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Treatment of equinocavovarus deformity in adults with the use of a hinged distraction++ apparatus.

We evaluated the results following the use of the Volkov-Oganesyan-Povarov hinged distraction apparatus to correct equinocavovarus deformity of the foot in sixty-five adults (eighty-three feet). The deformity was secondary to ischemic and neuropathic changes after trauma to the foot and ankle, poliomyelitis, Charcot-Marie-Tooth disease, or untreated clubfoot. The deformity often was associated with severe neurotrophic changes, extensive scarring of the skin, callosities, or osteomyelitis of the foot, all of which usually preclude operative reconstruction. The average age of the patients was twenty-six years (range, sixteen to fifty-six years). The average duration of follow-up was ten years (range, three to nineteen years). The duration of distraction was three to thirteen weeks, and the apparatus usually was kept in place for an additional two months after the desired position of correction had been achieved. The patient then wore a plaster cast for an average of eight weeks and custom-molded shoe-inserts for at least one year. A plantigrade foot was obtained with this external transosseous distraction device. Eight patients had inflammation of pin tracks, which was treated with local injection of antibiotics into the soft tissues and application of bandages soaked in antibiotics to the area around the pin sites. The pins were removed from five of the eight patients, and the apparatus was removed from three additional patients because of osteomyelitis. The results were classified as good in fifty-nine feet, satisfactory in twenty, and unsatisfactory in four. In the four feet (three patients) with an unsatisfactory result, a good result was obtained after an arthrodesis was done.

Adolescent↗

[Local vitamin A acid therapy in palmar/plantar hyperkeratoses (authors transl)].

Up to date, the treatment of palmar/plantar hyperkeratoses presents a therapeutic problem. The known therapeutic procedures result in short-term improvement only, if any at all. In these investigations involving 68 patients suffering from palmar/plantar hyperkeratoses of different etiology, small doses of vitamin A acid locally applied, produced a striking improvement in hypertrophic lichen planus of palms or soles: the regression was complete and in most cases permanent. The skin texture of patients with genetic keratoses and callosities became normal within a few weeks: but this condition remained free of symptoms only as long as vitamin A acid was used as a maintenance dose once or twice weekly. In hyperkeratotic eczema, pityriasis rubra pilaris, and verrucae plantaris vitamin A acid locally applied was found to be unsuitable for treatment. The possible side effects of this treatment are mentioned. Several possibilities regarding the way of action of vitamin A acid are discussed.

Callosities↗

Pseudo-knuckle pads: an unusual cutaneous sign of obsessive-compulsive disorder in an adolescent patient.

Knuckle pads are discrete benign cutaneous lesions overlying the extensor surfaces of the fingers and hand joints and are unrelated to trauma, whereas pseudo-knuckle pads may be considered as a form of callosity that appears after repeated trauma. This type of knuckle pad has been described in children with obsessive behavior as "chewing pads" and in adults as occupational disorder. Cases of pachydermodactyly, benign fibromatosis of the fingers, have been described as the unusual forms of knuckle pads that usually affect young adult males. We believe that pseudo-knuckle pads, chewing pads or pachydermodactyly are terms which have been used to decribe the same clinical situation reported in different patients. Here we describe a 12-year-old male patient with pseudo-knuckle pads along the metacarpophalangeal joints developed secondary to repeated trauma reflecting obsessive-compulsive disorder characterized by a tic-like habit. He received fluvaksamine 25 mg/day. The lesions started to disappear after three months of therapy. The recognition of pseudo-knuckle pads by dermatologists and pediatricians is very important in adolescent patients because these lesions may be clues for diagnosis of serious psychiatric problems. The collaboration of a dermatologist or pediatrician with a psychiatrist is essential in the follow-up of these patients.

Callosities↗

Prayer nodules in Moslem men.

