1/100. Disseminated histoplasmosis causing reversible gaze palsy and optic neuropathy.Subacute disseminated histoplasmosis is an uncommon entity. Typical neuro-ophthalmologic manifestations are usually secondary to histoplasmomas or encephalitis. A 45-year-old man noted blurred vision while receiving empiric antituberculosis therapy for fever and diffuse granulomatous disease of unknown origin. Vertical-gaze palsy, right horizontal-gaze paresis, and mild right optic neuropathy were found on neuro-ophthalmologic examination. Further questioning revealed a history of frequent contact with fighting cocks from south america. Magnetic resonance images were consistent with multiple hemorrhagic infarcts, areas of inflammation, or both, and cerebral angiography showed changes consistent with vasculitis. A previously obtained biopsy specimen from the duodenum was restained and found to be positive for fungal elements. serum antigen titers for histoplasma capsulatum demonstrated evidence of active infection. This case is a rare example of a supranuclear ocular motility disturbance and optic neuropathy secondary to an occlusive vascular process in a patient with subacute disseminated histoplasmosis.- - - - - - - - - - ranking = 1keywords = neuropathy (Clic here for more details about this article) |
2/100. Granulomatous hypophysitis and bilateral optic neuropathy.A 53-year-old woman with symptoms of hypopituitarism and ophthalmoplegia was diagnosed as having idiopathic granulomatous hypophysitis and later developed bilateral optic neuritis. She responded well to steroid treatment. Granulomatous hypophysitis is a rare entity, and this is the first reported case associated with optic neuritis.- - - - - - - - - - ranking = 0.66666666666667keywords = neuropathy (Clic here for more details about this article) |
3/100. ophthalmoplegic migraine: a recurrent demyelinating neuropathy?The demonstration by magnetic resonance imaging (MRI) scanning of thickening and enhancement of the cisternal part of the oculomotor nerve in patients diagnosed as "ophthalmoplegic migraine" prompts reconsideration of this uncommon disorder. The case histories of five patients, three male and two female, varying in age from 6 to 30 years, are presented here. Recurrent painful ophthalmoplegia started in infancy in two cases, childhood in two instances and adult life in one. One child had his first attacks at 3, 5 and 12 months of age, on each occasion 10 days after an injection of triple vaccine. The possibility of this condition being a recurrent demyelinating neuropathy is considered and its possible relationship to migraine explored.- - - - - - - - - - ranking = 0.83333333333333keywords = neuropathy (Clic here for more details about this article) |
4/100. Congenital fibrosis of the extraocular muscles associated with cortical dysplasia and maldevelopment of the basal ganglia.BACKGROUND: Congenital fibrosis of the extraocular muscles (CFEOM) is a rare condition that has been traditionally regarded as a primary eye muscle disease. Recent studies, however, suggest that CFEOM may be the result of a primary neuropathy with secondary myopathic changes. PURPOSE: To describe a previously unrecognized association between congenital fibrosis of the extraocular muscles and structural abnormalities of the brain. DESIGN: Small case series. methods: Detailed clinical examinations and neuroradiologic studies were performed on the three affected family members. In addition, genetic analysis of the family was performed. RESULTS: The three affected family members, mother and two children, have the ocular features of 'classic' congenital fibrosis of the extraocular muscles. All showed dilation of the left lateral ventricle secondary to hypoplasia of the body and tail of the ipsilateral caudate nucleus. There was fusion of an enlarged caudate nucleus head with the underlying putamen. Both children showed widespread bilateral cortical dysplasia. Genetic analysis of the family was inconclusive but consistent with linkage to the CFEOM1 locus on chromosome 12. Chromosomal analysis of the affected individuals did not show evidence of a deletion of chromosome 12 and haplotype analysis was not suggestive of a microdeletion. CONCLUSIONS: Cerebral cortical and basal ganglia maldevelopment can be found in individuals with CFEOM. This suggests that neuroimaging should be considered in the initial diagnostic evaluation of these patients, particularly if there is developmental delay.- - - - - - - - - - ranking = 0.16666666666667keywords = neuropathy (Clic here for more details about this article) |
5/100. Three patients with ophthalmoplegia associated with campylobacter jejuni.Cranial polyneuropathy is idiopathic in most patients. Idiopathic cranial polyneuropathy is an acute postinfectious syndrome, along with guillain-barre syndrome and miller fisher syndrome, in which the common preceding pathogen is campylobacter jejuni. serum anti-GQ1b antibodies are elevated in miller fisher syndrome and guillain-barre syndrome with ophthalmoplegia. Three patients with idiopathic cranial polyneuropathy with predominant ocular involvement are presented. C. jejuni isolated from stool specimens belonged to Penner serotypes O:4, O:23, and O:33. serum anti-GQ1b antibodies were elevated in all patients but demonstrated rapid reduction concomitant with clinical recovery. All patients recovered completely. Because both preceding C. jejuni infection and elevated anti-GQ1b antibodies decreasing with time were seen in all patients, the pathogenesis of idiopathic cranial polyneuropathy with ophthalmoplegia may be similar to that of miller fisher syndrome.- - - - - - - - - - ranking = 0.66666666666667keywords = neuropathy (Clic here for more details about this article) |
