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At least 1,099 records · Page 61Linked to original sources
PubMed1991

[Suprasellar tumors in patients over 70 years of age. Value of limited frontotemporal craniotomy. Visual results. Apropos of 5 cases].

The authors report their experience of the surgical approach for suprasellar tumors (Jugum or tuberculum sellae meningiomas, 3 cases, and suprasellar craniopharyngiomas, 2 cases) in over than seventy years old patients (71-78 y). A limited craniectomy was performed at the fronto-temporal junction using three adjacent burr-holes. The duramater was cut with an Y-shaped incision. Cerebrospinal fluid was sucked out through a lateral subfrontal route, allowing an easy cerebral collapse in elderly patients, which can sometimes avoid even retraction. Such an supra-orbital craniectomy allowed a subfrontal approach, opening of the sylvian fissure and control of the inter optic, inter carotido-optic and latero-carotidian spaces as well as a good access to the inter pedoncular cistern. In 4 cases the craniectomy was performed after fronto-temporal skin incision, but a trans eyebrow skin incision was used in 1 case. All patients had uneventful postoperative course. Follow-up was from 3 to 5 years. A visual improvement occurred in all cases. Benefits of this surgical method are discussed and compared with use of a bone flap in patients over than 70 years.

Aged↗
PubMed1991

Pili torti and onychodysplasia. Report of a previously undescribed hidrotic ectodermal dysplasia.

Ectodermal dysplasias are a large and heterogeneous groups of clinically and genetically distinct syndromes. We studied a family suffering from dystrophies of the distal part of the nails and trichodysplasia. Scalp, beard, pubic and axillary hair were broken off leaving a stubble 1-10 mm in length. Eyebrows, eyelashes and body hair were completely absent. Serum levels of copper and plasma levels of amino acids were within the normal range. Inheritance was autosomal recessive. Previous reports of ectodermal dysplasias and other complex syndromes with pili torti are reviewed.

Abnormalities, Multiple↗
PubMed1991

Ichthyosis, exocrine pancreatic insufficiency, impaired neutrophil chemotaxis, growth retardation, and metaphyseal dysplasia (Shwachman syndrome). Report of a case with extensive skin lesions (clinical, histological, and ultrastructural findings)

The Shwachman syndrome comprises exocrine pancreatic insufficiency, growth retardation, and bone marrow hypoplasia resulting in neutropenia. Clinical, morphological, and ultrastructural studies, as well as hair analysis, were performed in a patient with Shwachman's syndrome and severe ichthyosis. Clinical findings were lamellar ichthyosiform desquamation on the extremities. The hair was scanty and short on the scalp, in the eyelashes, and in the eyebrows. The nails were hyperkeratotic. Morphologic findings were slight, regular acanthosis and severe diffuse hyperkeratosis with variable parakeratosis. The granular layer was thickened. The papillary dermis showed very slight perivascular lymphocyte infiltration. The most prominent ultrastructural finding was the presence of solitary or multiple droplets of varying size in the cytoplasm of the keratinocytes. Hair analysis revealed no abnormalities; the cystine concentration in hair specimens was normal.

Bone Diseases, Developmental↗
PubMed1991

Eye cosmetics.

Eye cosmetics are useful to highlight and emphasize the eyes. Currently available eye cosmetics include eye shadows, eye shadow setting creams, under-eye concealers, eye-liners, mascaras, artificial eyelashes, and eyebrow pencils. Special care must be taken when patients with sensitive skin or contact lens wearers select eye cosmetics. Eye cosmetics may also be the cause of either irritant or allergic contact dermatitis, which are two causes of the upper-eyelid dermatitis syndrome.

Contact Lenses↗
PubMed1991

Trichotillomania.

Trichotillomania is a behavioral disorder characterized by the recurrent failure to resist removing one's own hair from the scalp, eyelashes, eyebrows, beard, axillary areas or pubic area. Patients report an increasing sense of tension immediately before the impulse to pull out their hair and a sense of gratification or relief during the act. On initial presentation, patients may deny that their hair loss is due to such behavior. The diagnosis of trichotillomania is excluded when there is a preexisting skin disorder or when the behavior results from underlying psychosis. Behavior modification training, psychotherapy, hypnosis and family counseling are common treatment approaches. Antidepressant medications such as amitriptyline and clomipramine are effective when depression or an obsessive-compulsive disorder coexists.

Adult↗
PubMed1990

How ancient is temporal arteritis?