Seven cases of Israeli Moslem men with hyperkeratotic nodular callosities of the legs and forehead are reported. These patients were randomly encountered over a two-year period in our dermatology clinic. The nodules were attributed to repeated trauma to the legs and forehead sustained during Moslem prayer. This article familiarizes the clinician with the morphologic characteristics of these lesions, which may occur in some Moslem men.

Adult↗

[The diabetic foot].

Neuropathy, arterial obstruction and infection are involved to varying degrees in the development of the "diabetic foot". A careful diagnosis is necessary in order to comprehend the various noxa and to introduce the adequate therapy. In case of the predominantly neuropathic foot with malum perforans, one must essentially proceed in a conservative way; only callosities and osteomyelitic parts must be removed. In case of the predominantly angiopathic foot, revascularizing measures are most important. After improving the blood flow, the necrotic parts of the foot are sparingly resected. As prevention of a possible relapse, two things are necessary: accurately fitted shoes in order to prevent pressure points, and extremely careful foot care in order to prevent infections.

Callosities↗

Russell's sign. Subtle hand changes in patients with bulimia nervosa.

Bulimia nervosa is a common eating disorder, affecting between 1% to 10% of adolescent girls and college aged women. Because excessive weight loss and amenorrhea are not significant features, as they are in anorexia, bulimia is much harder to diagnose. Orthopaedic surgeons have a unique opportunity to detect one of the few physical signs of the disease, which is skin lesions, consisting of abrasions, small lacerations, and callosities on the dorsum of the hand overlying the metacarpophalangeal and interphalangeal joints. These nondescript dorsal lesions are caused by repeated contact of the incisors to the skin of the hand that occur during self induced vomiting. This finding, known as Russell's sign, may be seen by orthopaedic surgeons during examinations for other reasons. Because eating disorders are recognized as a component of the female athlete triad of osteoporosis, amenorrhea, and eating disorders and because orthopaedic surgeons routinely care for female athletes susceptible to these disorders, recognizing this sign and its implications may have profound influence on the patient's musculoskeletal system and general health.

Adolescent↗

Knuckle pads from boxing.

A 21-year-old man developed painful hyperkeratotic, fissured, callosities on his knuckles ("knuckle pads") after having boxed for five evenings a week for six months. Boxer's knuckle pads are one type of traumatic skin injury regularly found in athletes, such as "jogger's toe", "athelete's nodule", "jog-ger's nipple", "runner's rump", "runner's bump", "rower's rump", "tennis toe", "skier 's shin", "surfer's nodule" "black heel", "black palm", "jazz ballet bottom" and "stria distensae". These types of traumatic skin injuries are briefly reviewed here.

Adult↗

[Clinical application of retrograde island flap carrying plantar metatarsal arteries as pedicle].

The skin and soft tissue defects or ulceration of the wight-bearing part of the sole was difficult to repair with medial plantar island flap, but would be treated with retrograde island flap carrying plantar metatarsal arteries as pedicle. Ten flaps were applied in 9 patients. They had either indolent ulcer or skin defect secondary to excision of painful corn or callosities of the front part of the sole. The flaps were 3 cm to 5 cm long and 3 cm to 4 cm wide, and they all survived following retrograde transfer. The patients were followed up for 1 to 10 years. It was found that the patients could bear weight on the operated foot and could walk without pain or lameness. The flaps were resistant to abrasion from long-time walking. It was concluded that this kind of flap was best suitable to repair the ulcers and defects over the front part of the sole despite there were some minor shortcomings such as the size of the flaps available was small and the donor site required split skin graft for coverage.

Adolescent↗

Possible Werner syndrome. A unique association with spontaneous digital gangrene in infancy and decreased life span of cultured skin fibroblasts.

