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PubMed2002

Acceleromyography of the orbicularis oculi muscle II: comparing the orbicularis oculi and adductor pollicis muscles.

BACKGROUND: The orbicularis oculi (OO) muscle has been recommended for neuromuscular monitoring when the adductor pollicis (AP) muscle is not available. We investigated whether neuromuscular block could be measured reliably from the orbital part of the OO muscle by the use of acceleromyography. METHODS: During propofol, fentanyl, and alfentanil anaesthesia two TOF-Guards (Organon Teknika NV, Boxtel, the Netherlands) with acceleration transducers placed on the distal phalanx of the thumb and over the middle of the eyebrow, respectively, were used to measure neuromuscular block simultaneously in 23 patients during vecuronium-induced and neostigmine-antagonized neuromuscular block. For both muscles, the simultaneously recorded first response (T1) in the train-of-four (TOF) and TOF-ratio were measured both during onset and recovery of the block. Furthermore, both the AP muscle T1 and TOF-ratio responses were plotted against 10% intervals of the OO muscle responses during onset and recovery, respectively. RESULTS: The orbicularis oculi muscle had a shorter latency and a faster recovery to TOF-ratio 0.80 compared with the AP muscle. During onset and recovery, pronounced variations of the AP muscle T1 and TOF-ratio responses were observed when compared with the OO muscle. CONCLUSION: A significant clinical disagreement exists between the degree of paralysis measured at the OO and the AP muscles. It is impossible to obtain a reasonable estimate of the degree of block at the AP muscle when the block is measured from the OO muscle with acceleromyography. If used, there is substantial risk of overlooking a residual block, and adequate recovery of the block should be confirmed by a final AP muscle measurement.

Adult↗
PubMed1999

Bilateral abortive cryptophthalmos associated with oculocutaneous albinism.

PURPOSE: To document a case of bilateral abortive cryptophthalmos associated with oculocutaneous albinism. METHODS: We describe a 13-year-old female patient with blond skin and hair who had anomalous face and eyes first noted at birth. RESULTS: The patient had fair hair and complexion, bilateral anomalous wedge of scalp hair, upper eyebrow and eyelid deformities, cup-shaped ears, broad based nose with depressed bridge and midline groove, iris transillumination and diffuse hypopigmentation and foveal hypoplasia in fundus examination. CONCLUSION: Based on the clinical and laboratory findings, the reported case represents tyrosinase-positive oculocutaneous albinism together with bilateral abortive cryptophthalmos. The oculocutaneous albinism can either be an entirely new criterion to the whole syndrome of cryptophthalmos, or, more probably, the condition can be due to a double affliction which is primarily related with consanguinity of the parents. The importance of ante-natal diagnosis and genetic counseling are emphasized in such cases where the two recessive genes for two different conditions coexist.

Adolescent↗
PubMed1988

The recognition of threatening facial stimuli.

Two studies examined the information that defines a threatening facial display. The first study identified those facial characteristics that distinguish between representations of threatening and nonthreatening facial displays. Masks that presented either threatening or nonthreatening facial displays were obtained from a number of non-Western cultures and scored for the presence of those facial features that discriminated between such displays in the drawings of two American samples. Threatening masks contained a significantly higher number of these characteristics across all cultures examined. The second study determined whether the information provided by the facial display might be more primary nonrepresentational visual patterns than facial features with obvious denotative meaning (e.g., diagonal lines rather than downturned eyebrows). The subjective response to sets of diagonal, angular, and curvilinear visual stimuli revealed that the nonrepresentational features of angularity and diagonality in the visual stimulus appeared to have the ability to evoke the subjective responses that convey the meaning of threat.

Aggression↗
PubMed2006

Evidence for strong dissociation between emotion and facial displays: the case of surprise.

Eight experiments examined facial expressions of surprise in adults. Surprise was induced by disconfirming a previously established schema or expectancy. Self-reports and behavioral measures indicated the presence of surprise in most participants, but surprise expressions were observed only in 4%-25%, and most displays consisted of eyebrow raising only; the full, 3-component display was never seen. Experimental variations of surprise intensity, sociality, and duration/complexity of the surprising event did not change these results. Electromyographic measurement failed to detect notably more brow raisings and, in one study, revealed a decrease of frontalis muscle activity in the majority of the participants. Nonetheless, most participants believed that they had shown a strong surprise expression.

