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PubMed1981

Congenital cholinergic nervous system dysfunction in identical twins.

We report identical male twins with secretory diarrhea, craniofacial abnormalities often found in G syndrome, hypospadias, and abnormalities of the cholinergic nervous system including achalasia, decreased tearing, corneal hypesthesia, positive Mecholyl test (eye and esophagus), and decreased sweating. The children also lacked fungiform papillae but were clinically distinct from patients with either the Riley-Day syndrome or acquired dysfunction of the autonomic nervous system. Assessment of autonomic nervous system function should be considered in infants with recurrent diarrhea or swallowing disorders or in children with morphologically characteristics suggesting G syndrome.

Abnormalities, Multiple↗
PubMed1994

Chronicity of the eosinophilia-myalgia syndrome. A reassessment after three years.

OBJECTIVE: To define the natural history and outcome of eosinophilia-myalgia syndrome (EMS) among a cohort of patients followed up at one center since the onset of their disease. METHODS: Fifty-seven patients with well-characterized EMS were evaluated prospectively at a university hospital for 21-64 months (mean 36 months). RESULTS: Eighty-eight percent of the patients continue to have symptomatic disease with > 3 clinical manifestations. Fatigue (91%), muscle cramping (75%), myalgia (70%), paresthesias with objectively demonstrated hypesthesias (62%), articular symptoms (54%), scleroderma-like skin changes (44%), and proximal muscle weakness (40%) are the most common features of chronic EMS. New findings identified among this cohort include cognitive symptoms in 86% of the study group, tremor, and myoclonus. CONCLUSION: The recognition of new manifestations in EMS and ongoing clinical disease in 88% of patients highlights the chronic nature of this disorder. Continued prospective followup of a large patient base is warranted to further define the natural history of this newly recognized illness.

Adult↗
PubMedDate not supplied

Combined approach for excision of cervical nerve tumors with dural extension.

Tumors of the cervical plexus are rare. Often these tumors are found on routine exam as asymptomatic masses. We present our experience in managing four patients with tumors with dural extension. Three of these lesions were neurilemomas and one was a meningioma. Symptoms and signs included weakness and hypoesthesia. Evaluation included complete neurologic examination with electromyography (EMG). Magnetic resonance imaging (MRI) was the best diagnostic tool to see tumor extent into the epidural and intradural space. Computed tomography (CT) or plain x-rays were used to evaluate the degree of destruction of the cervical spine. The surgical removal of these tumors was performed by a two-team approach. A posterior laminectomy was combined with an anterior neck exploration. Follow-up shows persistent upper extremity weakness in two patients, hypesthesia in three patients, and anesthesia of the anterior chest wall in two patients. Patients with these lesions should be informed of the potential neurologic consequences of removal.

Adult↗
PubMed2007

Meralgia paresthetica caused by hip-huggers in a patient with aberrant course of the lateral femoral cutaneous nerve.

"Hip-huggers" may be a precipitating factor for meralgia paresthetica (MP), especially in thin persons with an aberrant pathway of the lateral femoral cutaneous nerve (LFCN). We describe a 25-year-old woman with a long-standing history of MP caused by an abnormal course of the LFCN and tight trousers, specifically hip-huggers. Ultrasonography was useful for detecting the lesion site and the abnormal pathway of the LFCN. After neurectomy of the LFCN, most of the symptoms of MP were relieved, but mild hypesthesia remained in the lateral thigh.

Clothing↗
PubMed1995

Classic neurogenic thoracic outlet syndrome in a competitive swimmer: a true scalenus anticus syndrome.

Neurogenic thoracic outlet syndrome (TOS) is caused by compression of the lower brachial plexus usually by a cervical rib or a fibrous band. We describe a 16-year-old girl with weakness and wasting of her right hand, which progressed over the ensuing years. She had been a competitive long distance freestyle and butterfly swimmer since age 8 years. A neurological exam at age 20, revealed severe atrophy and weakness of all intrinsic right hand muscles, more so of the thenar muscles, and hypesthesia along the ulnar aspect of the hand and forearm. EMG, which showed a severe chronic axon loss lower trunk brachial plexopathy with minimal fibrillations, was typical for classic neurogenic TOS. Chest and cervical spine X-rays and MRI of the cervical spine were normal. A supraclavicular exploration confirmed the absence of a cervical rib or band. The lower trunk was thickened under the scalenus anticus which was sectioned. Neurolysis was also done. She was advised to abandon swimming. A clinical and EMG follow-up 2.5 years later showed no significant changes. This is the first case of true neurogenic TOS caused by scalenus anticus compression occurring in a competitive swimmer.