BACKGROUND: Werner syndrome is a hereditary disease characterized by several features generally associated with aging. However, the differences between Werner syndrome and the normal aging process are clear. Werner syndrome is usually diagnosed through the clinical signs and symptoms it presents. In recent years, however, the cultured skin fibroblasts of patients have grown slowly and patients have a short life span; these characteristics provide a useful diagnostic aid. OBSERVATIONS: We recently examined a 42-year-old single man who had short fingers, marked facial scars resulting from chilblain-like eruption in infancy, glaucoma caused by uveitis developed in his middle age, callosities on the soles, and immaturation of sexual glands. Repeated peripheral blood examinations showed a positive result for antinuclear factor, slight elevation of immunoglobulin levels, and a low leukocyte count. His family had repeated consanguineous marriages and his parents were cousins. We cultured skin fibroblasts from the patient's forearm and compared them with those of four normal control male subjects. The patient's skin fibroblasts showed a remarkably low population growth rate and a total replicative life span. CONCLUSION: The patients presented unique clinical features for Werner syndrome such as chilblain-like eruption in infancy and glaucoma caused by uveitis. Cell culture studies revealed cellular abnormalities compatible with Werner syndrome. We thus diagnosed the patients as possibly having Werner syndrome.

Adult↗

Left unilateral agraphia and tactile anomia. Disturbances seen after occulusion of the anterior cerebral artery.

A 53-year-old right-handed Japanese man had a callosal disconnection syndrome associated with an occlusion of the left pericallosal artery. Computerized tomography scan confirmed a left medial lesion affecting the cingulate gyrus, the paracentral lobule, the precuneus of the left hemisphere, and most probably the corpus callosum. This anterior cerebral artery syndrome was characterized by right hemiparesis with predominant crural involvement, unilateral left-sided "disconnection" agraphia, and left unilateral "disconnection" tactile naming deficit. To our knowledge, this is the first case in the literature of the anterior cerebral artery syndrome in which unilateral leftsided apraxia was not associated with agraphia. The case is also the first in which Kanji-Kana dissociation of an aphasic nature has been reported as a feature of isolated left agraphia.

Agraphia↗

Medial frontal cortex infarction and the alien hand sign.

Two right-handed patients with infarction involving the left medial frontal cortex are described. Both patients exhibited a form of transcortical motor aphasia and a psychomotor disturbance involving the right arm characterized by forced grasping, motor perseveration, and the presence of apparently purposeful movements that appeared to be dissociated from conscious volition. The latter feature is noted to be identical to the alien hand sign as described in the left arm of commissurotomized patients and patients with callosal neoplasm or infarction. It is suggested that the observed dissociation of conscious intention from purposeful movement may be related to dysfunction of the medial frontal cortex contralateral to the impaired extremity as much as to hemispheric disconnection.

Aged↗

How the brain integrates affective and propositional language into a unified behavioral function. Hypothesis based on clinicoanatomic evidence.

Recent publications suggest that the right hemisphere dominates in modulating the affective components of language. Disorders of language form right-sided focal brain lesions have been called "aprosodias" and can be classified in a manner similar to the aphasias. We describe a patient with motor aprosodia who subsequently died and underwent neuropathologic examination. From the neuropathologic findings and recent observations concerning the neurology of depression, we hypothesize that the motor integration of propositional and affective language takes place in the brainstem, whereas their higher-order integration takes place via the callosal connections between Wernicke's area on the left and its homologue on the right. Direct application of these functional and anatomic relations can help clinicians to properly interpret the often incongruous and disparate behavioral and language responses encountered in brain-damaged patients.

Affect↗

Magnetic resonance imaging correlates of dementia in multiple sclerosis.

Thirty-two patients with clinically definite multiple sclerosis were evaluated with neuropsychological procedures and magnetic resonance imaging (MRI). Neuropsychological evaluation included assessment of language, memory, cognition, visuospatial skills, and depression. Significant impairment in any three areas, compatible with diagnosis of a dementia syndrome, was observed in 28% of these patients, and lesser or no cognitive impairment characterized the remaining patients. Magnetic resonance imaging was used to evaluate the number and distribution of lesions as well as the presence of cerebral atrophy and atrophy of specific anatomic structures such as the corpus callosum. Results suggest that neither the number of lesions, the distribution of lesions, nor the extent of generalized cerebral atrophy was significantly greater in demented compared with non-demented patients. The primary finding was that atrophy of the corpus callosum was significantly more extensive on MRI scans in demented patients. Although the callosum itself may not be implicated directly in the pathogenesis of dementia, the presence of callosal atrophy on MRI scans should alert the physician to the possible occurrence of dementia in patients with multiple sclerosis.