Adult↗
PubMed2005

Orienting to eye gaze and face processing.

The author conducted 7 experiments to examine possible interactions between orienting to eye gaze and specific forms of face processing. Participants classified a letter following either an upright or inverted face with averted, uninformative eye gaze. Eye gaze orienting effects were recorded for upright and inverted faces, irrespective of whether the faces were simple, schematic faces or more realistic faces. In contrast, inversion affected orienting to targets appearing along the vertical axis. Switching the contrast between the iris and sclera reversed orienting to eye gaze. Lifting the eyelid to expose more of the iris-sclera contrast led to a potentiation of orienting to eye gaze. Raising the eyebrow alone without the eyelid did not affect orienting. The findings suggest that local perceptual information is critical for orienting to eye gaze and that the effect can occur with a degree of independence from certain types of face processing.

Adolescent↗
PubMed2003

New displays and new emotions: a commentary on Rozin and Cohen (2003).

In this article, the authors elaborate on 3 ideas advanced in P. Rozin and A. B. Cohen's (2003) innovative study of facial expression. Taking a cue from their discovery of new expressive behaviors (e.g., the narrowed eyebrows), the authors review recent studies showing that emotions are conveyed in more channels than usually studied, including posture, gaze patterns, voice, and touch. Building on their claim that confusion has a distinct display, the authors review evidence showing distinct displays for 3 self-conscious emotions (embarrassment, shame, and pride), 5 positive emotions (amusement, desire, happiness, love, interest), and sympathy and compassion. Finally, the authors offer a functional definition of emotion to integrate these findings on "new" displays and emotions.

Arousal↗
PubMed2005

Plucked human hair as a tissue in which to assess pharmacodynamic end points during drug development studies.

We have demonstrated the feasibility of detecting and quantifying six cell-cycle-related nuclear markers (Ki67, pRb, p27, phospho-p27 (phosphorylated p27), phospho-pRb (phosphorylated pRb), phospho-HH3 (phosphorylated histone H3)) in plucked human scalp and eyebrow hair. Estimates of the proportion of plucked hairs that are lost or damaged during processing plus the intra- and intersubject variability of each nuclear marker with these techniques are provided to inform sizing decisions for intervention studies with drugs potentially impacting on these markers in the future.

Adolescent↗
PubMed2000

An autosomal dominant form of hereditary hypotrichosis simplex maps to 18p11.32-p11.23 in an Italian family.

We report on a three-generation Italian family with dominant transmission of a form of hereditary hypotrichosis simplex (HHS). The nine affected adults presented with sparse, thin and short hair. Somewhat less sparse and longer hair was observed in the two affected young children in the third generation. Reduced hair growth affected the scalp and body, although normal eyelashes, eyebrows and growth of men's beards were observed. No associated abnormality was detected and the overall psychomotor development of the affected individuals was normal. A phenotypic variation was observed amongst the family members and is suggestive of a reduced penetrance of the trait or the effect of a modifying factor. After exclusion, in our family, of linkage to loci previously described in other forms of atrichia or hypotrichosis, we performed a genome-wide linkage analysis, which resulted in a positive lod score at 18p11.32-p11.23. We defined a critical region of about 35 cM flanked by markers D18S853 and D18S40. The highest two-point lod score was obtained with the microsatellite markers D18S1376, D18S53 and D18S453 (lod score of 3.31 at theta = 0.00). The 18p11.32-p11.23 locus represents the first chromosome region shown to be associated with hereditary hypotrichosis simplex.

Adult↗
PubMed1999

The molecular basis of congenital atrichia in humans and mice: mutations in the hairless gene.