Adolescent↗
PubMed1991

The epidemiology of headache among children with brain tumor. Headache in children with brain tumors. The Childhood Brain Tumor Consortium.

In this report we present aspects of the epidemiology of headache (i.e., pain in the head, face, ear, or neck) among children with brain tumors. The data are derived from the 3,291 subjects in the Childhood Brain Tumor Consortium databank. Overall, 62% of the children with brain tumors experienced chronic or frequent headaches prior to their first hospitalization: 58% of children with supratentorial tumors and 70% of children with infratentorial tumors. The relative frequency of headache increased through age 7 and then leveled off regardless of tumor location. For children under age 5, headache rarely had a duration of more than 1 year prior to hospitalization. Among children over age 4, headache duration of at least one year was significantly greater for supratentorial than for infratentorial tumors. Children with a brain tumor and headache had a different distribution of symptoms and neurologic signs than those without headache. Tumor location and headache status were interactively associated with the presence of other symptoms and neurologic signs. Children with headache had a greater number of other symptoms and neurologic signs. Regardless of tumor location among children with headache, nausea or vomiting, papilledema, and hypoactive tendon reflexes were more frequent, while upper extremity weakness, optic atrophy, and irritability were less frequent. Diplopia, coma, stiff neck, anesthesia or hypesthesia, pupillary abnormalities, and abnormalities of personality, academic performance, or speech were associated with headache in children with supratentorial tumors. No specific symptoms or neurologic signs were associated with headache in children with infratentorial tumors. Supratentorial craniopharyngioma, ependymoma, and protoplasmic astrocytoma were associated with significantly high rates of headache as was infratentorial pilocytic astrocytoma.

Adolescent↗
PubMed1994

Trigeminal neuralgia: differentiation between intracranial mass lesions and ordinary vascular compression as causative lesions.

We investigated 164 patients who presented with trigeminal neuralgia as their initial symptom at our institute. Twenty-two patients (13.4%) had intracranial causative lesions other than vascular compression. There were 19 tumors (10 epidermoids, 5 meningiomas, and 4 neurinomas) and 3 vascular lesions (2 arteriovenous malformations and 1 aneurysm). Eleven patients (50%) demonstrated only trigeminal neuralgia without any other neurological deficits. Preoperative computed tomography could not identify any causative lesions in 6 of these 22 patients. In 3 of the 6 patients, a causative lesion was clearly detected only by magnetic resonance imaging, while in 1 of the 6 patients an arteriovenous malformation was detected by angiography alone. Intraoperatively tumor was encountered in 3 cases even though preoperative computed tomography could not detect any apparent causative lesions. A higher incidence of hypesthesia in the trigeminal nerve regions as well as a reduced corneal reflex was noted in patients with a mass lesion compared to those with vascular compression. We thus conclude that magnetic resonance imaging should be performed on all patients who complain of trigeminal neuralgia in order to rule out mass lesions, however, angiography is still considered useful for the diagnosis of some vascular lesions.

Adult↗
PubMedDate not supplied

Mimetic modulation for problem creases of the face.

Problem creases of the face such as frontal lines and frown lines, crow's feet, deep nasolabial and perioral folds, and cervical bands may be caused by the aging process, excessive exposure to the sun, disease, or genetic disposition. The condition may become aggravated by habitual hyperkinesia of certain mimetic muscles like the frontalis, corrugators, orbicularis oculi, levatores labii superioris, zygomatici, and the platysma. The diagnosis is established clinically by electromyography and selective muscle and nerve blocks. In these cases we advocate regulation of the mimetic hyperkinesia through selective myotomy, myectomy, and neurotomy of the responsible mimetic muscles (mimetic modulation). These procedures may be performed exclusively or in combination with a blepharoplasty, rhytidectomy, or other procedure. Our experience with 60 patients over the past five years (medium = 3.5 years) is presented. Problems and complications such as paresthesia and hypesthesia, partial paresis and asymmetry, incomplete correction, and recurrences are discussed. We believe that mimetic modulation is a valuable concept in treating problem creases and thereby improves the results of the aesthetic surgery of the face.