Adult↗

Corpus callosum agenesis and psychosis in Andermann syndrome.

Recent illustrations by cerebral magnetic resonance imaging of anomalies of the corpus callosum in schizophrenics have kindled renewed interest in this association. We studied 62 patients affected by the Andermann syndrome, a polymalformative familial syndrome combining frequent congenital corpus callosum agenesis, mental retardation, psychotic episodes, peripheral neuropathy, and some dysmorphic features. Twenty of 62 patients presenting with psychosis were compared with 20 nonpsychotic patients matched according to sex and age. The psychotic patients presented an atypical psychosis as defined by the Diagnostic and Statistical Manual of Mental Disorders, Third Edition, beginning in postadolescence. No significant relationship was observed between corpus callosum agenesis and psychosis. However, a significant association between posterior fossa atrophy and psychosis was established in our study. Although there are limitations in using cross-sectional data for this purpose, the findings suggest an association between cerebellar anomalies and schizophrenialike syndrome and rule out an implication of developmental callosal defects in such psychiatric disorders.

Adolescent↗

Reverse Shapiro's syndrome. A case of agenesis of corpus callosum associated with periodic hyperthermia.

OBJECTIVE: Investigation of the mechanism of the idiopathic periodic hyperthermia associated with agenesis of the corpus callosum. SETTING: Tertiary care referral center. PATIENT: Fourteen-year-old girl who was the first case of reverse Shapiro's syndrome (ie, agenesis of the corpus callosum associated with periodic hyperthermia, as opposed to hypothermia as described in the original report by Shapiro et al). INTERVENTION: Magnetic resonance imaging scans, endocrinological examinations for hypothalamus, electroencephalograms, and levodopa therapy. MAIN OUTCOME MEASURE: Body temperature. RESULT: No neurologic or physical abnormalities were noted beside the callosal agenesis. Neuroimaging examinations could not reveal any structural abnormality of the diencephalon. Endocrinological examinations and electroencephalograms were found to be normal. The hyperthermia returned to normal by a small dose of levodopa, but a larger dose reversed the hyperthermia to hypothermia. CONCLUSIONS: These observations suggest that the hyperthermia observed in this case may have been caused by the dopaminergic denervation of the hypothalamic thermoregulatory center, resulting in the supersensitivity of its dopaminergic receptors.

Adolescent↗

Atrophy of the corpus callosum, cortical hypometabolism, and cognitive impairment in corticobasal degeneration.

OBJECTIVE: To investigate whether atrophy of the corpus callosum is associated with cognitive impairment and cerebral cortical hypometabolism in corticobasal degeneration. DESIGN: Prospective clinicoradiological correlation with magnetic resonance imaging and positron emission tomography. SETTING: A university hospital. PATIENTS: Eight right-handed patients with clinically diagnosed corticobasal degeneration (mean+/-SD age, 64+/-8 years). MAIN OUTCOME MEASURES: Midsagittal corpus callosum area-skull area ratio (on T1-weighted magnetic resonance images), the sum of the scaled scores of the 6 subtests on the Wechsler Adult Intelligence Scale-Revised (Digit Span, Arithmetic, Picture Arrangement, Object Assembly, Block Design, and Digit Symbol), and cerebral metabolic rate of glucose (measured with positron emission tomography by using fludeoxyglucose F 18 as a tracer). RESULTS: Compared with 36 age-matched right-handed control subjects, the patients had significantly decreased callosal area-skull area ratio. The reduction in this ratio was greatest in the middle half of the corpus callosum. The atrophy of the corpus callosum was accompanied by a decreased mean cortical glucose metabolic rate with hemispheric asymmetry and a decrease in the sum of the scaled subtest scores of the Wechsler Adult Intelligence Scale-Revised. CONCLUSIONS: Atrophy of the corpus callosum with middle predominance is present in corticobasal degeneration, and this atrophy is associated with cognitive impairment and cerebral cortical hypometabolism with hemispheric asymmetry. Atrophy of the corpus callosum might reflect the severity of the disconnection between cortical regions, and this may be an important factor in the development of cerebral cortical dysfunction in corticobasal degeneration.

