1/80. Schwannoma in patients with isolated unilateral trochlear nerve palsy.PURPOSE: To describe the clinical features of patients with isolated unilateral trochlear nerve palsy secondary to imaging-defined schwannoma of the trochlear nerve. methods: A chart review of all patients seen at the Neuro-ophthalmology Unit at Emory University since 1989. Of 221 patients with trochlear nerve palsy, six had a lesion consistent with a trochlear nerve schwannoma. RESULTS: The six patients had isolated unilateral trochlear nerve palsy. Duration of diplopia before diagnosis averaged 6 months. magnetic resonance imaging demonstrated circumscribed, enhancing lesions along the cisternal course of the trochlear nerve, all measuring less than 5 mm in greatest dimension. Five of the patients were seen in follow-up, over periods ranging from 11 to 26 months from initial presentation (mean, 15.6 months; standard deviation, 6.0 months). All of these patients remained stable except one, who was slightly worse at 15 months by clinical measurements and magnetic resonance imaging. None of these patients have developed additional symptoms or signs of cranial nerve or central nervous system involvement. CONCLUSIONS: The differential diagnosis of an isolated unilateral fourth cranial nerve palsy should include an intrinsic neoplasm of the trochlear nerve. magnetic resonance imaging is useful, both for diagnosis and follow-up. These patients can remain stable and may not require neurosurgical intervention.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/80. Neuro-ophthalmologic manifestations of Maffucci's syndrome and Ollier's disease.patients with Ollier's disease (multiple skeletal enchondromas) and Maffucci's syndrome (multiple enchondromas associated with subcutaneous hemangiomas) may develop skull base chondrosarcomas or low-grade astrocytomas as a delayed consequence of these disorders. We report three patients with Ollier's disease and Maffucci's syndrome who had diplopia as the initial manifestation of intracranial tumors. Since patients with Maffucci's syndrome and Ollier's disease are at risk for the delayed development of brain and systemic neoplasms, neuroophthalmologists must be aware of the need for long-term surveillance in patients affected by these conditions.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/80. Benign triton tumor of the trigeminal nerve.Benign triton tumor (neuromuscular hamartoma) is a rare tumor of the peripheral nerves, comprising mature neural and striated muscle elements. We report the third case, according to the literature, of a benign triton tumor affecting the Vth cranial nerve in a 4-year-old male child. The tumor was seen to involve the mandibular division of the trigeminal nerve in the infratemporal fossa and extend intracranially by eroding the base of middle cranial fossa. The mass was totally excised. In the tumor, bundles of axons intermingling with fascicles of relatively mature skeletal muscle were found. This type of hamartomatous lesions of neuroectodermal-mesenchymal origin could have arisen as the result of incorporation of mesenchymal tissue into nerve sheaths during embryogenesis or aberrant differentiation of neuroectodermal component into mesenchymal elements.- - - - - - - - - - ranking = 135.26948147875keywords = muscle (Clic here for more details about this article) |
4/80. Neuromyotonia of the abducens nerve after hypophysectomy and radiation.The clinical signs of the rarely encountered ocular neuromyotonia consist of transient involuntary tonic contraction and delayed relaxation of single or multiple extraocular muscles, resulting in episodic diplopia. With a mean time delay of 3.5 years, this motility disorder frequently follows tumor excision or adjuvant radiation near the skull base. Ocular neuromyotonia may reflect inappropriate discharge from oculomotor neurons with unstable cell membranes because of segmental demyelinization by tumor compression and radiation-induced microangiopathy. In the present paper, the authors present the case of a 53-year-old patient with a history of transsphenoidal hypophysectomy and adjuvant radiotherapy, who underwent strabismus surgery for abducens palsy.- - - - - - - - - - ranking = 67.634740739376keywords = muscle (Clic here for more details about this article) |
5/80. Clinical features of congenital absence of the superior oblique muscle as demonstrated by orbital imaging.INTRODUCTION: Absence of an extraocular muscle was considered rare when demonstrable only by surgical exploration or necropsy. This study presents advances in orbital imaging to correlate clinical findings with absence of the superior oblique (SO) muscle. methods: We performed high-resolution coronal orbital imaging by magnetic resonance imaging (222 orbits) or computerized radiographic tomography (32 orbits) in 127 patients with strabismus. We reviewed the histories and ocular motility examinations in patients who had absence of 1 or both SO muscles. Findings were compared with patients who were clinically diagnosed with SO palsy but had demonstrable SO muscles on orbital imaging. RESULTS: SO muscles were absent in 6 patients. All had histories suggesting congenital strabismus. In patients old enough for quantitative testing who had unilateral SO muscle absence, visual acuity was a least 20/25 in all and stereopsis was better than 80 arc/s in one. Three patients were orthotropic in primary position. Five patients with unilateral SO muscle absence had clinical findings variably consistent with SO palsy, whereas a sixth patient with Duane syndrome had clinically unsuspected bilateral SO muscle absence. Versions and patterns of hypertropia in patients with SO muscle absence overlapped findings of 20 patients with SO palsy but demonstrable SO muscles. CONCLUSIONS: Imaging can frequently demonstrate absence of the SO muscle in patients with SO palsy. Such patients may have good vision and stereopsis and clinical findings indistinguishable from SO palsy without absence of the SO muscle. Orbital imaging should be considered in the evaluation of congenital SO palsy to facilitate planning of effective surgical correction.- - - - - - - - - - ranking = 1082.15585183keywords = muscle (Clic here for more details about this article) |
