Transient monocular blindness caused by vasospasm.
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Biomedical subjects
Publications and source records attributed to J B Selhorst.
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26 patients admitted with clinical pictures consistent with meso-diencephalic infarction were studied using computerized axial tomography, magnetic resonance imaging and angiography. All of the patients presented symptoms in two of three major categories which comprise the "top of the basilar" syndrome. Lesions in the territory of the basilar-communicating artery were demonstrated in 22 of them. The remaining patients died before adequate neuroimaging documentation was obtained. Overall prognosis appeared relatively good since 80% of the patients recovered completely or incompletely from the ictus. Meso-diencephalic infarction is a very important and not uncommon subtype of cerebrovascular disorder.
We report 3 patients with post-traumatic tremor and describe the characteristics of the tremor and accompanying neurologic signs. Radiographic and pathologic examination indicated a contralateral midbrain localization of the lesion. The tremor responded to anticholinergic or dopaminergic therapy.
We describe two patients with absolute, complete, binocular inferior altitudinal hemianopias. These altitudinal visual-field defects (AVFDs) involved both nasal and adjacent temporal quadrants and respected the horizontal meridian. The reported conditions and locations in the visual system that cause "conventional" AVFDs and their bilateral occurrence are reviewed. Division of the visual system into altitudinal halves results from the topographic arrangement of nerve fibers and their blood supply in the retina, optic nerves, and visual cortex. Lesions at these locations resulting in AVFDs are usually due to vascular occlusion or may result from a penetrating missile injury to the superior occipital lobes. It is important to emphasize that the nerve fiber layer of the retina respects the horizontal meridian only in the nasal field, not in the temporal field; therefore incomplete AVFD is more common. Because of the anastomotic blood supply of the occipital pole, only AVFDs due to occipital infarcts spare fixation. Diagnosis of retinal branch artery occlusion or anterior ischemic optic neuropathy is aided by the presence of a unilateral AVFD along with ipsilateral funduscopic abnormalities and, in most bilateral examples, by its sequential temporal development. Bilateral occipital infarcts, as in our two cases, are characterized by sudden, simultaneous onset of AVFDs, an absence of retinal, retinovascular, or optic nerve abnormality and confirmation by computed tomographic scanning.
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We describe two patients and a previously reported patient who acquired unique pendular vergence oscillations of the eyes and concurrent contractions of the masticatory muscles, i.e., oculomasticatory myorhythmia (OMM). The smooth disjunctive eye movements cycled with a frequency of 0.8 to 1.2 Hz. An analysis of peak velocities (15 to 200 degrees/sec) with respect to peak amplitudes (5 to 25 degrees) revealed dynamics characteristic of normal vergence movements. The pathological alterations resulting in pendular vergence oscillations implicate a separately functioning, physiologically normal vergence system within the brainstem. In addition to paralysis of vertical gaze, each patient also experienced progressive somnolence and intellectual deterioration. An intestinal biopsy in 1 patient established a diagnosis of Whipple's disease, which led to appropriate treatment and amelioration of the OMM. A pathological diagnosis of Whipple's disease of the central nervous system was made in the other 2 patients; results of an intestinal biopsy in one of these patients were normal. No patient had palatal myoclonus, and olivary pseudohypertrophy was not found in two autopsy examinations. Thus, OMM is a distinct movement disorder and has been recognized only in Whipple's disease. We conclude that patients with OMM should be treated presumptively for Whipple's disease of the central nervous system, even if a jejunal biopsy is normal.
Two women (26 and 40 years old) developed an unusual microangiopathy that affected the brain and retina. Psychiatric symptoms initially overshadowed the subacute features of the progressive neurologic disorder. Ophthalmoscopic findings of multifocal branch retinal artery occlusions provided clinical evidence of vasculopathy. Laboratory data did not reveal evidence of the known vasculitides, including systemic lupus erythematosus (SLE) and syphilis. Cerebral angiography suggested vasculitis in the younger patient. Brain biopsy in the older patient (after 3 months of steroid therapy) revealed a sclerosis of the small pial and cortical vessels that was consistent with a "healed" angitis. Both patients seemed to respond to steroid therapy.
Three patients with headache and increased intracranial pressure had elevated blood, serum, and adipose levels of the organochlorine insecticide chlordecone (Kepone). These patients were among 23 employees who suffered from chronic chlordecone intoxication resulting from industrial exposure. In our three patients, investigations eliminated an intracranial mass or other known causes of psuedotumor cerebri. In all three patients, the capacity for cerebrospinal fluid (CSF) absorption was assessed by graded infusions into the subarachnoid space, and was found to be impaired even when papilledema was minimal.
A patient is described with the dermatological features of blue rubber bleb nevus syndrome (BRBNS), focal seizures, and lateralized neurological signs. CAT scan demonstrated a nonenhanced density in the region of the vein of Galen. Neuropathological examination showed that this density was a clot within a vein of Galen malformation. Hemangiomas that grossly resembled the skin lesions of BRBNS were seen on the cerebral surface. Many of these hemangiomas were thrombosed and overlay patchy zones of infarction. Numerous vascular malformations of varying histological types were also found within the brain and systemic organs. Bluish, compressible, often raised hemangiomas of the skin should alert physicians to the BRBNS and the potential for vascular malformations ot occur within the brain as well as systemic organs. Diagnosis of BRBNS involving the brain may assist in interpretation of radiographic findings. The tendency of these malformations to thrombose may account for focal neurological deficits.