Congenital atrichia is a form of total alopecia inherited in an autosomal recessive pattern. In individuals affected with this form of hair loss, hairs are typically absent from the scalp, and patients are nearly completely devoid of eyebrows, eyelashes, axillary and pubic hair, following shedding of the natural hair shortly after birth. We have recently linked this disorder to the chromosomal region 8p12, and cloned the human hairless gene, which resides within this interval. We have identified several mutations in the hairless gene in atrichia families from around the world. In hairless mice, the hair matrix cells appear to undergo a premature and massive apoptosis, together with a concomitant decline in Bcl-2 expression, a loss of NCAM positivity, and a disconnection with the overlying epithelial sheath essential for the movement of the dermal papilla. As a consequence, the hair bulb and dermal papilla remain stranded in the dermis, and indispensible messages between the dermal papilla and stem cells in the bulge are not transmitted, so no further hair growth occurs. These findings suggest that the hairless gene product may play a crucial role in maintaining the delicate balance between cell proliferation, differentiation and apoptosis in the hair follicle, as well as in the interfollicular epidermis.

Alopecia↗
PubMed1999

Marie Unna congenital hypotrichosis: clinical description, histopathology, scanning electron microscopy of a previously unreported large pedigree.

Marie Unna congenital hypotrichosis (MUCH) is a rare autosomal dominant condition in which abnormalities are confined to hair shaft structure and hair density. We report a six-generation pedigree consisting of 59 members of whom 16 are affected; nine identified affected individuals are living. Affected individuals are born with adequate, normal to coarse hair. During early infancy the scalp hair becomes more coarse and wiry and stands out from the head. All affected individuals have sparse to absent eyebrows, eyelashes and body hair including secondary sexual hair. In some individuals, scalp hair is progressively lost beginning at puberty or beyond, until only a sparse fringe in the tonsorial distribution remains. The hair shafts are uniformly increased in diameter, measuring up to 0.12 mm. Individual hair shafts are deeply pigmented, variable in diameter, twisted, and bent at odd angles; some have a longitudinal groove visible on scanning electron microscopy. Cross-sectional shapes are variable and irregular, exhibiting oval, angular to reniform shapes. Multiple anagen hairs are extractable on gentle hair pull. Other ectodermal structures are unaffected except for exceptionally widely spaced upper incisor teeth seen in 50% of affected individuals. Histologically, there are dramatically reduced numbers of follicles per unit area, averaging nine total hairs per 4 mm cross-section as compared with a normal of 40. A mild to moderate inflammatory infiltrate is present, but little fibrosis and no scarring. The mechanism of progressive hair loss is unknown.

Adolescent↗
PubMed2004

Treatment of primary orbital lymphomas.

We present an overview of the treatment and clinical outcome of 15 orbital lymphomas, carried out in our center from 1993 to 2002. The surgical approach was determined by the location, and type of the lesion. Lateral orbitotomy was performed in five laterally located lesions, a transconjunctival approach in five medial, basal, extra-, intraconal lesions. Pterional intradural and extradural and intradural approaches (three) were used in case of intracranial involvement or location in the apex and optic canal. Two lesions of the lid or extraconal space were operated via eyebrow incision. Ten patients were diagnosed as extranodal marginal zone lymphoma. Diffuse large B-cell lymphoma (DLCL) was encountered in two patients, follicular lymphoma (FCL) in two patients, and diffuse lymphoplasmacytic/lymphoplasmacytoid lymphoma in one patient. All patients except one failed to reveal systemic disease and had a localized orbital lymphoma. Twelve of the patients responded to radiation therapy with usually 40 Gy. Cyclophosphamide, doxorubicin, vincristine, prednisone chemotherapy was applied in two patients, once in combination with radiotherapy. Local recurrence was seen in one patient with FCL. Systemic manifestation occurred in one patient with DLCL, undergoing ifosfamide, carboplatin, etoposide chemotherapy with complete remission. The course of orbital non-Hodgkin's lymphoma is variable and requires a multidisciplinary treatment. Therapeutic options include surgical biopsy, radiation therapy, and chemotherapy.

Adult↗
PubMed1998

180-kDa bullous pemphigoid antigen defective generalized atrophic benign epidermolysis bullosa: report of four cases with an unusually mild phenotype.

