A patient with cluster type headaches demonstrated bilateral and alternating ocular sympathetic dysfunction during a spontaneous as well as a nitroglycerin-induced attack. Biochemical evaluation revealed postganglionic pupillary dysfunction on the symptomatic side and preganglionic pupillary dysfunction contralaterally. These findings defy a simple explanation regarding a central or peripheral origin of ...

Moebius syndrome is characterized by congenital paralysis, usually bilateral, of the lateral rectus muscles and the muscles of facial expression. Esotropia is common in these patients. Few reports exist of the surgical results after treatment of strabismus in such patients. We report the case of a 19-month-old white boy with ...

An 85-year-old man developed in the last 5 years three attacks on Ménière's syndrome associated with facial paralysis. The syndrome could be interpreted as a transient ischemic attack in the territory of the anterior inferior cerebellar artery. An alternative hypothesis could be to admit a Ménière's disease with compression of ...

The authors present two cases of bilateral subclavian steal syndrome, a rare condition that does not commonly cause neurovascular symptoms. Lateralizing hemispheric events occur usually with carotid lesions. Vertebral-basilar insufficiency is three times more common in bilateral than in unilateral subclavian steal syndrome. Arm-exercise-induced brain-stem dysfunction is rare, and is ...

This article remarks on the possibility of recontouring the face by working on the facial skeleton with the sole purpose of softening the facial appearance. The author describes a one-step surgical procedure performed on a 38-year-old man who had serious social problems because of his aggressive and threatening facial appearance ...

A patient had bilateral macular coloboma with aggregations of pigment clumps located perivascularly, predominantly paravenously, and in other parts of the retina. The Toxoplasma IgG antibody was negative. The diagnosis of bilateral macular coloboma with pigmented paravenous retinochoroidal atrophy was made and seemed to be a developmental abnormality in origin.

A 74-year-old man with an occult carcinoma of the colon developed pigmentation of the mouth and penis typical of the Peutz-Jeghers syndrome as the first manifestation of bilateral diffuse uveal melanocytic proliferation. The simultaneous appearance of extraocular pigmented lesion and those in the uveal tract of both eyes of this ...

A family with three generations of male-to-male transmission of a rare syndrome is presented. The syndrome includes bilateral cervical branchial sinuses, bilateral preauricular sinuses, bilateral malformed auricles and bilateral hearing impairment. Two important aspects of this syndrome are discussed. First, male-to-male transmission rules out a sex-linked mode of inheritance. Second, ...

The treatment of nonsyndromic and syndromic bilateral coronal synostosis has been outlined. Essential features of this individualized approach include technique modification in bone remodeling and fixation according to the age of the patient as well as additional emphasis on elongating the anterior cranial base or periorbital segment in patients with ...

Moebius syndrome (MS) is described in an infant with central hypoventilation and brainstem calcification. The patient had limb defects and bilateral paralysis of the 6th, 7th, 9th, 10th, and 12th cranial nerves. Mechanical ventilation was continued from birth because of shallow spontaneous respiration. Computed tomography revealed brainstem atrophy and four ...

This case report describes 99mTc(V)-dimercaptosuccinic acid (DMSA) accumulation in a pheochromocytoma in a patient with Sipple's syndrome. Scintigraphy with 99mTc(V)-DMSA demonstrated uptake in medullary carcinoma of the thyroid gland (MCT). Iodine-131 metaiodobenzylguanidine (MIBG) scintigraphy showed the bilateral pheochromocytomas but did not demonstrate uptake in the MCT.

A case is reported of a 5-month-old girl with Hallermann-Streiff syndrome who was evaluated for possible premature closure of the cranial sutures. Skull radiographs revealed numerous Wormian bones along sutures in the parietal skull bilaterally. Hallermann-Streiff syndrome is added to the list of diseases in which extensive Wormian bones can ...

In six patients with ocular Lyme borreliosis, bilateral granulomatous iridocyclitis and vitritis were present in five. One of these five also had bilateral optic neuritis. Another patient developed combined trochlear and facial nerve palsies. A syndrome resembling pars planitis with atypical features such as granulomatous keratic precipitates and posterior synechiae ...

The embryonic chick face is composed of a series of facial primordia, epithelium-covered buds of mesenchyme, which surround the presumptive mouth. The protruding adult upper beak containing the prenasal cartilage is formed from the frontonasal mass, the paired maxillary primordia form the sides of the face, while the lower beak ...

Recurrent facial paralysis (RFP) is a rare disorder that in some individuals may lead to worsening sequelae. Melkersson-Rosenthal syndrome is a variant of RFP that is associated with recurrent facial edema. In the past, decompression of the mastoid segment of the facial nerve has not been successful in preventing recurrences. ...

A manic-like state occurred in a 44-year-old right-handed woman with bilateral orbitofrontal and right temporoparietal traumatic contusions. In a brief trial, we assessed the effect of clonidine, carbamazepine, dopa therapy, and placebo on manic symptoms and cognitive functions. Clonidine rapidly reversed the manic syndrome. The patient's behavior did not change ...