A patient presenting with persistent headache of vascular quality and an intermittently progressive chiasmal syndrome had a blue-domed haemorrhagic cyst beneath the right optic nerve and underwent surgical drainage with good results. As prompt surgical intervention may proivde significant return of visual function, detailed diagnostic evaluation of similar cases is recommended.
Industrial overexposure to chlordecone, an organochlorine insecticide, caused tremor in 76 of 148 exposed workers. Chlordecone was absorbed through oral, respiratory, and dermal routes, the last possibly the most significant. Epidemiology of this incident disclosed low-level, widespread environmental exposure of man to chlordecone. In 23 workers with chronic chlordecone intoxication, tremor was associated with opsoclonus, pleuritic pain and arthralgia. No seizures were reported. The site of action of chlordecone on the central nervous system is unknown. It concentrates in human adipose and hepatic tissue but is not biodegradable, either in humans or elsewhere in nature.
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Three patients with proved internal carotid artery occlusion and diseased external carotid artery or common carotid artery had amaurosis fugax. Evidence for retinal embolization through external carotid collaterals is retinal emboli and infarction after carotid artery manipulation, amaurosis during external carotid angiography, and relief of amaurosis by endarterectomy of this vessel. The abundance of external carotid collaterals, their size, and natural incidence are discussed. Reports clinically support embolization to the central retinal artery and ophthalmic artery through these collaterals.
Intermittent corectopia was recently observed in a patient with bilateral, rostral midbrain infarction. Results from neuropathologic examination disclosed isolated but intact Edinger-Westphal nuclei. An explanation of midbrain corectopia centered on segmental innervation of the pupil by the Edinger-Westphal nucleus. In the presence of a paralyzed dilator muscle, select, central inhibition of sphincter tone resulted in an oval and eccentric pupil.
Three postmenopausal women developed photoreceptor degeneration one to four months preceding or following discovery of an anaplastic tumor. Two patients had transitory visual obscurations and bizarre visual sensations. Ring scotomas progressed to severe visual field loss. Retinal arteries were markedly narrowed. Electroretinograms revealed almost total absence of response in one patient, and another complained of the recent onset of night blindness. In all three patients severe degeneration of the photoreceptor cells associated with melanophagic activity was shown histologically. In two patients neuropathologic examination from the retinal bipolar cells to the occipital cortex revealed no significant alterations.
Photoelectric eye movement recording in 9 patients with cerebellar disorders defined three features of saccadic overshoot dysmetria: (i) saccades were hypermetric and successively diminished in amplitude; (ii) saccadic initiation interval averaged 173 ms: and (iii) eye position was constant during the intersaccadic period. These characteristics indicated that the visually evoked saccades subserving foveation had increased gain, and were modelled by computer simulations using a sampled-data control model with increased feed-forward gain. Eight patients with saccadic overshoot dysmetria had cerebellar neoplasms, vermis-splitting surgical procedures and mid-line cerebellar signs. This clinical evidence suggests that vermian dysfunction is responsible for saccadic overshoot dysmetria. Normally, the cerebellar vermis appears to play an adaptive role by continuously adjusting gain of the direct visual motor pathway. When cerebellar disorder exists, adaptive gain modulation is lost, and, if gain then increases, saccadic overshoot dysmetria is a result.
A distinctive cerebellar ocular motor disorder, macrosaccadic oscillation, evolved simultaneously with an acute cerebellar syndrome in 4 patients, 2 with haemorrhagic metastatic melanoma deep in the vermis, a third with a presumed cerebellar haematoma and a fourth with focal demyelinating disease. Ocular oscillations were conjugate, horizontal, symmetrical, occurred in bursts of several seconds duration, had amplitudes of 30 degrees to 50 degrees, and were evoked whenever the patient attempted to shift visual fixation or pursue a moving target. Photo-electric recordings in one patient with tumour defined features of this disorder of saccadic eye movement: (i) oscillation was composed of saccades, (ii) frequency was 2 Hz, (iii) bursts occurred with amplitude first increasing and then decreasing, (iv) intervals between beginnings of saccades averaged 260 ms and (v) eye position did not exhibit systematic drift during the intersaccadic period. These features documented the inreased gain and instability of the visually guided saccadic system. By using increased feed-forward gain in a sampled-data control model we simulated the pattern of macrosaccadic oscillation. We belive that the acute loss of the calibrator function of the cerebellum accounts for the gain abnormality underlying macrosaccadic oscillation.
During surgical removal of a pituitary adenoma, conduction in the anterior visual pathways was monitored by continuous recording of visual evoked responses (VER). The method employed a scleral contact lens with an embedded flashing diode for delivery of visual stimuli. Evoked potentials of nearly normal latency, amplitude, and form were recorded from occipital scalp electrodes immediately after the optic nerves were decompressed. Restoration of the VER was correlated with restoration of normal vision.