Overaction of the inferior oblique(IO) muscle is manifested by elevation of the adducted eye and from the clinical point of view there are two types of overaction. The primary type is of unknown cause, whereas the secondary type is usually related to the palsy of the ipsilateral superior oblique or contralateral superior rectus. An ultrastructural study on the overacting IO muscles was performed compared to normal IO muscles by electron microscopy. Of 16 biopsies of overacting IO muscles, four had primary overacting inferior obliques and twelve had secondary overacting inferior obliques due to paralysis of superior oblique muscle. Additional four IO muscle, obtained from patients with intraocular diseases served as control specimens. The most striking abnormalities were aggregations of mitochondria and degenerating mitochondrial profiles and increased vacuolization in primary and secondary overacting muscles. Many muscle fibers were in different stages of atrophy, and hypertrophy and regeneration of muscle fibers were sometimes visible. The results suggest that the primary overacting IO muscle might be the result of a paresis of the superior oblique muscle.
Biopsy ; Humans ; Mitochondria/ultrastructure ; Ocular Motility Disorders/*pathology ; Oculomotor Muscles/*ultrastructure ; Ophthalmoplegia/pathology ; Vacuoles/ultrastructure

Myasthenia gravis is a chronic disease characterized by voluntary muscle weakness and fatigue. Myasthenia gravis was first described by Thomas Willis in 1672. The etiology of myasthenia gravis is not clarified but recently it has been suggested that it is an autoimmune disease. Ocular myasthenia gravis is characterized clinically by blepharoptosis and external ophthalmopleia. The illness has a tendency to exacerbation and spontaneous remission. The authors observed a case of severe ocular myasthenia gravis which had developed total external ophthalmoplegia. And we reviewed the literature of recent years related to myasthenia gravis, particulary for its etiology, clinical course, pathology, diagnosi, treatment, and prognosis.
Autoimmune Diseases ; Blepharoptosis ; Chronic Disease ; Fatigue ; Muscle, Skeletal ; Myasthenia Gravis* ; Ophthalmoplegia* ; Pathology ; Prognosis ; Remission, Spontaneous

Chronic progressive external ophthalmoplegia (CPEO) is a rare clinical syndrome characterized by slowly progressive paralysis of extraocular muscles. We report a male patient who had a 20 year history of CPEO. Histological examination of left deltoid muscle showed characteristic ragged red fibers. Electron microscopy revealed a number of abnormal mitochondria which contain paracrystalline inclusion bodies.
Biopsy ; Chronic Disease ; Humans ; Male ; Middle Aged ; Mitochondria/ultrastructure ; Muscles/ultrastructure ; Ophthalmoplegia/*diagnosis/pathology

Congenital fiber type disproportion (CFTD) is a form of congenital myopathy characterized by histologic findings of the smallness of type 1 fiber and type 1 fiber predominance. It is usually associated with hypotonia and motor weakness of the limb muscles at birth or the neonatal period. We report a 6-year-old girl with limb weakness and ophthalmoplegia, whose muscle pathology showed the classic pattern of CFTD without any other abnormality.
Child ; Extremities ; Female ; Humans ; Muscle Hypotonia ; Muscles ; Muscular Diseases ; Myopathies, Structural, Congenital* ; Ophthalmoplegia* ; Parturition ; Pathology

Child ; Cranial Nerves ; pathology ; Eyelids ; Female ; Humans ; Male ; Ophthalmoplegia ; etiology ; Polyradiculopathy ; complications

BACKGROUNDOculomotor palsy is a common complication in patients with posterior communicating aneurysm. This study was conducted to investigate the postoperative recovery of patients with posterior communicating aneurysm complicated with oculomotor palsy and to analyze the factors influencing length of recovery.

METHODSFrom 2000 to 2006, 148 patients with posterior communicating aneurysm were treated at our hospital, with 74 of them having concurrent unilateral oculomotor palsy. All of the patients underwent craniotomy after the diagnosis by means of whole-brain digital subtraction angiography (DSA). The patients were divided into two groups for observation of postoperative recovery during the follow-up period. Patients in group A were treated with simple pedicle clipping of the aneurysm while patients in group B were treated with pedicle clipping of the aneurysm and decompression of the oculomotor nerve.

RESULTSOf the 40 patients in group A, 20 underwent surgery within 14 days and completely recovered from oculomotor palsy in 10 - 40 days. Fourteen patients underwent surgery within 14 - 30 days, of whom 12 completely recovered within 30 - 90 days and 2 cases recovered incompletely. The remaining six patients underwent surgery after more than 30 days; of these, four patients recovered completely and two recovered incompletely. Of the 34 cases in group B, 15 underwent surgery within 14 days and completely recovered from oculomotor palsy in 10 - 40 days. Sixteen patients underwent surgery in 14 - 30 days, of whom 14 completely recovered in 30 - 90 days and 2 recovered incompletely. The remaining three patients underwent surgery after more than 30 days, of whom two patients recovered completely and one recovered incompletely.

CONCLUSIONSEarly diagnosis and surgical treatment of patients with unilateral oculomotor palsy induced by posterior communicating aneurysm are important to full postoperative recovery of the oculomotor nerve. No correlation was found, however, between decompression of the oculomotor nerve, such as excision or puncture of the aneurysm, and postoperative recovery time.