6/80. Isolated trigeminal neuropathy due to trigeminal nerve root hemorrhage.BACKGROUND: Isolated trigeminal neuropathy is uncommon; causes include trauma, inflammation, or neoplasm. methods: We report a patient who fell and struck his head during a myocardial infarction, was treated with streptokinase, and developed symptoms and signs of an isolated trigeminal sensory neuropathy. RESULTS: Imaging showed hemorrhage in the trigeminal nerve root; follow-up imaging showed resolution of the hemorrhage, but no underlying structural lesion. CONCLUSION: A combination of head trauma plus thrombolysis resulted in an isolated trigeminal neuropathy.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/80. Hereditary motor and sensory neuropathy associated with auditory neuropathy in a Gypsy family.In a Slovene Gypsy family of 19 subjects from four generations three patients with clinical characteristics compatible with hereditary motor and sensory neuropathy - Lom (HMSNL). were found They had severe distal and milder proximal muscle atrophy and weakness with areflexia of myotatic jerks. Two had facial weakness at the time when already wheelchair bound. All sensory modalities were affected distally in the limbs. Sluggish pupillary responses to light and convergence were found. They had skeletal abnormalities. One patient had polydactily on the hand. Nerve conduction studies were compatible with demyelinative polyneuropathy. Nerve biopsy showed mainly axonal loss without hypertrophic changes. Auditory neuropathy was diagnosed in all of them. None of the patients had duplication of 17p1.2-12 or point mutations in the Protein zero. Peripheral myelin protein and Connexin32 genes. Similar disorder that mapped to 8q24 was previously described in some Bulgarian and Italian Gypsy families. Members of our family may suffer from the same hereditary disease and may carry the same ancestor mutation, which was in the past spread in European Gypsy populations.- - - - - - - - - - ranking = 67.634740739376keywords = muscle (Clic here for more details about this article) |
8/80. Leptomeningeal glioblastoma presenting with multiple cranial neuropathies and confusion.glioblastoma multiforme (GBM) is the commonest primary malignant neoplasm of the CNS. Usually, patients present with seizures and headache but in the elderly, confusion and generalised cognitive decline are more frequently the initial features. Multiple cranial nerve lesions as a manifestation of leptomeningeal meningitis is a rare presentation of GBM. The diagnosis is not often suggestive on either brain computed tomography (CT) or magnetic resonance imaging (MRI) and is usually confirmed by cerebrospinal fluid (CSF) cytology or histology. We describe the case of an 80-year-old man, who presented with multiple cranial nerve palsies and confusion secondary to leptomeningeal gliomatosis, in whom GBM was detected along the intra-ventricular lining of the left lateral ventricle at ventriculoscopy, in the absence of a distinct parenchymal lesion.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/80. Spontaneous carotid dissection presenting lower cranial nerve palsies.Cranial nerve palsy in internal carotid artery (ICA) dissection occurs in 3--12% of all patients, but in 3% of these a syndrome of hemicranias and ipsilateral cranial nerve palsy is the sole manifestation of ICA dissection, and in 0.5% of cases there is only cranial nerve palsy without headache. We present two cases of lower cranial nerve palsy. The first patient, a 49-year-old woman, developed left eleventh and twelfth cranial nerve palsies and ipsilateral neck pain. The angio-RM showed an ICA dissection with stenosis of 50%, beginning about 2 cm before the carotid channel. The patient was treated with oral anticoagulant therapy and gradually improved, until complete clinical recovery. The second patient, a 38-year-old woman, presented right hemiparesis and neck pain. The left ICA dissection, beginning 2 cm distal to the bulb, was shown by ultrasound scanning of the carotid and confirmed by MR angiogram and angiography with lumen stenosis of 90%. Following hospitalisation, 20 days from the onset of symptoms, paresis of the left trapezius and sternocleidomastoideus muscles became evident. The patient was treated with oral anticoagulant therapy and only a slight right arm paresis was present at 10 months follow-up. Cranial nerve palsy is not rare in ICA dissection, and the lower cranial nerve palsies in various combinations constitute the main syndrome, but in most cases these are present with the motor or sensory deficit due to cerebral ischemia, along with headache or Horner's syndrome. In the diagnosis of the first case, there was further difficulty because the cranial nerve palsy was isolated without hemiparesis, and the second case presented a rare association of hemiparesis and palsy of the eleventh cranial nerve alone. Compression or stretching of the nerve by the expanded artery may explain the palsies, but an alternative cause is also possible, namely the interruption of the nutrient vessels supplying the nerve, which in our patients is more likely.- - - - - - - - - - ranking = 67.634740739376keywords = muscle (Clic here for more details about this article) |
10/80. dysarthria as the isolated clinical symptom of borreliosis--a case report.This report presents a case of dysarthria due to hypoglossal nerve mono-neuropathy as the only consequence of neuroborreliosis. The 65-year-old man with a seven-months history of articulation disturbances was examined. The speech of the patient was slow and laboured. A slight weakness of the muscles of the tongue (left-side) was observed. The patient suffered from meningitis due to borrelia burgdorferi infection in 1999 and initially underwent a successful antibiotic treatment. Detailed radiological investigation and psychological tests were performed and co-existing neurological diseases were excluded. To describe profile of speech abnormalities the dysarthria scale was designed based on S. J. Robertson dysarthria Profile. There were a few disturbances found in self-assessment of speech, intelligibility, articulation, and prosody but especially in the morphology of the articulation muscles, diadochokinesis, the reflexes (in the mouth, larynx and pharynx). Needle EMG examination confirmed the diagnosis of mono-neuropathy of left hypoglossal nerve. The study confirms the fact that neuroborreliosis may evoke chronic consequences.- - - - - - - - - - ranking = 135.26948147875keywords = muscle (Clic here for more details about this article) |
| | Next -> |