Realism is one of the characteristics of Amarnian art; some details of The Harpist represented in the tomb of Pa-Aton-Em-Heb (1350 BC, 18th Dynasty) might gives clues to a diagnosis: the eyes are closed with swollen lids, and the harpist appears to stare into space; he is round shouldered with a very wasted face, his temporal ara is heightened and hollowed by a broken line joining the extremity of the eyebrow with the corner of the eye. These details are not found elsewhere. Did the harpist's blindness result from temporal arteritis associated to polymyalgia rheumatica?

Giant Cell Arteritis↗
PubMed1990

Microsurgical replantation of two totally avulsed scalps.

We describe our experience with two patients in whom total avulsion of the scalp occurred. Successful replantation was carried out by microvascular surgery, with 90 and 95% survival of the scalp. Normal hair regrowth and ability to raise the eyebrows was achieved in both cases. A single superficial temporal artery was anastomosed, providing excellent perfusion of the entire scalp. Microvascular replantation should be attempted with all scalp avulsions, regardless of appearance and mechanism of injury. The patient must be transferred to a medical center with comprehensive microvascular surgery capabilities. The avulsed scalp should be cooled to 4 degrees C immediately, but prolonged ischemia time does not preclude a successful result.

Adolescent↗
PubMed1990

Human hair morphology: a scanning electron microscopy study on a male Caucasoid and a computerized classification of regional differences.

The present study was performed to provide a better understanding of the morphological variations of mammalian hair. Terminal hair samples were obtained from different regions of the body of the same Caucasian male. All hair samples were either cleaned or treated before being examined with scanning electron microscopy. As human scalp hair grew it appeared small like lanugo hair, but the increase in diameter appeared to have been relatively rapid. As hair increased in diameter the appearance of the scales changed. Neck hair was slightly smaller in diameter than scalp hair, and axillary hair was slightly smaller in diameter than neck hair. Nostril hair was larger than scalp or axillary hair. Eyelash hair was much smaller and much shorter than eyebrow hair. Neck hair, forearm hair, and shin hair were smaller than hair from most other regions of the body. Chest hair was similar in size to scalp hair, and pubic and sideburn hair were larger than scalp hair. A morphological feature called "steak-boning" was more characteristically present in whiskers of Caucasoids than Orientals or Blacks. "Steak-boning" occurred most frequently in hair of the mustache, followed by that of the chin, sideburn, cheek and under the chin. Cut surfaces of whiskers were different for electric as compared with straightedge razors. Hair morphology varied relative to the body region. Computer analysis of resin-embedded hair made it possible to classify arm, mustache, cheek, chin, head, shin, and pubic hair, and to quantify cross-sectioned differences.

Hair↗
PubMedDate not supplied

Ocular findings in the inmates of a leprosy rehabilitation centre.

This study is aimed at presenting the profile of ocular lesions observed in the inmates of a leprosy rehabilitation centre. Corneal involvement was found to be the highest (52.7%), followed by lesions of the eyebrows (47.3%) and eyelids (30.9%), uveal tract involvement (18.1%) and chronic conjunctivitis (1.8%).

Adolescent↗
PubMed1989

[Bazex, Dupré and Christol syndrome. Apropos of a case with prolymphocytic leukemia].

We report a case of Bazex-Dupré-Christol (BDC) syndrome in a girl who died of prolymphocytic leukaemia at the age of 10 years. This is the first published case where the syndrome is associated with a blood disease. A review of the literature has enabled us to collect the various symptoms of BDC syndrome and to discuss the relationship of the syndrome with basal cell nevomatosis. We do not think that the blood disease was incidental. The child was 5 years' old in June 1972 when she was first seen in our out-patient clinic for severe hypotrichosis which had been present at birth and involved the eyelashes and eyebrows. Examination of the skin showed numerous milia on the face, a small lenticular polycystic lesion on the right ear, follicular atrophodermia on the back of the hands and hypotrichosis of the face. The right ear lesion was removed and found to look like Winer's cutaneous calcinosis. At light microscopy, many hairs showed like pili torti or, more rarely, like trichorrhexis nodosa. Despite the absence of basal cell carcinoma, the diagnosis of BDC syndrome was regarded as final. The patient died aged 10 years of prolymphocytic leukaemia revealed by pancytopenia. Combined chemotherapy with Rubidomycin, Oncovin and methotrexate had proved ineffective. Having collected from the literature 44 adequately documented cases summarized in table I, we describe the anatomico-clinical manifestations of Bazex-Dupré-Christol syndrome. Follicular atrophodermia is present in 85 p. 100 of the cases, but its date and mode of onset are difficult to evaluate.(ABSTRACT TRUNCATED AT 250 WORDS)

Alopecia↗
PubMedDate not supplied

Occipitofrontalis muscle: functional analysis revealed by electromyography.