6/100. Neuro-ophthalmic sarcoidosis.sarcoidosis is a multisystem disorder in which ocular involvement occurs in about one-quarter and neurosarcoidosis in 7 per cent of patients. When the retina is involved, the reported incidence of central nervous system sarcoidosis is 37 per cent. The patient described had a transient papular eruption of the legs, bilateral hilar lymphadenopathy, polyarthralgia with knee effusions, and bilateral facial and peripheral neuropathy. Ocular involvement was characterized by anterior uveitis (in the initial stages), vitreous flare, bilateral disc oedema, macular oedema, streak haemorrhages, peripheral periphlebitis, nerve fibre bundle defects, and candle-wax spots. fluorescein angiography showed no fluorescence of the candle-wax spots nor of the adjacent vessels. However, there was hyperfluorescence of two retinal lesions. This patient had unilateral internal ophthalmoplegia, only three cases of which have been reported in the literature. Her health was restored by heavy, prolonged corticosteroid therapy. Her family history revealed that an uncle died of sarcoidosis complicated by cryptococcal meningitis. The literature on retinopathy in sarcoidosis is reviewed and the lesions noted in the posterior segment are listed.- - - - - - - - - - ranking = 0.16666666666667keywords = neuropathy (Clic here for more details about this article) |
7/100. Orbital apex syndrome: a rare presentation of extramedullary hematopoiesis: case report and review of literature.We describe a case of compressive neuropathy in the orbital apex due to extramedullary hematopoiesis (EMH). A 64-year-old man with polycythemia Rubra Vera developed unilateral visual loss, proptosis, complete ophthalmoplegia, and facial paresis. bone marrow biopsy showed myelofibrosis. magnetic resonance imaging demonstrated enhancement at the orbital apex and subtle optic canal narrowing. decompression of the optic nerve with biopsy of surrounding bone showed EMH. The patient received a course of radiation without benefit. We suggest including the diagnosis of EMH of the orbital apex bones in the differential diagnosis of patients with myeloproliferative disorders who develop an orbital apex syndrome.- - - - - - - - - - ranking = 0.16666666666667keywords = neuropathy (Clic here for more details about this article) |
8/100. Rare actinobacillus infection of the cavernous sinus causing painful ophthalmoplegia: case report.OBJECTIVE AND IMPORTANCE: actinobacillus actinomycetemcomitans is a gram-negative coccobacillus that is known to cause a wide array of clinical infections in debilitated patients, including periodontal disease, soft tissue abscess, pleural empyema, endocarditis, cerebral abscess, and meningitis. We report a rare A. actinomycetemcomitans cavernous sinus abscess that caused painful ophthalmoplegia in a healthy man. CLINICAL PRESENTATION: A 34-year-old man presented with a 3-month history of acute onset of left-sided retro-orbital pain that progressed to a complete left Cranial Nerve VI palsy and Cranial Nerve V1 and V2 hypesthesia. magnetic resonance imaging revealed the presence of an enhancing lesion in the left cavernous sinus, which encased and narrowed the ipsilateral intracavernous segment of the internal carotid artery. Routine blood and cerebrospinal fluid analyses were normal. tolosa-hunt syndrome was suspected, and the patient was treated with high-dose corticosteroids. An open biopsy was performed after failed prolonged corticosteroid therapy. INTERVENTION: A left frontotemporal craniotomy and cavernous sinus exploration through an interdural approach were performed. A soft reddish mass was found in the cavernous sinus around Cranial Nerve V1 and V2. Multiple biopsies were obtained. Pathological analysis revealed a purulent infection containing multiple gram-negative coccobacilli. The patient's pain improved immediately, and cranial neuropathy resolved during the next several weeks. After cultures demonstrated growth of A. actinomycetemcomitans, a regimen of orally administered amoxicillin and metronidazole was initiated. Eight months after surgery, the patient was free of symptoms and a repeat magnetic resonance imaging scan was normal. CONCLUSION: We present a rare bacterial abscess of the cavernous sinus causing painful ophthalmoplegia. In patients who present with presumed tolosa-hunt syndrome and do not improve with prolonged high-dose corticosteroid therapy, an open biopsy is recommended to exclude a bacterial infection.- - - - - - - - - - ranking = 0.16666666666667keywords = neuropathy (Clic here for more details about this article) |
9/100. Isolated acquired unilateral horizontal gaze paresis from a putative lesion of the abducens nucleus.In three patients, acute horizontal gaze pareses developed that could not be overcome with the oculocephalic maneuver, indicating a putative lesion of the ipsilateral abducens nerve nucleus. None of the patients had a facial nerve paresis or evidence of a trigeminal sensory neuropathy. Although most lesions that affect the abducens nerve nucleus also damage the ipsilateral fasciculus of the facial nerve, small lesions in this region can produce an isolated horizontal gaze paresis.- - - - - - - - - - ranking = 0.16666666666667keywords = neuropathy (Clic here for more details about this article) |
10/100. Polyneuritis cranialis with contrast enhancement of cranial nerves on magnetic resonance imaging.The disorder of multiple cranial nerve palsies without spinal cord involvement is referred to as polyneuritis cranialis (PC) and is rare. It is thought to be an acute post-infective polyneuropathy or a variant of guillain-barre syndrome. Electrophysiological evidence of demyelination has been reported, but no radiological abnormalities of the affected cranial nerves have been noted. We report a case of PC where contrast enhanced magnetic resonance imaging (MRI) showed enhancement of the peripheral segments of the oculomotor and abducens nerves. This case illustrates the utility of MRI in the assessment of cranial nerve palsies.- - - - - - - - - - ranking = 0.16666666666667keywords = neuropathy (Clic here for more details about this article) |
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