Generalized atrophic benign epidermolysis bullosa (GABEB) is a rare variant of non-lethal junctional epidermolysis bullosa characterized by generalized skin blistering healing with atrophy and by atrophic alopecia with onset in childhood. Other features include mild mucosal blistering, dental abnormalities and nail dystrophy. We report four additional cases of GABEB from two families originating from the same isolated village. The patients shared an unusually mild clinical phenotype with cutaneous blisters strictly limited to trauma sites and rare occurrence of oral mucosal lesions. Scalp, eyelash and eyebrow alopecia was present in only two cases. Immunofluorescence studies showed a markedly reduced expression of the 180-kDa bullous pemphigoid antigen (BP180), and northern analysis of cultured keratinocytes indicated that the gene encoding for BP180 is affected in these GABEB patients.

Adult↗
PubMed2001

Randomized, placebo-controlled, double-blind study on clinical efficacy of ciclopiroxolamine 1% cream in facial seborrhoeic dermatitis.

BACKGROUND: Antifungal agents are beneficial in the treatment of seborrhoeic dermatitis. OBJECTIVES: To perform a randomized, vehicle-controlled, double-blind clinical study with an antifungal ciclopiroxolamine (CIC) 1% cream in patients with mild-to-moderate seborrhoeic dermatitis of the face. METHODS: One hundred and twenty-nine patients were enrolled, 57 patients in the CIC group and 72 patients in the vehicle group, and comprised the study population for efficacy (intent-to-treat analysis) and safety. Patients were randomly allocated to apply either the CIC cream or the vehicle on their facial lesions, twice daily for a maximum of 28 days (initial phase), followed by a once daily application of the test products for another 28 days (maintenance phase). Test lesions were defined as lesions localized in the nasolabial folds and/or the eyebrow. The main efficacy parameter (end-point) was the proportion of patients who achieved complete disappearance of erythema and scaling (treatment responders) at the end of the initial phase (28 days or less) and of the maintenance phase (28 days). RESULTS: At baseline, both treatment groups were comparable in terms of demographic data and lesional status. At the end of the initial phase, responders to treatment were higher with CIC (25 patients, 44%) than with the vehicle (11 patients, 15%) (P < 0.001, Fisher exact test). At the end of the maintenance phase, responders in both groups were even higher, comprising 27 patients (63%, n = 43) in the CIC group and 15 patients (34%, n = 44) in the vehicle group (P < 0.007, intergroup analysis). The local tolerance was good in the two groups, except for a higher rate of lesional exacerbation in the vehicle group. No drug-related systemic adverse event was observed during the study. CONCLUSIONS: CIC administered in a cream demonstrated a good therapeutic value in mild-to-moderate seborrhoeic dermatitis of the face.

Administration, Cutaneous↗
PubMed2001

Keratin K6irs is specific to the inner root sheath of hair follicles in mice and humans.

BACKGROUND: Keratins are a multigene family of intermediate filament proteins that are differentially expressed in specific epithelial tissues. To date, no type II keratins specific for the inner root sheath of the human hair follicle have been identified. OBJECTIVES: To characterize a novel type II keratin in mice and humans. METHODS: Gene sequences were aligned and compared by BLAST analysis. Genomic DNA and mRNA sequences were amplified by polymerase chain reaction (PCR) and confirmed by direct sequencing. Gene expression was analysed by reverse transcription (RT)-PCR in mouse and human tissues. A rabbit polyclonal antiserum was raised against a C-terminal peptide derived from the mouse K6irs protein. Protein expression in murine tissues was examined by immunoblotting and immunofluorescence. RESULTS: Analysis of human expressed sequence tag (EST) data generated by the Human Genome Project revealed a fragment of a novel cytokeratin mRNA with characteristic amino acid substitutions in the 2B domain. No further human ESTs were found in the database; however, the complete human gene was identified in the draft genome sequence and several mouse ESTs were identified, allowing assembly of the murine mRNA. Both species' mRNA sequences and the human gene were confirmed experimentally by PCR and direct sequencing. The human gene spans more than 16 kb of genomic DNA and is located in the type II keratin cluster on chromosome 12q. A comprehensive immunohistochemical survey of expression in the adult mouse by immunofluorescence revealed that this novel keratin is expressed only in the inner root sheath of the hair follicle. Immunoblotting of murine epidermal keratin extracts revealed that this protein is specific to the anagen phase of the hair cycle, as one would expect of an inner root sheath marker. In humans, expression of this keratin was confirmed by RT-PCR using mRNA derived from plucked anagen hairs and epidermal biopsy material. By this means, strong expression was detected in human hair follicles from scalp and eyebrow. Expression was also readily detected in human palmoplantar epidermis; however, no expression was detected in face skin despite the presence of fine hairs histologically. CONCLUSIONS: This new keratin, designated K6irs, is a valuable histological marker for the inner root sheath of hair follicles in mice and humans. In addition, this keratin represents a new candidate gene for inherited structural hair defects such as loose anagen syndrome.

