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PubMed1977

[Erythematodes and nervous system (author's transl)].

Lupus erythematodes is related to the vegetative nervous system in Raynaud's disease, the butterfly distribution in the face, the involvement of the lateral part of the eyebrows (Hertoghe) and in cerebral attacks. Involvement of peripheral and central animal nervous systems is common and produces primary and secondary syndromes. Prominent are polymyositis or lupus-myopathy, in the brain mainly functional psychoses, epileptiform attacks, and a variety of focal, often very massive, signs. There are very clear differences from the neurologic signs of dermatomyositis, less so from arteritis nodosa and even less from progressive sclerodermia. Peripheral neurologic syndromes in lupus erythematosus are less common and more subtle than in arteritis nodosa. Bio-electric parameters of the petitmal-trias have been found. The relative absence of collagenoses from the spinal cord is also noticed in lupus erythematosus. Signs of involvement here appear to be limited and often subacute. Differential diagnosis has to consider many neurologic diseases, a special problem when MS is simulated just as in sclerodermia. Since cerbral attacks appear early in about 5%, the use of anticonvulsive drugs, particularly of the hydantoin group, provides special problems. Signs of myasthenia demand further attention. The neuropathologic changes are known in essential points and can obviously hardly be mistaken for inflammatory processes due to other causes. Certain basic facts appear to apply to other collagenoses. All 4 "grand" or "classic" collagen diseases are very similar and have much in common, but also show more or less definite differences. This is true for their clinical and anatomical appearance and last but not least to their neurologic aspects. The "roof-concept" of Collagenosis" is once more proved to be justified. As far as is known today the autonomic system plays a decisive and obligatory role only is sclerodermia. This syndrome is also the only one which contains the odd phenomenon of atrophy of which the best-known form is facial hemia-atrophy.

Autonomic Nervous System↗
PubMed1981

Hair changes due to zinc deficiency in a case of sucrose malabsorption.

A 7-year-old girl suffering from chronic diarrhoea due to sucrase deficiency was referred because of poor hair growth. Her scalp hair had a poor, colourless appearance and was much thinned in the occipital region. Her skin was dry, but otherwise normal. P-zinc was low (7.9 mumol/l), whereas P-albumin was normal. Oral zinc therapy, 40 mg daily, had a marked beneficial effect on her scalp hair, eyebrows and eyelashes, which became thicker and pigmented. Beau lines appeared on thumb-nails and 4th left finger-nail. A rise in P-zinc and S-alkaline phosphatase levels was observed during the zinc supplementation. Microscopic examination of her poor scalp hair, using polarized light, revealed well-defined abnormalities of the hair shafts, as reported by others in a case of acrodermatitis enteropathica: 1) a marked individual variation in diameter, 2) narrowing often associated with waving or sharp bending and broken ends, 3) striation with a tendency to trichonodosis. Such changes were absent in the pigmented hair appearing after the start of zinc therapy.

Alkaline Phosphatase↗
PubMed1984

Head-lice infestations and their treatment.

Head-lice infestations periodically cause disruption at schools and crèches. The life cycle of the insect and the treatment of infestation are discussed. Treatment is usually relatively simple and effective, relying mainly on the use of insecticides such as gamma benzene hexachloride, malathion and pyrethrins. Appropriate therapy for problem areas, for example the eyebrows, is also described.

Anoplura↗
PubMed1984

Two patients with interstitial del (14q), one with features of Holt-Oram syndrome. Exclusion mapping of PI (alpha-1-antitrypsin).

Patient no 1, a boy, was carrier of a de novo del (14) (pter- greater than q23::q32- greater than qter). Patient no 2, a boy, had a de novo del (14) (pter- greater than q23::q24.2- greater than qter). Common dysmorphisms included bushy eyebrows, frontal bossing, and micrognathia. Patient no 2 had features of Holt-Oram syndrome, i.e. congenital heart defect and severe ulnar defect. Patient no 1 had congenital heart defect but no typical osseous disorders. The association of Holt-Oram syndrome and del 14q24.1 is stressed. Patient no 1 was heterozygous for Pl (alpha-1-antitrypsin) phenotypes. The gene locus could thus be excluded from q24 and q31, and tentatively assigned to q32.1.

