration was done with immediate regression of symptom. When a disease develops in oculomotor cistern, precise evaluation with proper MRI sequence should be performed to rule out tumorous condition and prevent injury of the oculomotor nerve.
Adult ; Arachnoid Cysts/*diagnosis/surgery ; Female ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Neurosurgical Procedures ; Oculomotor Nerve/*pathology ; Oculomotor Nerve Diseases/*diagnosis/surgery

BACKGROUNDAlthough neuroradiological findings of Möbius syndrome have been reported as a result of brain and brainstem abnormalities, magnetic resonance imaging (MRI) now permits the direct imaging of the cranial nerve (CN) and branches in the orbits. This study presents the MRI findings in patients with sporadic Möbius syndrome.

METHODSProspectively, CNs were imaged in the cistern using head coils and three dimensional fast imaging employing steady-state acquisition (3D-FIESTA), yielding a 0.5 mm(2) resolution in planes of 0.8 mm thickness in seven patients with sporadic Möbius syndrome. The cavernous and intraorbital segment of the CN and the extraocular muscles (EOMs) were imaged with T1 weighting in all patients. The cavernous segment was imaged in coronal planes, while the intraorbit in quasicoronal planes were imaged using surface coils. Intraorbital resolution was 0.16 mm(2) within 2.0 mm thick planes.

RESULTSIn the seven patients, the CN were absent or showed hypoplasia in the cistern, cavernous sinus, and orbit. Abducens (CN VI) and facial (CN VII) nerves were absent on the affected sides. Unilateral CN IX (glossopharyngeal nerve) in two cases displayed dysplasia. Branches from the inferior division of CN III were observed to innervate the lateral rectus (LR) bilaterally in three cases and unilaterally in one case, and had intimate continuity with the LR muscle in two cases bilaterally and two cases unilaterally. Hypoplasia of EOMs was shown in five cases. Dysplasia of the medulla on the left side was found in one patient.

CONCLUSIONSDirect imaging of CNs and EOMs by MRI is useful in diagnosis of Möbius syndrome. It can directly demonstrate the abnormalities of the CN and orbital structures. The absence or hypoplasia of CN VI and CN VII may be the most common radiologic features in sporadic Möbius syndrome, and hypoplasia of CN IX may be an associated feature. The abnormality of EOMs and aberrant innervations in the orbit should be observed, and may be important for the study of the etiology.

Cranial Nerves ; pathology ; Humans ; Imaging, Three-Dimensional ; Magnetic Resonance Imaging ; methods ; Mobius Syndrome ; pathology ; Oculomotor Nerve ; pathology

BACKGROUNDSome studies indicated that cases of idiopathic oculomotor nerve palsy can be explained by vascular compression of the oculomotor nerve. Vascular contact with or compression to the cisternal segment of the oculomotor nerve has been reported frequently in asymptomatic individuals. In this study, we retrospectively analyzed the relationship between the oculomotor nerve's cisternal segment and adjacent arteries in asymptomatic patients and the prevalence of this occurrence via magnetic resonance imaging (MRI).

METHODMRI of bilateral oculomotor nerves in 93 asymptomatic patients were reviewed. The oculomotor nerve-artery relationship was evaluated and classified from levels 1 to 3, representing the degrees of contact on oblique transverse and oblique sagittal reconstructed MRI. Prevalence of the nerve-artery relationship at each level was described. The correlation between the nerve-vessel relationship (levels) and the age was analyzed by Spearman's rank correlation analysis.

RESULTSCisternal segment of the oculomotor nerve did not have contact with any artery (level 1) in 27.4% (51/186) nerves. One hundred nerves made contact with at least one artery (level 2), but their shapes or configurations were not changed; 35 nerves (18.8%) were displaced or distorted due to artery compression (level 3). The posterior cerebral artery had the greatest incidence of making contact with or compressing the cisternal segment of the oculomotor nerve (58.1%). No significant correlation between nerve-vessel relationship (levels) and the age was found in this study.

CONCLUSIONSWhether oculomotor nerve contact with or compression by one or more arteries is of high prevalence in asymptomatic individuals as evidenced by MRI examination. There is no correlation with individual age. Discretion should be used when making an etiological diagnosis of vascular compression for patients with oculomotor nerve palsy. Further investigation of other causes is warranted.

Adolescent ; Adult ; Age Factors ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Magnetic Resonance Imaging ; methods ; Male ; Middle Aged ; Nerve Compression Syndromes ; complications ; pathology ; Oculomotor Nerve ; pathology ; Oculomotor Nerve Diseases ; etiology ; pathology ; Young Adult

Axenfeld-Rieger syndrome (ARS) is associated with ocular and systemic anomalies. PITX2 is known to be a major controlling gene in the pathogenesis of ARS and is associated with differentiation in both the neural crest and mesoderm during eye development. A 4-year-old girl with bilateral ARS had 20 prism diopters (PD) of exotropia with 30PD of A- pattern deviation, more than 20PD of dissociated vertical deviation (DVD), and severe superior oblique overaction (SOOA). During surgery we observed that the SO inserted more posteriorly than normal. We believe this finding is one of the abnormal manifestations of the development of the extraocular muscles in ARS.
*Abnormalities, Multiple ; Anterior Eye Segment/*abnormalities ; Child, Preschool ; Eye Abnormalities/*diagnosis/surgery ; Eye Movements ; Female ; Follow-Up Studies ; Humans ; Oculomotor Muscles/*abnormalities/surgery ; Ophthalmologic Surgical Procedures/*adverse effects ; Optic Nerve/abnormalities ; Postoperative Complications ; Sclera/*pathology/surgery ; Syndrome ; Tooth Abnormalities/*genetics

Acute sensorineural hearing loss is unusual as initial manifestations in a child with acute lymphoblastic leukemia (ALL), even though facial or oculomotor nerve palsy as early finding of leukemia was reported. The pathology of sensorineural hearing loss in leukemia showed as leukemic cell infiltration, hemorrhage, infection, and local ischemia due to hyperviscosity. A 12-year-old boy with ALL was admitted due to multiple cervical lymphadenopathy with left sided sensorineural deafness. He complained gross hematuria and purpura on whole body. His initial complete blood cell counts were hemoglobin 11.9 g/dL, hematocrit 34.3%, white blood cells 164,000/muL (segmented neutrophils 3%, lymphocytes 11%, monocytes 2%, immature blast 84%), and platelet 28,000/muL. Pure tone audiogram revealed profound sensorineural hearing loss of the left ear at all frequencies. His brain MRI showed no definite abnormal findings without hemorrhage or infarction in inner ear or temporal lobe. He received induction chemotherapy and total 4 times of transtympanic steroid injection with 1 week interval. His hearing power at complete remission was more improved than admission, but not completely recovered until 5 months. We proposed that hearing impairment might be an initial manifestation in acute leukemia with hyperleukocytosis.
Blood Cell Count ; Blood Platelets ; Brain ; Child* ; Deafness ; Ear ; Ear, Inner ; Hearing ; Hearing Loss ; Hearing Loss, Sensorineural* ; Hematocrit ; Hematuria ; Hemorrhage ; Humans ; Induction Chemotherapy ; Infarction ; Ischemia ; Leukemia ; Leukocytes ; Lymphatic Diseases ; Lymphocytes ; Magnetic Resonance Imaging ; Male ; Monocytes ; Neutrophils ; Oculomotor Nerve Diseases ; Pathology ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Purpura ; Temporal Lobe