Recent sonographic studies have demonstrated enlarged choroid plexuses to be an abnormality associated with myelomeningocele. We report a patient with hydrocephalus and myelomeningocele who had huge bilateral enlargement of the choroid plexuses which were large enough to occlude the foramen of Monro of the shunted and collapsed ventricle. It produced ...

A patient with Balint's syndrome caused by bilateral parieto-occipital lesions lost spontaneous blinking, suggesting that humans, like nonhuman primates, have parietal lobe neurons that are important for blinking. Although the functions of spontaneous blinking are not known, they may help initiate some saccades and, like saccades, be involved in the ...

Two young adults, aged 24 and 31, had a long history of small, poorly reactive pupils. There was no history of large pupils, and a review of old photographs confirmed 10 and 5 years, respectively, of miosis. Both were found to have bilateral tonic pupils that were supersensitive to diluted ...

Foix-Chavany-Marie syndrome (FCMS), or faciopharyngoglossomasticatory diplegia, is an uncommon syndrome that can result from vascular or developmental lesions of the anterior opercula bilaterally. We report the first pathological documentation of the developmental form of this disorder. Pathological examination revealed bilateral failure of opercular closure, opercular polymicrogyria, periventricular gray-matter heterotopias, and ...

Testicular tissues of 22 buffalo bulls (Bubalus bubalis) which suffered from three types of infertility were examined histologically. Nine bulls with no sexual libido showed underdeveloped seminiferous tubules; five of them also had various forms of germinal tissue hypoplasia (bilaterally complete, partial or incomplete) and in the other four the ...

Bilateral anterior opercular syndrome is clinically characterized by facio-pharyngo-glossomasticatory diplegia due to bilateral opercular lesions. Reported cases of the syndrome have been relatively rare probably because of difficulty in determining opercular damage on clinical symptoms alone. We report a case of this syndrome in whom bilateral opercular damage could be ...

A patient with a permanent tracheostomy was seen and noted to have bilateral abductor vocal cord paralysis. He was being investigated neurologically and was found to have several types of autonomic failure, specifically including postural hypotension. It was felt that he had Shy-Drager syndrome. A review of the literature showed ...

Divergence paralysis is a clinical syndrome that is characterized by esotropia and diplopia at distance fixation and fusion at near fixation. This syndrome is thought to result from a lesion in the brain stem, probably in the midbrain or in the pontine region. This paper reports a case in which ...

Recurrent facial paralysis is an infrequent problem for the otolaryngologist. This paralysis may be associated with the Melkersson-Rosenthal syndrome, a triad of recurrent facial paralysis, relapsing facial edema, and associated fissured tongue. Most patients do not have the accompanying stigmata of this syndrome. This paralysis may occur unilaterally or bilaterally. ...

A 27 year old woman with mitral valve prolapse presented with somnolence, bilateral Babinski signs, and grasp reflexes. As somnolence cleared, vertical gaze palsy and Korsakoffian memory deficit were apparent. Initial CT scan was normal, but NMR scan 24 hours after the onset of symptoms revealed prolonged T2 relaxation in ...

A patient who had selective paralysis of downward gaze caused by bilateral lesions of the dorsolateral mesencephalic periaqueductal gray (PAG) matter is reported. Her necropsy findings differed from all previous reports of the syndrome, in that regions of the mesencephalon that have been considered as critical for executing downward gaze ...

A ten-year-old autistic boy with high myopia and very poor vision presented a bilateral anomaly of the disc, diagnosed as morning glory syndrome. Further investigation showed an absence of the corpus callosum and probably herniation of an encephalocele into the third ventricle. The association of bilateral morning glory syndrome and ...

A patient with a classical locked-in syndrome is described, in whom bilateral ptosis was observed on the 2nd day. On the 4th day, ophthalmoplegia was complete with the exception of a voluntary downward movement of the right eye. The syndrome can be explained by the association of bilateral horizontal pontine ...

We report on a father and son who have telecanthus, hypertelorism, strabismus, and pes cavus. In addition, the son has hypospadias, bilateral inguinal hernia, clinodactyly and camptodactyly of fingers bilaterally, a small tissue mass on the tip of his nose, and radiographic findings including flared metaphyses of long bones and ...

A 9-year-old girl with bilateral Morning Glory syndrome developed bilateral bullous retinal detachment. There was a small break on the temporal margin of the disc in the left eye. The retinal detachment in this eye was successfully treated by drainage of the subretinal fluid, vitrectomy, intravitreal air injection, and photocoagulation ...

The authors describe a patient who suffered two successive, right and left, strokes that caused bilateral rolandic operculum damage. The clinical picture was characterized by selective impairment of volitional facio-pharyngo-glosso-masticatory movements with sparing of automatic and reflex motor activity (Foix-Chavany-Marie syndrome). Though completely speechless, the patient was not aphasic. This ...

A syndrome similar to gangrenous ergotism was seen in 2 Friesian heifers grazing meadow hay containing perennial rye grass seed heads parasited by an ergot presumed to be Claviceps purpurea. Clinical signs were bilateral hind limb lameness and gangrene. There was angiographic evidence of vasoconstriction in the lower hind limbs ...

