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PubMed1976

[Examination of retinal correspondence with minimal dissociation (author's transl)].

With the phase difference haploscope the incidence of anomalous retinal correspondence in patients with convergent strabismus was examined with respect to the degree of deviation, the duration of squint and the fixation pattern of the deviated eye. Anomalous correspondence was found more often when small angles were present, in older patients and in cases with excentric fixation. Even when the deviation exceeded 19 degrees and the fixation was central, we observed harmonious anomalous correspondence in 30% of cases, The evaluation of the correspondence in children with esotropia and central fixation by means of paradoxic diplopia after correction of the deviation with prisms revealed harmonious anomalous correspondence more often than with the phase difference haploscope, although the conditions of casual seeing are changed in this test. We have attempted to ascribe these findings to simultaneous stimulation of the central retinal areas of both eyes, the nearly unlimited visual field and the short binocular exposure of the pictures in our method. Finally we described our first experiences with a modified Kongruenztest. With this simple technic it is possible to examine the state of correspondence under conditions of casual seeing.

Adolescent↗
PubMedDate not supplied

Repair of zygomaticomalar complex fractures. The Swiss method.

The zygomaticomalar complex fracture, also known as the trimalar, tripod, or tetrapod fracture, is a fracture commonly encountered by facial plastic surgeons. Failure to correctly reduce these fractures can lead to facial asymmetry, trismus, diplopia, enophthalmos, and parasthesias. Approaches to the zygomaticomalar complex have been well documented in the literature and include the transconjunctival lateral canthotomy, subciliary blepharoplasty, brow, gingivobuccal, and coronal incisions, or some combination thereof. Fracture fixation is accomplished by the internal placement of miniplates and microplates across properly reduced fracture lines to resist torsional rotation and masticatory distraction. The method of fracture reduction and fixation employed at the University Hospital of Bern, Switzerland, shares much with previously published and accepted methods of medial and lateral buttress reconstruction, but it introduces several original concepts in an attempt to maximize functional and aesthetic outcomes.

Bone Plates↗
PubMed1979

Pituitary pseudotumor. Mimicry of recurrent prolactinoma by a chronic intrasellar hematoma.

In the increasing cohort of patients being observed after pituitary microneurosurgery for prolactin-secreting adenomas, tumor recurrence remains a concern. We describe a patient with a prolactinoma treated by transsphenoidal pituitary surgery who resumed cyclic menses for one year postoperatively before manifesting progressive headaches, intermittent diplopia, secondary amenorrhea, and anterior pituitary failure. Invasive and noninvasive neuroradiologic procedures suggested recurrent tumor with suprasellar extension. However, reoperation of the pituitary disclosed a chronic intrasellar hematoma expanding under increased pressure within the enclosed sellar space. We discuss a putative mechanism for the pathophysiology of this syndrome, and tentatively designate it "pituitary pseudotumor". Recognition of this entity is important not only because reoperation is required for accurate diagnosis, but also because treatment with radiotherapy or bromocriptine mesylate for presumptive tumor progression or recurrence would be ineffective.

Chronic Disease↗
PubMed1986

Lateropulsion and upbeat nystagmus are manifestations of central vestibular dysfunction.

An elderly man presented with acute onset of gait instability, characterized by leaning to the left while walking, vertigo, diplopia, and transient facial numbness. The examination was remarkable for ocular lateropulsion and primary position upbeat nystagmus. Computed tomography of the brain revealed a right-hemispheric cerebellar infarction. This report illustrates that lateropulsion can occur in cerebellar lesions, but that it may be contralateral to the cerebellar hemisphere involved. Primary position upbeat nystagmus and lateropulsion may represent forms of central vestibular dysfunction due to interruption of vestibulo-ocular pathways.

Aged↗
PubMed1987

Magnetic resonance imaging in a case of autopsy-proved adult subacute necrotizing encephalomyelopathy (Leigh's disease).