The occipital and frontal bellies of the right occipitofrontalis muscle were studied electromyographically in thirty volunteers in various movements, using a special steel electrode (80 microns diameter). The occipital and frontal bellies have independent actions despite the galea aponeurotica. The frontal belly initially raises the homolateral eyebrow, followed by a displacement of the galea aponeurotica, drawing the scalp forward; therefore it is active in the formation of the transversal wrinkling in the forehead. The occipital belly eventually can be active during raising of the eyebrown, nevertheless it does not interfere with the action of the frontal belly. The occipital belly alone is responsible for drawing back the galea aponeurotica. The occipital belly is also active during smiling and yawning, and can be active during the movements of the auricula.

Adult↗
PubMed1989

Duplication 7p de novo and literature review.

We report on a boy with duplication of the short arm of chromosome 7 (karyotype 46,XY, dup (7) (p11.2----pter), QFQ, GTG, RBA). The boy showed delayed closure of fontanels, reduced eyebrows, short nose with low and broad nasal bridge, small upper and prominent full lower lips, severe delay of speech development. Comparison with the phenotype of 15 reported cases from the literature in relation to the extent of the duplicated segment did not show a clear phenotype/karyotype correlation.

Abnormalities, Multiple↗
PubMedDate not supplied

[Epidemiological immunological studies on leprosy in Okinawa. 6. Lepromin, lymphocyte transformation, FLA-ABS and leproagglutination tests in the in-and out-patients with leprosy and the relationships among these tests and personal or family history or clinical findings of the patients].

In- and out-patients with leprosy in Okinawa were surveyed by using the following immunological tests: cutaneous reaction to Mitsuda's lepromin (40 million bacilli/ml) (LPR), lymphocyte transformation test with Dharmendara's antigen (DL-LTT), that with phytohemagglutinin (PHA-LTT), fluorescent leprosy antibody absorption test (FLA-ABS) and Leproagglutination test with cardiolipin-lecithin antigen (LAT). A correlation between two tests and a relationship between the test and personal or family history or clinical finding of the patients were evaluated by non-parametric statistics. Spearman's rank correlation coefficient was significant between LPR and DL-LTT, while a reverse correlation was found between LPR and FLA-ABS, DL-LTT and FLA-ABS, and DL-LTT and LAT, respectively. High reactivity in PHA-LTT was found in almost all of patients, irrespective of type and stage of leprosy, suggesting general immune responsiveness of leprosy patients in Okinawa. The other tests showed a significant difference in the mean rank of reaction values according to clinical findings such as type and stage of leprosy, property of skin lesion, loss of eyebrow, and bacteriological test. The use of LPR and DL-LTT for the study of cell-mediated immunity in leprosy was therefore confirmed. FLA-ABS was found useful for early serodiagnosis, while LAT for the study of autoimmunity in leprosy. None of these immunological tests showed significant difference among the subgroups of patients classified by the following items: presence or absence of consanguineous patient with leprosy, living place, place of onset, presence or absence of neural symptoms such as the enlargement of peripheral nerve, claw hand and facial palsy, ocular and nasal involvement, ENL in lepromatous and borderline leprosy and conditions in the treatment. On the other hand, LPR showed higher reactivity in female than in male, in the patients at home than those discharged, and in the patients with drop foot than those without. Higher reactivity of DL-LTT was also found in male than in female and in the patients with plantar ulcer than those without. FLA-ABS reactivity was significantly higher in the discharged patients than the inpatients, in the period of 5 to 9 years after onset than that of 20 or more years, but the reactivity was lower in the patients with drop foot than those without. LAT reactivity was significantly higher in the 30-39 years old patients than 0-19 years old, and in the patients with physical work than those with mental.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1989

[Rubinstein-Taybi syndrome--a report of three cases].

The main features of Rubinstein-Taybi syndrome include special facial appearance, broad thumbs, great toes and mental retardation. This syndrome presents many organogenetic and systemic deformities and various congenital ocular abnormalities, such as epicanthus, antimogoloid palpebral fissures, highly arched eyebrows, long eyelashes, obstruction of nasolacrimal ducts, strabismus and iris coloboma. In this paper we report three children with Rubinstein-Taybi syndrome with the above systemic and ocular clinical findings, and think that the lacrimal abnormality should not be overlooked in this syndrome. We brief discuss the genetics of this syndrome and suggest that the search for minimal chromosomal defects by high resolution technique might be useful for etiologic and genetic research.