Amino Acid Sequence↗
PubMed2002

Hereditary, focal, transgressive palmoplantar keratoderma with associated clinical findings: a new entity?

We report on a 24-year-old male originating from Yugoslavia with a focal, transgressive palmoplantar keratoderma presumably inherited as an autosomal recessive trait. Associated clinical findings were hyperkeratotic lichenoid papules on the knees and elbows, psoriasis-like lesions in the groins and on the scalp, a spotty or reticulate hyperpigmentation of the face, trunk and extremities and a partial alopecia of the left eyebrow and eyelashes. The patient's sister was affected by similar but less pronounced cutaneous changes. Although our case shares some similarities with other hereditary palmoplantar keratodermas there remain substantial differences. We therefore believe this case to represent a new entity.

Adult↗
PubMed2002

Hereditary hypotrichosis simplex: report of a family.

We report a family with hereditary hypotrichosis simplex of the scalp, a rare disorder that was first described in 1974. In our family, four out of 10 siblings were affected, including three females and one male. Examination showed thinning of the scalp hair and sparse body hair. Eyebrows, eyelashes, pubic and axillary hair were normal. Skin, nails and teeth were also normal. Hair shaft examination did not reveal any structural abnormalities. Normal follicular units, hair shafts within follicles, eccrine glands and a lack of inflammation were seen on histopathology. The primary pathology underlying this genodermatosis is unclear, but the anagen phase of the hair cycle is clearly compromised.

Adolescent↗
PubMed2003

Postmenopausal frontal fibrosing alopecia.

Recently a new entity, postmenopausal frontal fibrosing alopecia, was added to the established subtypes of scarring alopecias affecting postmenopausal women. This condition is characterized by a progressive frontal hairline recession associated with scarring. We studied the clinical and histopathologic features in four women with this disorder. Of note, a history of bilateral oophorectomy in two of them appears to be a new association. All four cases had frontoparietal recession of the hairline and two of them also had loss of their eyebrows. None of our four patients had any mucous membrane or other skin lesions. Histological examination showed perifollicular fibrosis and lymphocytic inflammation around the isthmus and infundibular areas of the follicles. No effective treatments have emerged for this type of postmenopausal alopecia, but progression of the hair loss and scarring appears to be self-limiting. We believe that this condition is a distinct clinicopathological variant of lichen planopilaris.

Adult↗
PubMed1999

Unilateral transient forehead paralysis following injury to the temporal branch of the facial nerve.

BACKGROUND: Cutaneous surgery in the temporal region of the forehead can lead to injury to the superficial temporal branch of the facial nerve. A flattened forehead and with ipsilateral forehead paralysis can occur with damage to this nerve. METHODS: A case is presented of transient forehead paralysis resulting from Mohs' micrographic surgery with reconstruction of the defect. The paralysis resolved over a period of fifteen months. RESULTS: The anatomy of the nerve makes it susceptible to injury during cutaneous surgery. The area of danger is the area superior to the zygomatic arch and lateral to the lateral eyebrow where the nerve is closest to the skin. CONCLUSIONS: Restoration of motor function usually occurs without intervention, but may take several months. Should motor function not recur, nerve grafting of a repair of the ptotic brow may be needed. The anatomy of the nerve is reviewed and brow lifting options are discussed.

Aged↗