Leigh's disease, or subacute necrotizing encephalomyelopathy (SNE), in adults is rare, and its diagnosis has depended on the postmortem identification of characteristic lesions in a typical distribution. We observed an autopsy-proved case of SNE in which the diagnosis was established by the distribution and evolution of lesions documented by serial magnetic resonance imaging (MRI). A 21-year-old woman insidiously developed diplopia and gait disturbance, and subsequently deteriorated to a vegetative state over seven months. An initial MRI obtained one month after presentation showed increased signal intensity that surrounded the aqueduct of Sylvius and involved the tectum of the midbrain. Serial MRI scans showed these lesions to extend and symmetrically involve the tectum of the midbrain, caudate, putamen, globus pallidus, and substantia nigra, while sparing the mammillary bodies and red nuclei. Despite treatment with 2 g of thiamine administered intravenously daily, she continued to deteriorate and died. Results of an autopsy established the diagnosis of SNE and confirmed the MRI-identified distribution of lesions. To our knowledge, this case is the first report of MRI findings in an adult with autopsy-proved SNE, suggesting that MRI can be valuable in the early diagnosis of this disease.

Adult↗
PubMed1993

Wallenberg's lateral medullary syndrome. Clinical-magnetic resonance imaging correlations.

OBJECTIVE: To correlate clinical and radiologic findings in patients with lateral medullary infarction. DESIGN: Case series with "blinded" evaluation of brain imaging. SETTING: Hospitalized and ambulatory patients at the Neurological Institute of New York (NY). PATIENTS: Thirty-three consecutive patients with lateral medullary syndrome were evaluated by the Stroke Center between 1983 and 1989. RESULTS: Ataxia (70%), numbness either of the ipsilateral face or of the contralateral body (64%), vertigo (51%), and dysphagia (51%) were the most frequent symptoms at onset. Eleven patients had ocular symptoms (diplopia or blurred vision). Horner's syndrome was found in 91%, ipsilateral ataxia in 85%, and contralateral hypalgesia in 85%. Nystagmus (61%) and facial weakness (42%) were less frequent. Head computed tomography was abnormal only when a cerebellar infarction was present (three cases). Magnetic resonance imaging, obtained in 22 cases, was normal in two; a lateral medullary infarction alone was present in 12, and a lesion extending beyond the lateral medulla was found in eight. No correlation was noted between facial weakness or ocular symptoms and infarction extending beyond the lateral medullary region. Vertebral artery disease was confirmed by vascular imaging or insonation studies in 73% of patients. CONCLUSIONS: The triad of Horner's syndrome, ipsilateral ataxia, and contralateral hypalgesia will clinically identify patients with lateral medullary infarction. Facial weakness and ocular symptoms are frequent and do not necessarily imply that the infarction extends beyond the lateral medulla. Cerebellar infarcts only infrequently accompany lateral medullary syndrome, suggesting that most of the posterior inferior cerebellar artery territory is spared, despite the high frequency of vertebral artery occlusion.

Adult↗
PubMed1996

Rippling muscles and myasthenia gravis with rippling muscles.

OBJECTIVE: To describe a patient with rippling muscles and myasthenia gravis. DESIGN: Clinical, laboratory, electrophysiologic, and muscle biopsy data are reported. SETTING: Medical office and hospital. PATIENT: We describe a patient with rippling muscles (as seen in rippling muscle disease) and myasthenia gravis (MG) with thymoma. There was no family history of rippling muscle disease in our patient. Diplopia and other symptoms of MG were initially overshadowed by the striking rippling phenomenon. The rippling resolved when the MG became florrid. INTERVENTIONS: The patient was treated with pyridostigmine, prednisone, and plasmaphereses before removal of a thymoma. MAIN OUTCOME AND RESULTS: His MG improved with treatment and the rippling has not recurred 4 months after thymectomy. CONCLUSIONS: Rippling muscle disease is rare and usually inherited. Our patient with rippling muscles (but no family history of rippling muscle disease) and MG suggests that rippling muscles may also be triggered by an autoimmune phenomenon.

Biopsy↗
PubMed1999

Recurrent orbital myositis: report of a familial incidence.

BACKGROUND: In orbital myositis, painful diplopia develops owing to an enlargement of the extraocular muscles. Diagnosis is established based on history, clinical manifestations, and therapeutic response to steroids, with the findings of magnetic resonance imaging providing additional information. OBSERVATION: We observed a family in which 4 members had an ophthalmopathy suggestive of orbital myositis. The affected members are a sibling pair (female and male) and 2 children of the brothers of their father's father. CONCLUSION: The familial incidence suggests a potential genetic predisposition in the development of orbital myositis.