Abnormalities, Multiple↗
PubMedDate not supplied

[Lucio-Latapí leprosy and the Lucio phenomenon].

The Lucio-Latapí's leprosy or diffuse lepromatous leprosy is a clinical variety of lepromatous leprosy first described by Lucio and Alvarado in 1852 and reidentified by Latapí in 1936. It is frequent in Mexico (23%) and in Costa Rica and very rare in other countries. It is characterized by a diffuse infiltration of all the skin which never is transformed into nodule, by a complete alopecia of eyebrows and eyelashes and body hair, by anhydrotic and dysesthesic zones of the skin and by a peculiar type of lepra reaction named Lucio's phenomenon or necrotic erythema which is a vascularitis of vessels especially of the dermohypodermic union and of the hypodermis. Clinically this vascularitis is represented by well-shaped erythematous spots, later becoming necrotic with scabs, ulcerations and scars. Three points of confusion are stressed: the differences between nodules and nudosities, Lucio's leprosy and Lucio's phenomenon and necrotic erythema and necrotic erythema nodosum leprosum. The differences between the pure and primitive form of Lucio's leprosy and the secondary one is also discussed such as the laboratory findings, histopathological data, pronostic and treatment. Lucio's leprosy is considered the most anergic one of the all immunological spectrum of leprosy.

Drug Therapy, Combination↗
PubMed1984

[Benign late syphilis: 2 cases, gummatous syphilis and nodular syphilis].

Two mild syphilis cases are hereby described. Their clinical and morphological characteristics differ. The first case showed syphilitic gums on the right eyebrow rim and tuberous lesions, some of which ulcerated. They made up plaques like archs on limbs. The second case showed nodular lesions distribute on practically all the cutaneous tegument. The specific and non-specific syphilitic tests were positive. The CRL study on the cellular and serological aspects was normal. The histopathologic pieces were compatible with the clinical diagnosis. Lesions evolved to a quick disappearance with the penicillin G benzatine treatment established. Attention is drawn to the possibility of noticing new cases of mild syphilis due to the present high incidence of the sexually transmitted diseases.

Adult↗
PubMed1978

[The tricho-rhino-phalangeal syndrome (Giedion) (author's transl)].

We describe here six members of a family with Giedion's syndrome: sparse, fine, slow-growing hair, pear-shaped nose, cone-shaped epiphyses of the middle phalanges and variable growth retardation. There are other signs: elongated philtrum, thin upper lip, big and low set ears, medially broad eyebrows but narrow at the tip, clinobrachydactyly and marked dental caries, are as well observed in those patients. Differential diagnosis with other dysplasias is pointed out and both histological and trichological data are being valued.

Adult↗
PubMed1984

Oral zinc as an adjunct to dapsone in lepromatous leprosy.

Oral zinc was tried in 15 cases of multibacillary leprosy as an immunostimulant in addition to conventional antileprosy drugs. Results were compared with those in ten similar cases treated with dapsone alone. Cases treated with zinc showed faster clinical improvement, regrowth of eyebrows, rapid fall in the bacterial index (BI) and in the bacterial index in the granuloma (BIG), early and greater influx in lymphocytes in the granuloma, and neovascularization. Upgrading occurred in 6 out of 15 patients taking zinc, but in only 1 out of 10 patients in the control group. Five out of six patients who showed upgrading in the treated group became lepromin positive. Only one patient (BL) showed lepromin conversion in the control group.

Adult↗
PubMed1983

[Coffin-Siris syndrome. Critical study of the literature apropos of a case].