Aged↗

Bilateral functional MRI activation of the basal ganglia and middle temporal/medial superior temporal motion-sensitive areas: optokinetic stimulation in homonymous hemianopia.

OBJECTIVE: To determine to what extent sensorimotor control is achieved for each hemisphere separately or interactively during small-field optokinetic stimulation in patients with complete homonymous hemianopia. DESIGN: Functional and structural neuroimaging using high-resolution magnetic resonance imaging. SETTING: University medical center research facility. PATIENTS: Three patients with complete homonymous hemianopia after acute infarction of the right posterior cerebral artery. MAIN OUTCOME MEASURES: Anatomical location of activated structures during horizontal optokinetic stimulation and T2-weighted anatomical magnetic resonance imaging. RESULTS: Occipitotemporal cortical areas (Brodmann areas 39 and 40) were the only activated cortical structures that showed statistically significant (P<.01) activation on the affected hemisphere. Of the subcortical areas, activation of thalamic nuclei appeared to be missing on the affected side, whereas the basal ganglia (putamen, globus pallidus, and caudate nucleus) were bilaterally activated. CONCLUSIONS: Bilateral activation of the basal ganglia confirms the concept of the basal ganglia-thalamocortical motor loop and of the efference copy of oculomotor pathways from each hemisphere. Our findings suggest 2 possible explanations for the activation of occipitotemporal areas (the human homolog of middle temporal/medial superior temporal areas) on the infarcted hemisphere: involvement of direct extrastriatal visual pathways or interhemispheric callosal connections between right and left middle temporal/medial superior temporal areas.

Aged↗

Marchiafava-Bignami disease: computed tomographic scan, 99mTc HMPAO-SPECT, and FLAIR MRI findings in a patient with subcortical aphasia, alexia, bilateral agraphia, and left-handed deficit of constructional ability.

OBJECTIVES: To report and discuss the neuropsychological deficits and neuroimaging findings in a patient with probable Marchiafava-Bignami disease. DESIGN AND METHOD: A right-handed woman with chronic alcoholism demonstrated mutism, impaired comprehension of spoken language, alexia, and right-handed agraphia. The syndrome of interhemispheric disconnection was manifested by left-handed deficit of constructional ability and agraphia. The patient underwent brain computed tomographic scans, technetium 99 hexylmethylpropylene amineoxime-single photon emission computed tomography, and magnetic resonance imaging (MRI) that also included fluid attenuated inversion recovery images. SETTING: Clinical neurology department. RESULTS: The patient's symptoms were related to scattered lesions of the corpus callosum and to extensive symmetrical lesions of the centrum semiovale. Only the latter were detected by computed tomographic scans. Results of single photon emission computed tomography did not show areas of focal hypoperfusion. Results of fast spinecho MRI showed all lesions were hyperintense in T1-weighted images and hypointense in T2-weighted images. Fluid attenuated inversion recovery images revealed that periventricular lesions had a hypointense core surrounded by a hyperintense rim; callosal lesions were still hyperintense. CONCLUSIONS: We believe that our patient's symptoms are due to the discontinuous affection of the corpus callosum and to the bilateral cutting of the outflow from the cortex. The MRI findings may be interpreted as indicating central necrosis and peripheral demyelination of periventricular lesions and demyelination of the corpus callosum. The combined use of fast spin echo and fluid attenuated inversion recovery MRI reproduced with more accuracy than fast spin echo MRI alone some features of Marchiafava-Bignami disease known from observations at autopsy.

Adult↗