Adult ; Aged ; Female ; Humans ; Intracranial Aneurysm ; complications ; pathology ; surgery ; Male ; Middle Aged ; Ophthalmoplegia ; etiology ; pathology ; surgery ; Treatment Outcome

A case of painful ophthalmoplegia with unilateral ocular pain, fixed eyeball to all directions of gaze, and loss of vision is presented. After intensive steroid therapy, conjunctival chemosis subsided markedly, but no improvement was seen in other clinical signs. We took a CT scan of orbit brain and performed nasopharyngeal biopsy and open biopsy through craniectomy. Based on the results of clinical features and findings of the CT scan and tissues, we diagnosed painful ophthalmoplegia secondary to nasopharyngeal carcinoma metastasized to orbital apex and brain.
Brain Neoplasms/pathology/secondary ; Carcinoma, Squamous Cell/*complications/pathology/secondary ; Female ; Humans ; Middle Aged ; Nasopharyngeal Neoplasms/*complications/pathology ; Ophthalmoplegia/*etiology ; Orbital Neoplasms/pathology/secondary ; Pain/*etiology

Blepharoptosis is a common indication for surgery in plastic surgery units, yet its possible underlying pathology frequently remains unidentified. A 52-year-old man with a 20-year history of progressive bilateral ptosis (right>left) presented with recurrent ptosis of both eyes; he had undergone an operation on the levator aponeurosis 12 years prior. Due to the suspicion of an underlying disease, he was evaluated further. Chronic progressive external ophthalmoplegia in transition to the more severe syndromic variant Kearns-Sayre syndrome, a mitochondrial disorder causing myopathy, was diagnosed. The patient was treated with coenzyme Q10, and he underwent ptosis surgery on both eyes. This case illustrates a potentially multi-systemic disease that was diagnosed by a further evaluation of a common symptom, in this case worsening blepharoptosis. Awareness of myopathic symptoms is necessary to prevent overlooking serious yet improvable conditions.
Blepharoplasty ; Blepharoptosis* ; Humans ; Kearns-Sayre Syndrome* ; Middle Aged ; Mitochondrial Diseases ; Muscular Diseases ; Ophthalmoplegia, Chronic Progressive External ; Pathology ; Surgery, Plastic

The cavernous sinus of skull base is a extremely rare metastastatic site for hepatocellular carcinoma (HCC). A 51-year-old man was diagnosed with HCC by liver biopsy and palliative radiotherapy on HCC including main portal vein was performed. One month later, he was admitted due to sudden onset ptosis. Neurologic findings were normal except for abnormal movement of right eye, and it raised the possibility of abnormality in the right occulomotor, trochlear and the abducens nerves. Contrast-enhanced CT scan of brain showed a mass with homogeneous enhancement involving the right cavernous sinus. T2-weighted axial MR images demonstrated a homogeneous mass with intermediate signal intensity, and contrast-enhanced axial T1-weighted MR images demonstrated a mass with homogeneous enhancement in the right cavernous sinus. We describe a case of HCC metastasis to the cavernous sinus with symptoms of ptosis and disturbance of right eyeball movement.
Blepharoptosis/*etiology/pathology ; Carcinoma, Hepatocellular/complications/*diagnosis/*secondary ; Cavernous Sinus/*pathology ; Humans ; Liver Neoplasms/complications/*pathology ; Male ; Middle Aged ; Ophthalmoplegia/pathology ; Skull Base Neoplasms/diagnosis/*secondary ; Tomography, X-Ray Computed

PURPOSE: To evaluate the spectrum of mitochondrial DNA (mtDNA) aberrations in patients with suspected chronic progressive external ophthalmoplegia (CPEO) and to establish the molecular diagnostic method for CPEO in Koreans. METHODS: We performed mtDNA analyses for single deletions with long-range PCR and direct sequencing, and for the nine important point mutations including 3243A>G and 8344A>G with PCR/RFLP in muscles, bloods and paraffin-embedded muscle sections of 16 Korean patients with suspected CPEO. RESULTS: Three novel single mtDNA deletions were identified in three patients' muscles: 3159bp deletion from np 6657 to np 9815, 7591bp from np 8429 to np 16019, and 6191bp from np 7799 to np 13989. In addition, multiple mtDNA deletions were found in one patient. None of the blood specimen had mtDNA deletions even in the patients with mtDNA deletion in muscle. All single deletion junctions were flanked by direct repeats of 6-8 bp. None of the nine mtDNA point mutations were found in muscles, bloods or paraffin-embedded muscle sections. CONCLUSIONS: We identified three novel single deletions by mtDNA analyses in the muscles of 3 patients with CPEO. However, point mutations were not found. Furthermore, we established a molecular diagnostic method for CPEO in Korea. Long-range PCR and direct sequencing of the muscles were appropriate as a molecular diagnostic method for CPEO in Koreans.
Diagnosis* ; DNA, Mitochondrial* ; Humans ; Korea ; Muscles ; Ophthalmoplegia, Chronic Progressive External* ; Pathology, Molecular ; Point Mutation ; Polymerase Chain Reaction ; Repetitive Sequences, Nucleic Acid