The authors report a case of the Coffin Siris syndrome which associates a ungueo-digital syndrome (special by the bilateral aplasia or severe hypoplasia of nails and third phalanx of fifth toes and fingers) to other anomalies: facies with thinly fine hairs contrasting with bushy and dense eyebrows and body hypertrichosis, hypotonia and mental retardation. The connections of the Coffin Siris syndrome with the trisomy 9 p+ syndrome and the fetal hydantoïn syndrome are discussed.

Abnormalities, Multiple↗
PubMed1980

[Increase of LDH A and partial trisomy 11p (author's transl)].

An increase of LDH A activity is observed in an adolescent patient trisomic for 11p with the exception of band 11p13. The clinical syndrome is delineated: broad faces, abundant eyebrows in their internal portion, enophtalmia, hypoplasic nasal bridge, hypertelorism, epicanthus, cleft palate or lip, macroglossia, hypotrophic muscles, soft and abundant skin, mental retardation.

Abnormalities, Multiple↗
PubMed1980

[Burn scars of the face and their treatment potential].

Hypertrophic scars and contractures are the result of the contraction of healing burns, giving a rigid mask-like, expressionless appearance of the face. Skin replacement should be done in stages, successively in the various regional entities of the face. The treatment of the burn-scarred skin of the face includes various methods of pedicles flaps, splitt- and full-thickness grafts, hair-bearing grafts to restore eyebrow etc. These alternatives are shown in this paper and illustrated by case reports from our clinic.

Adolescent↗
PubMed1980

Suture tarsorrhaphy system to control keratopathy after ptosis surgery.

Four tarsorrhaphy sutures are placed through the lower eyelid and brow following ptosis surgery in a system designed for patients with external ophthalmoplegia, third nerve paralysis, and myashenia gravis. Three of the sutures connect the lower eyelid to the forehead, and the fourth suture is passed through the lower eyelid and taped to the cheek. The three lid-brow sutures are released during the first two postoperative weeks, one by one, and topical ointment instillations are gradually tapered. The tarsorrhaphy system allows the cornea to adapt gradually to the lagophthalmos that follows ptosis surgery. It also keeps the eyelids partially closed during the first two postoperative weeks in patients with frontalis sling surgery who have marked difficulty lowering their eyebrow and closing their eyelids because of early postoperative forehead edema and pain. The tarsorrhaphy system has prevented serious keratopathy in six patients with ptosis associated with abnormal ocular motility and in one patient with lagophthalmos following trauma. It also allowed the six ptosis patients to have full, rather than partial, correction of their ptosis.

Blepharoptosis↗
PubMed1981

[Trichorhinophalangeal syndrome (Giedion)].

Three cases of the Tricho-rhyno-phalangeal syndrome (TRF) are described. Two cases were in the same family with some affected membres (Camacho et al., 1978). The third case, of sporadic presentation, showed radiological signs of Perthes' disease. All cases presented the main clinic and radiologic alterations of the TRF syndrome: hypotrichia, pear-shaped nose and cone shaped epiphyses. Some of them presented other characteristic features such as: darwism, prominent nose, elonged philtrum, thin upper lip, baldness of the external 1/2 eyebrows, prominent ears of low implantation, shortening of some fingers (of metacarpals and metatarsal), onicodystrophy, etc. The differential diagnosis must be stablished with: a) congenital processes which present facial abnormalities, specially of the nose, b) congenital atrichias o hypotrichias associated to osteoarticular troubles of the growth and c) congenital syndromes with epiphyses in cone (peripheric dysostosis). Finally, we find, by scanning electron microscope studies, an increase of the cuticular cells in detachment, an inespecific sign that denotes a special lability of these patients' hair to the action of the outside agents on it.

Adolescent↗
PubMed1982

[Tachistoscope and dichotic listening test of the subject after the transection of the posterior part of the corpus callosum].