Chromosome Aberrations↗
PubMed1989

Anatomical observations of holoprosencephaly in swine.

In a study of swine congenital anomalies, nine newborn piglets with varying degrees of optic hypotelorism including cyclopia were collected. Nasal and maxillary development were abnormal in all animals regardless of the degree of eye fusion. All animals except one had intact upper lips and hard palates that carried two or three small extopic teeth. The "snout" was only a medial wedge-shaped rudiment projecting from the upper lip. It was distally covered by a typical snout-like glabrous epithelium and carried small vibrissae. Six animals also had a tubular proboscis dorsal to the eye. The distal tip of the proboscis was covered by glabrous epithelium. External nares and nasal passageways, albeit blind-ended, were prominent in the proboscis. The nasofrontal bones projected into the base of the proboscis. Seven piglets were hairless except for fine vibrissae and some eyebrow hairs. All animals had some degree of ear abnormalities, e.g., malposition and absence of external auditory meatus. In all animals the brain was malformed. This abnormality varied from complete absence of the forebrain to an alobar structure with gyri. The remainder of the body of each animal was normal. Developmental anomalies of the nose and eye generally reflect malformations of the forebrain, although the etiology of these defects is unclear. The cyclopia associated with the medial proboscis suggests that both the telencephalon and diencephalon are dysplastic. The presence of glabrous epithelium in two regions on the face suggests that studies of the development of the midline face in the swine will help to elucidate the etiology of the holoprosencephalic series. In this way the pig may prove to be an excellent modeling system for human holoprosencephaly.

Abnormalities, Multiple↗
PubMed1987

[Blink reflexes in hemifacial spasm with special reference to the synkinetic responses].

Blink reflexes were studied in 20 patients with hemifacial spasm. The latencies of R1 on the affected side was not different from that on the unaffected side in 10 patients who had no previous facial nerve block, but increased in 10 patients with experience of facial nerve block. Synkinetic responses (SR 1, SR 2 and SR 2 c) were observed by the simultaneous recording from the orbicularis oris muscle on the affected side. SR 1 were obtained in all patients (100%), but SR 2 in 18 patients (90%) and SR 2 c in 19 patients (95%). The mean latency of SR 1 was slightly longer than the mean latency of R 1. When the site of stimulation was moved along the eyebrow from the supraorbital nerve approaching to the stylomastoid foramen, the latencies of R 1 and SR 1 became shorter, but R 2 and SR 2 were suppressed with increased latency. Following local anesthesia (xylocaine) to the temporal branch of the facial nerve, SR 1 was abolished with preserved R 1, R 2 and SR 2. On the other hand, R 2 and SR 2 were abolished with preserved R 1 and SR 1 following xylocaine block to the supraorbital nerve. The result suggested that the afferent pathway of SR 1 is the facial nerve (antidromic conduction) and that of SR 2 is the trigeminal nerve. Ephaptic transmission of those antidromic and orthodromic impulses forming a reverberating circuit should be one of the etiological factors in hemifacial spasm.

Adult↗
PubMed1985

[A case of Cornelia de Lange syndrome with retentio testis].

A 6-year-old boy whose chief complaint was retentio testis was referred to our clinic. He had mental retardation, shortness of stature of prenatal onset, retarded growth, microbrachycephaly, bushy eyebrows, long eyelashes, low-set ears and micromelia, and was diagnosed to have Cornelia de Lange syndrome. As in most cases of Cornelia de Lange syndrome the cause of our case was not clear, and chromosome analysis showed a 46,XY constitution. In our case, we performed bilateral orchidopexy for retentio testis because of the problem of fertility and the danger of malignancy. Cornelia de Lange syndrome is rare and the urologic literature dealing with this disease has never been reported in Japan. The clinical features of this disease are discussed in the present report.

Brain↗
PubMedDate not supplied

[Lethal syndromes with thin bones].

The authors report six cases from six different families of lethal brittle bone disease with narrow diaphyses and thin ribs. This phenotype should be dissociated from the lethal forms of osteogenesis imperfecta and encompass two diseases. In the first, autosomal recessive, the metaphyses of long bones are narrow, with a membranous ossification, without cartilagenous residue. Cultured fibroblasts demonstrate a marked increase in type V collagen. In the second type, the metaphyses are enlarged and the babies have a facial dysmorphism with hypoplasia of the eyebrows, frontal bossing and a small mouth.

Bone Diseases, Developmental↗