Adolescent↗
PubMed2000

Multifocal dural enhancement associated with temporal arteritis.

OBJECTIVE: To report the association of temporal arteritis and enhancement of the dura mater and temporalis muscle. DESIGN AND METHODS: A single patient with a complaint of headache and diplopia was studied. SETTING: Academic medical center. PATIENT: A 69-year-old man presented with lateral rectus weakness, temporal artery tenderness, and an erythrocyte sedimentation rate of 65 mm/h. INTERVENTION: Biopsy of temporal artery and dura mater. MAIN OUTCOME MEASURES: Brain magnetic resonance imaging and pathological findings. RESULTS: Magnetic resonance imaging of the brain showed multifocal dural enhancement and enhancement of the temporalis muscles. The temporal artery showed a necrotizing vasculitis and the dura showed perivascular inflammatory cells. CONCLUSION: It is proposed that the temporal arteritis caused the multifocal dural enhancement and temporalis muscle enhancement on magnetic resonance imaging.

Aged↗
PubMed2002

Botulinum toxin A treatment for primary hemifacial spasm: a 10-year multicenter study.

BACKGROUND: Botulinum toxin A (BTX) is the currently preferred symptomatic treatment for primary hemifacial spasm (HFS), but its long-term efficacy and safety are not known. OBJECTIVE: To assess the long-term effectiveness and safety of BTX in the treatment of primary HFS. DESIGN: Retrospective review of medical records of the 1st and 10th years of treatment. SETTING: Outpatient clinics of 4 Italian university centers in the Italian Movement Disorders Study Group. PARTICIPANTS: A series of 65 patients with primary HFS who had received BTX injections regularly for at least 10 years. MAIN OUTCOME MEASURES: Mean duration of improvement and quality of the effect induced by the preceding treatment (measured using a patient self-evaluation scale) and occurrence and duration of adverse effects in the 1st and 10th years of treatment. RESULTS: Using a mean BTX dose per treatment session similar to that used by others, we obtained a 95% response rate and an overall mean duration of improvement of 12.6 weeks during year 1. The effectiveness of BTX in relieving the symptoms of primary HFS, as measured by the response rate and average duration of improvement, remained unchanged in the 1st and 10th years. Patients needed statistically similar BTX doses in the 1st and 10th years. The rate of local adverse effects (including upper lid ptosis, facial weakness, and diplopia) diminished significantly in the 10th year of treatment. CONCLUSION: Treatment with BTX effectively induces sustained relief from symptoms of HFS in the long term, with only minimal and transient adverse reactions.

Adult↗
PubMed2002

Atypical brainstem encephalitis caused by herpes simplex virus 2.

BACKGROUND: Herpes simplex encephalitis is one of the most common and serious sporadic encephalitides of immunocompetent adults. Herpes simplex virus 2 (HSV-2) infections of the central nervous system usually manifest as subacute encephalitis, recurrent meningitis, myelitis, and forms resembling psychiatric syndromes. OBJECTIVES: To report and discuss magnetic resonance imaging (MRI) findings and clinical features in atypical brainstem encephalitis and facial palsy associated with HSV-2. SETTING: Neurology department of a tertiary referral center. PATIENT: A 37-year-old woman was admitted to the hospital with fever, diplopia, left hemiparesis, sensory change in the face and limbs, personality changes, frontal dysexecutive syndrome, and a stiff neck. Brain MRI showed multifocal high-signal intensities in the pons, midbrain, and frontal lobe white matter on T2-weighted and fluid-attenuated inversion recovery images. Cerebrospinal fluid (CSF) polymerase chain reaction (PCR) amplification analysis was positive for HSV-2. Acyclovir therapy was started, and the encephalitic symptoms disappeared with a negative conversion of HSV-2 PCR in the CSF. However, after the discontinuation of acyclovir therapy, peripheral facial palsy occurred on the left side. A possible relapse or delayed manifestation of the HSV-2 infection was suspected, and the acyclovir therapy was restarted. A complete remission was achieved 3 days after the treatment. She was discharged without any neurologic sequelae. CONCLUSIONS: We describe a patient who developed atypical encephalitis due to HSV-2 and peripheral facial palsy, which could also be related to the HSV-2. This case suggests that HSV-2 should be considered among the possible causes of atypical or brainstem encephalitis and that the PCR amplification method of the CSF can help reveal the possible cause of HSV-2.