The authors made neuropsychological studies by the tachistoscope and the dichotic listening test on a subject who had undergone the transection of the posterior part of the corpus callosum. As to the tachistoscopic recognition, stimulus material was composed with the various Japanese letters (Katakana, Hiragana, Kanji), various faces (variations of the eyebrow form and the mouth form) and various slopes of line. Table 1 shows results of the cases (the subject was the present case, subjects 1 and subject 2 were past cases). It was seen that the performance of the subject on Japanese letters tasks showed greater right visual field superiority than the one of subject 1 and subject 2. As to the auditory recognition, the tasks used for the dichotic listening test were the following (Table 2, 3, 4). Different digits (three pairs) of the subject showed greater right ear superiority (right ear: 61.1, left ear 5.9) than the ones of subject 1 and subject 2.

Brain Neoplasms↗
PubMed1982

Transcutaneous cranial electrical stimulation decreases narcotic requirements during neurolept anesthesia and operation in man.

The influence of transcutaneous cranial electrical stimulation (TCES) on fentanyl requirements was evaluated in 50 patients undergoing urologic operations with pure neuroleptanesthesia (droperidol, diazepam, fentanyl, and air oxygen) with (group I) or without (group II) simultaneous TCES. All patients had silver electrodes (three) applied between the eyebrows and behind each mastoid process and attached to a 167-kHz current generator. Current was delivered only to group I. The wave form was a complex nonsinusoidal, nonsquare wave pattern which was applied intermittently in a 3-msec-on 10-msec-off sequence. All patients had anesthesia induced with droperidol (0.20 mg/kg IV), diazepam (0.2 mg/kg IV), and pancuronium (0.08 mg/kg IV), and, after tracheal intubation, had anesthesia maintained with fentanyl in 100-microgram intravenous increments every 3 minutes whenever and as long as systolic arterial blood pressure and/or heart rate were greater than 20% of control (preanesthetic induction) values. Fentanyl requirements averaged 6.1 +/- 0.5 and 7.9 +/- 0.4 microgram/kg/min for a mean total dosage of 9.0 +/- 0.9 and 12.5 +/- 0.8 microgram/kg for the entire operation in groups I and II, respectively. These differences between groups were statistically significant (p less than 0.05). The data demonstrate that TCES augments the analgesic effects of fentanyl and thus reduces fentanyl requirements during urologic operations with neuroleptanesthesia.

Aged↗
PubMed1978

[Occlusion of the central retinal artery following injection of embolizing material (author's transl)].

The injection of a suspension of hydrocortisone into a keloidal scar of the nose of a 72 year-old man and of a foamy solution of hydroxypolyethoxydodecane into a little haemangioma situated above the right eyebrow of a 29 year-old woman was followed by sudden blindness of the homolateral eye in each case. This is basically explained by the involuntary injection of embolizing material into the anastomoses between the external and internal carotid arteries within the orbit. Therefore, the injection of embolizing material into the skin of the face should be avoided.

Adult↗
PubMed1982

[Type III shortrib-polydactyly syndrome (Verma-Naumoff) in concomitance with ectodermal dysplasia (author's transl)].

Reported in this paper is a case of shortrib-polydactyly syndrome in a stillborn male infant. The case was identified as Type III (Verma-Naumoff) on account of typical skeletal findings, such as very short ribs, micromelia, postaxial hexadactyly of all extremities, and shortened cranial base, with due consideration being also given, in that context, to characteristic radiographic and histological changes, including metaphysial spurs of long cylindrical bones. Malformations were recorded also from kidneys, ureters, small intestine, and pancreas.--Thin downy head-hair, missing eyebrows, precocious dentition, as well as partial hypoplasia and aplasia of nails were interpreted as signs of ectodermal dysplasia.--Shortrib-polydactyly syndrome is based on autosomal recessive inheritance. Early genetic advice should be offered to parents. Systematic prenatal diagnosis is necessary in case of another pregnancy.

Abnormalities, Multiple↗