1/9. Focal myoclonus-dystonia of the leg secondary to a lesion of the posterolateral putamen: clinical and neurophysiological features.We report on a patient with spontaneous and stimulus-sensitive myoclonic jerks and dystonia of the right leg that had been present since infancy. magnetic resonance imaging showed a linear area of gliosis confined to the left posterolateral putamen. This is the first report of focal myoclonus-dystonia of the lower limb secondary to a putaminal lesion.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
2/9. Hyperekplexia and sudden neonatal death.Fifteen patients with hyperekplexia were identified in 3 families; diagnostic clinical characteristics were defined which allowed for early recognition and treatment. During the first 24 hours of life, spontaneous apnea and sluggish feeding effort were observed. After the first 24 hours, surviving infants exhibited the hyperekplexic startle response to nose tapping. This startle response is characterized by sudden muscular rigidity, feeding-induced oropharyngeal incoordination, and poor air exchange often with apnea, persisting with repetitive nose tapping. Untreated infants experienced recurring apnea until 1 year of age. Three of 15 patients died unexpectedly during the neonatal period. patients treated with clonazepam (0.1-0.2 mg/kg/day) had no serious apneic episodes and startle reflexes were diminished. The pathophysiologic mechanism for hyperekplexia remains obscure. Electroencephalographic studies were consistently normal. The response to and tolerance of benzodiazepines are striking in newborns and infants and suggest an aberrant central nervous system reflex as the etiology; therefore, hyperekplexia should be considered in the evaluation of neonates and infants with apnea, aspiration pneumonia, episodic muscular rigidity, hyperexcitability, and near-miss sudden infant death syndrome. The need for immediate monitoring of at-risk infants, observation for signs of hyperekplexia, and initiation of clonazepam in these patients are emphasized. Hyperekplexic startle response to nose tapping should be included in the routine examination of all newborns.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
3/9. acetazolamide in the treatment of abnormal oculovestibular response.We treated seven patients with incapacitating vertigo elicited by walking down a grocery store aisle or driving a car. Results of neurologic, neuro-ophthalmic, and neuroradiologic examinations were normal. Episodic vertigo secondary to an abnormal oculovestibular response was diagnosed. Each patient was given a trial of 250 to 500 mg of acetazolamide daily. Symptoms resolved completely in four patients, two patients had near resolution of symptoms, and one patient had no relief. Carbonic anhydrase activity has been demonstrated in the inner ear, and acetazolamide has been shown to affect the ion balance of the inner ear fluids.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
4/9. spasm of the near reflex associated with cerebrovascular accident.spasm of the near reflex most often has a functional basis, but may be associated with organic disease. This case report describes a patient with a progressive 11-year history of spasm of the near reflex associated with cerebrovascular accidents and reviews the differential diagnosis, management and aetiology of this disease.- - - - - - - - - - ranking = 6keywords = near (Clic here for more details about this article) |
5/9. spasm of the near reflex mimicking myasthenia gravis.spasm of the near reflex and myasthenia gravis both may present as isolated abduction deficits, either unilaterally or bilaterally. Although they each have been mistaken for other more serious causes of neurologic dysfunction, convergence spasm has not been reported previously as mimicking myasthenia gravis. Two cases of convergence spasm were diagnosed initially as myasthenia gravis with positive Tensilon tests. The cases emphasize the need to consider convergence spasm in the diagnosis of any patient with isolated abduction deficits, as well as to suggest that double-blinded Tensilon tests may be unnecessary and possibly misleading.- - - - - - - - - - ranking = 5keywords = near (Clic here for more details about this article) |
6/9. Abnormal oculovestibular response.In normal individuals, the visual and vestibular systems interact through a common subcortical center located near the vestibular nuclei. When the patient is healthy, this interaction allows appropriate integration of what might otherwise be conflicting information regarding environmental motion and moving within the environment. In patients with active peripheral vestibular lesions, such as those seen in Meniere's disease, this delicate interaction loses effectiveness. In such patients, optokinetic stimuli are capable of precipitating episodes of incapacitating vertigo with its associated vegetative symptoms (abnormal oculovestibular response--AOVR). Since the brainstem is unable to accommodate for active peripheral lesions, suffering may be prolonged for many years. Vestibular nerve section, however, converts this active lesion to a static peripheral lesion, allowing for brainstem compensation and cessation of optokinetic-induced vertiginous symptoms.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |
7/9. spasm of the near reflex associated with organic disease.spasm of the near reflex is characterized by transient attacks of convergence, accommodation, and miosis. It is usually observed in young individuals and considered functional. We studied seven patients with spasm of the near reflex who had associated neurologic disorders or head trauma. Two patients had posterior fossa abnormalities (cerebellar tumor, arnold-chiari malformation), two patients had pituitary tumors, one patient had a vestibulopathy, and two patients had a history of antecedent head trauma.- - - - - - - - - - ranking = 6keywords = near (Clic here for more details about this article) |
8/9. paralysis of accommodation in infectious mononucleosis.A case report of a 22-year-old patient with accommodative paralysis is presented including (1) the five-year history beginning with infectious mononucleosis; (2) recent clinical examination showing accomodative paralysis and reduced pupilary responses to light and near; (3) objective recordings confirming both the absence of any accommodation and the presence of pupillary responses to monocular and binocular near stimuli and to light, the latter with pupillary escape; and finally (4) neuropharmacological tests showing 7-diopter accommodative responses to pilocarpine (an acetylcholine substitute acting directly on the ciliary muscle receptor sites) and absent responses to demecarium bromide (a cholinesterase blocking agent which potentiates neurally released acetylcholine). infectious mononucleosis includes ocular signs and symptoms. In young persons with accommodative difficulties, infectious mononucleosis should be suspected.- - - - - - - - - - ranking = 2keywords = near (Clic here for more details about this article) |
9/9. Tonic pupils with acute ophthalmoplegic polyneuritis.Two patients with the symmetrical ophthalmoplegic variant of acute idiopathic polyneuritis (Fisher's syndrome) developed bilateral dilated pupils with cholinergic supersensitivity and light-near dissociation (tonic pupils), indicating involvement of the postganglionic parasympathetic neuron within the orbit. Supranuclear eye movement dysfunction also was present, indicated by relative levator sparing in both patients and by preservation of Bell's phenomenon in 1 patient despite paralysis of voluntary upward gaze. The lesions that produce the eye signs of ophthalmoplegic polyneuritis are not necessarily restricted to the cranial nerve trunks. The present cases support a relationship between the ophthalmoplegic variant of the guillain-barre syndrome and acute postinfectious encephalomyelitis (brainstem encephalitis) on the one hand, and idiopathic autonomic neuropathies such as the Holmes-adie syndrome and pandysautonomia on the other.- - - - - - - - - - ranking = 1keywords = near (Clic here for more details about this article) |