Acyclovir↗
PubMed2004

Congenital adrenal hyperplasia and multiple sclerosis: is there an increased risk of multiple sclerosis in individuals with congenital adrenal hyperplasia?

BACKGROUND: Congenital adrenal hyperplasia (CAH) is an inherited recessive disorder of adrenal steroidogenesis, generally caused by a total or partial deficiency in 21-hydroxylase, due to a deletion of or mutations in the CYP21 gene (the gene that codes for 21-hydroxylase). Impaired cortisol biosynthesis results in corticotropin hypersecretion, which leads to overproduction of intermediate metabolites and androgens. OBJECTIVE: To describe for the first time, to our knowledge, a patient with CAH and multiple sclerosis (MS). DESIGN: Case report. PATIENT: A 22-year-old woman, diagnosed at birth as having a salt-losing 21-hydroxylase deficiency, had sudden visual loss in the right eye and pyramidal, sensory, and cerebellar signs. Repeated brain magnetic resonance images showed focal white matter lesions in periventricular areas, the corpus callosum, the cerebellum, and the brainstem. A cerebrospinal fluid examination revealed several oligoclonal bands. Thereafter, she had 2 relapses, characterized by ataxia and diplopia, and recovered after corticosteroid treatment. RESULTS: The reported case fulfills the diagnostic criteria for CAH and MS. CONCLUSIONS: Some clues suggest that the association between CAH and MS could be nonincidental: a possible MS susceptibility locus is on chromosome 6p21, on which the CYP21 gene is located; the CYP21 gene and the CYP21P pseudogene alternate in tandem with the C4 genes (the genes that code for the homonym complement protein) (C4AQ0 is particularly frequent in patients with relapsing-remitting MS); and, in previous studies, brain magnetic resonance imaging showed T2-hyperintense focal areas in the white matter of CAH patients. Our observation should alert neurologists to the presence of signs and symptoms suggestive of late-onset CAH in MS patients and, in turn, endocrinologists to the appearance of neurological signs and symptoms in CAH patients.

Adrenal Hyperplasia, Congenital↗
PubMed2004

Orbital venous malformations: current multidisciplinary treatment approach.

OBJECTIVE: To evaluate the clinical, radiological, and histopathological features, treatment, and outcome of a series of orbital venous flow malformations (OVMs) with the aim of delineating a more systematic approach for treatment. METHODS: A 38-year retrospective review of 22 patients with OVMs followed up at 1 institute. RESULTS: Eighteen of 22 patients (13 women and 9 men) showed clinical or radiological evidence of distensibility. The mean age at the initial manifestation was 28.3 and 50.7 years in patients with distensible and nondistensible OVMs, respectively. Eight patients (36.3%) had deep orbital lesions, 6 (27.3%) had superficial orbital lesions, and 8 (36.3%) had combined orbital lesions. All 3 patients with deep nondistensible OVMs had a sudden onset of proptosis and pain or diplopia secondary to thrombosis or hemorrhage. Seventeen patients required treatment. All 4 nondistensible lesions were treated by surgical excision. A variety of techniques were used to treat distensible OVMs including carbon dioxide laser ablation, percutaneous alcohol sclerotherapy, or embolization with Guglielmi detachable coils after surgical exposure and surgical excision. Of the 14 patients with follow-up, 8 had complete resolution of the signs and symptoms and 6 patients showed marked improvement following surgery. Mean follow-up was 57.8 months. CONCLUSION: If intervention is indicated, less invasive methods such as carbon dioxide laser ablation and percutaneous alcohol sclerotherapy for superficial and combined orbital lesions and endovascular treatment by Guglielmi detachable coil embolization for deep orbital lesions should be considered before proceeding with more invasive procedures.

Adolescent↗
PubMed2005

Psychological disturbance in graves ophthalmopathy.