A 48-year-old man presented with signs of pseudobulbar palsy. On computed tomography (CT) he was found to have extensive tissue loss in the region of the sylvian fissure and insula bilaterally. This appearance on CT represents the radiological correlate to anatomic descriptions of the operculum syndrome, and is useful in ...

A patient with lifelong, bilateral horizontal gaze palsies is presented. The anatomical findings of bilateral absence of the abducens nerve as it affected the brainstem, the course of the cranial nerves to the extraocular muscles, and muscle innervation are described. The possible relationship of these findings to Duane's syndrome and ...

In a prospective study of the ocular manifestations of Kawasaki's disease (mucocutaneous lymph node syndrome) in 18 children (11 boys and seven girls, ranging in age from 5 months to 9 years), we found bilateral injection of the bulbar conjunctiva in 16, bilateral iridocyclitis in 14, superficial punctate keratitis in ...

The triad of meningioma of the falx cerebri, bilateral optic atrophy, and erosion of the clinoids appeared in a 33-year-old woman. The optic atrophy was the initial sign of the triad. The syndrome may be the result of several causes, but an elevation of intracranial pressure may be the common ...

Symmetry in severe unilateral ptosis, the jaw-winking syndrome, and ptosis with aberrant third-nerve regeneration is best achieved with bilateral frontalis suspension. However, the levator function needs to be removed in the normal side with unilateral ptosis and bilaterally with the paradoxically innervated levator. Levator transposition to the arcus marginalis is ...

We have reported a sibship in which all three males had azoospermia and a normal 46,XY karyotype. The mechanisms for the production of azoospermia were Kallmann's syndrome and bilateral agenesis of the globus major (head of the epididymis) in the proband and bilateral agenesis of the vasa deferentia and seminal ...

The differential diagnosis in 170 patients between birth and 18 years of age is reviewed. There are a number of obvious physical findings and historical features that allow one to make a diagnosis rather quickly. Pain, vesicles, a red pinna, vertigo, and sensorineural hearing loss suggest herpes zoster oticus. Slow ...

The clinical syndrome of a 65-year-old patient with a slit-shaped right-sided meso-diencephalic lesion was analysed. A cerebellar syndrome with limb-kinetic ataxia, intention tremor and hypotonicity in all extremities as well as ataxic dysarthria was found. The disruption of the two cerebello-(rubro)-thalamic pathways probably explained the signs of bilateral cerebellar dysfunction. ...

Upper lid and vertical eye movements are usually preserved in patients with the "locked-in" syndrome, a condition manifested by anarthria, spastic quadriparesis, and intact mental function, and caused by lesions in the ventral portion of the pons. We describe a presumed mesencephalic "locked-in" syndrome in a patient who had total ...

The authors report a case of olivopontocerebellar atrophy (OPCA) with velopharyngolaryngeal paralysis. The cerebellar syndrome appeared in a 66 year-old woman and ran its course until her death at 75. The velopharyngolaryngeal paralysis occured two years after the beginning of the cerebellar symtomatology and was limited for 6 months to ...

We report four patients with paralysis of downward gaze but with intact upward gaze, including one with detailed clinico-pathological studies and another with a focal computerized axial tomographic (CT) scan abnormality confirming the presence of bilateral lesions of the dorsomedial red nucleus, including the fasciculus retroflexus. It is suggested that ...

A 28 year old female with Melkersson-Rosenthal syndrome developed an acute, peripheral facial paralysis following mucosal biopsy of the ipsilateral upper lip. The sole, previous facial palsy had occurred 24 years earlier. Immunological and virological studies were non-contributory. The occurrence of facial palsy secondary to regional trauma is briefly reviewed, ...

A 65-year-old female patient with Paget's disease displayed dementia, a bilateral Parkinsonian syndrome, gait ataxia, bilateral grasp reflex of the feet, urinary incontinence and a left hemicerebellar syndrome. She had a marked basilar impression; the radionuclide cisternogram was suggestive of a communicating hydrocephalus with ventricular dilatation. After a shunting procedure ...

Two cases with bilateral Duane's Syndrome are presented. Both showed immediate large angle exotropia following bilateral medial rectus recession for esotropia. In both cases, the forced duction test was then found to be positive in adduction and negative after disinsertion of the lateral rectus. It is suggested that both the ...

A 5-year-old deaf-mute girl was shown on petrous bone tomography to have bilateral enlarged vestibules with assimilation of the lateral semicircular cannals, bilateral absence of the cochlea, aplasia of the left internal auditory canal with only the facial nerve compartment being present, and hypoplasia of the right internal auditory canal. ...

A syndrome characterised by bilateral paralysis and associated with degenerative changes in skeletal musculature, has been noted in adult breeder hens, and their progeny broilers, in a commercial poultry organisation in Queensland. Paralysis occurred between the ages of 24 to 63 weeks in broiler breeder hens and at approximately 6 ...

Ten survivors of sporadic bilateral retinoblastoma had 14 offspring, of whom eight were affected, seven of them in both eyes. Other reports from the literature raise the total of similar unselected cases to 19 survivors with a total of 39 offspring, of whom 17 were affected in both eyes and ...