OBJECTIVE: To study mood disturbance in Graves ophthalmopathy. METHODS: Forty-eight patients (mean age, 55 years; 40 women and 8 men) with Graves ophthalmopathy from a university-based referral center were classified into two groups, 24 with moderate/severe disease (study group) and 24 with negligible/mild disease (control group). The groups were matched with regard to demographic and medical characteristics. All participants completed a mood survey to assess differences in degree of emotional distress. MAIN OUTCOME MEASURE: The Profile of Mood States survey, a 65-item self-reported inventory designed to assess emotional distress, was the primary outcome measure. A total mood disturbance score was assigned by summing the scores derived on the 6 subscales of the survey--tension, depression, vigor, confusion, fatigue, and anger. RESULTS: Analysis of variance revealed that patients with moderate/severe Graves ophthalmopathy showed significantly greater emotional distress than patients with mild/negligible Graves ophthalmopathy on the Profile of Mood States mean total score (P<.001). Additionally, patients who had disfigurement (proptosis) as the predominant clinical feature had significantly elevated emotional distress compared with the control group (P = .01), whereas no significant difference was detected between the control group and patients with diplopia as the predominant clinical feature (P = .20). CONCLUSION: Patients with moderate to severe Graves ophthalmopathy have significant mood disturbance, especially when disfiguring signs are predominant. We propose that evaluation of the psychological burden of the disease should be considered in routine follow-up and in decisions regarding treatment.

Adult↗
PubMed1977

Multiple myeloma. Orbital involvement in a youth.

Orbital involvement by multiple myeloma is rare and affects older individuals usually. The youngest reported patient in a recent series that cites a median age of 56 years is 30 years of age. The present case involves a 19-year-old pregnant black female subject who complained initially of diplopia and whose presenting sign was proptosis. An extensive medical work-up and electron microscopic study were necessary to establish the diagnosis.

Adolescent↗
PubMed1981

Sinus involvement in inflammatory orbital pseudotumor.

Orbital pseudotumor is a difficult diagnosis to establish preoperatively. The relationship between sinus disease and orbital pseudotumor is controversial. We describe two patients with unilateral proptosis, diplopia, palpable orbital masses, ocular discomfort, and sinus problems of short duration. Echographically, both had low reflective masses in the orbit and the adjacent sinuses. Roentgenograms and echograms were interpreted as showing erosion of the bony orbital wall. A presumptive diagnosis of sinus malignant neoplasm with orbital extension was made. Sinus histopathologic examination in one case and nasal histopathologic examination in the other showed chronic inflammatory changes compatible with the diagnosis of pseudotumor. At orbitotomy, one patient had vessels communicating between the orbital and sinus lesions, and both patients had irregular pitting of the bone next to the histologically proved orbital pseudotumors. The lytic erosive changes predicted preoperatively were not present. Simultaneous orbital and sinus pseudotumors seem to be a distinct clinicopathologic entity. Those concerned with the diagnosis and management of orbital disease should be aware of this entity.

Adult↗
PubMed1981

Update of ocular reticulum cell sarcoma.

Reticulum cell sarcoma involving the vitreous and the brainstem occurred in a 45-year-old man. He initially was seen with diplopia from a partial left-sided third cranial nerve palsy, which is rare. Later, a typical uveitis developed in the right eye. An initial diagnosis of brainstem glioma, based primarily on the computed tomographic scan findings and clinical history, was ultimately proved erroneous when the correct diagnosis was shown by the results of a cytologic examination of vitreous aspirate. Excellent visual response to a moderately high oral dose of steroids occurred, which has not been usual in other reported cases. Definitive cobalt (gamma) radiation therapy (6,000 rad to the brainstem and 4,000 rad to the vitreous) has produced a one-year remission at this time.

Brain Neoplasms↗
PubMed1982

Idiopathic orbital myositis: report of six cases.

Idiopathic orbital myositis, a subgroup of inflammatory orbital pseudotumor, occurred in six patients with acute onset periorbital pain, diplopia, and in most cases, eyelid swelling. Proptosis, ductional restrictions, and responsiveness to oral prednisone administration were characteristic. Computed tomography and orbital ultrasonography showed enlargement of one or more extraocular muscles, with sparing of other orbital soft tissues. One patient had involvement of all recti muscles in both orbits. The distinction of orbital myositis from Graves' ophthalmopathy is made on clinical grounds, with acute orbital pain and corticosteroid responsiveness being uncharacteristic of the latter. The possibility of orbital myositis being an immune-mediated process is discussed.

Adult↗

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