Adult↗
PubMed1986

Generalized argyrosis in man: neurotological, ultrastructural and X-ray microanalytical findings.

Generalized argyrosis can produce a number of abnormalities, including skin discoloration, liver and kidney dysfunction. We describe a patient with generalized argyrosis following long-term self-treatment with oral silver intake, in whom skin discoloration, progressive taste and smell disorders, vertigo and hypesthesia were observed. These findings were confirmed by chemosensory tests and electrophysiological investigations. The development of hypogeusia was assessed by subjective tests, while the progression of hyposmia was followed by recording olfactory evoked cortical potentials. Light and electron microscopy of tissue samplings demonstrated electron-dense mineral deposits in basal membranes, in macrophages, in the perineurium of peripheral nerves, along elastic and collagenous fibers, and in necrotic cells of the oral submucosa. Silver and sulfur deposits in affected tissues could be defined by X-ray microanalysis. The quantitative ratio between silver and sulfur in involved tissues was similar to that of an inorganic silver-sulfide (Ag2S) standard. The minute increase in the sulfur content when compared to the inorganic standard suggested a sulfur containing organic matrix of the tissue precipitates. Our findings indicate that the affinity of silver for membrane and neuronal structures and the deposition of silver as an insoluble compound (Ag2S) induce the progression of clinical disease.

Ageusia↗
PubMed1983

Original construction of thermo-esthesiometer and its application to vibration disease.

A thermo-esthesiometer was devised for the investigation of sensory disorders among operators of vibration tools. Moreover, its usefulness for diagnosis was investigated. The esthesiometer was composed of a copper pipe 80 cm long that was heated at one end with an electric heater and chilled at the other end with a cooling mixture of NaCl and ice. The temperature at each point of the copper pipe was measured using a sliding surface-thermometer. It gave a sigmoidal temperature gradient between 8.5 degrees and 47.5 degrees C at a room temperature of 20 degrees C. It took about 30 min to complete a stable temperature gradient from the start of heating and cooling. The profile of the temperature gradient curve was a function of room temperature. The stability of the temperature gradient curve was to be maintained satisfactorily for more than 5 h at a constant room temperature below 28 degrees C. The size of this apparatus, when it was broken up, was small enough to carry around easily. Using this esthesiometer, the warm and cool thresholds were measured at the fingertips of operators of vibration tools. From the results obtained the hypesthesia of temperature sense was shown to correlate very strictly with the stage of vibration disease. In particular, an upward deviation of warm threshold and an increase of the width of the neutral zone were observed in those patients.

Adult↗
PubMed1994

Cranio-cervical decompression for Chiari I malformation. A retrospective evaluation of functional outcome with particular attention to the motor deficits.

We report a series of 26 patients affected by a Chiari I malformation treated at our department between 1987 and 1993. All patients underwent pre- and postoperative evaluation by magnetic resonance imaging (MRI). Sequential perioperative motor evoked potential (MEP) recordings were performed in 8 patients. The preoperative symptoms can be divided into four subgroups: cephalgias (84.6%), cranial nerve deficits (69.2%), motor deficits (76.9%), sensory deficits (73%). Twenty-five out of 26 patients underwent craniovertebral decompression, 1 a transoral anterior decompression. One patient died 2 months after surgery because of progressive pulmonary failure. We registered following postoperative complications transient hypoglossal palsy (1 case), vertigo (2 cases), meningitis (1 case), minor CSF leaks (3 cases). Cephalgias subsided in 17 and improved in 4 out of 22 patients. Cranial nerve deficits improved in 8 and were stabilized in 7 out of 18 patients. A limited recovery of trigeminal function was possibly due to nuclear lesions. Five patients whose vestibular disturbances were not relieved by surgery were put on a course of carbazepine. Vertigo resolved in one case and in two others improved. While hypesthesia improved after decompression, the other sensory deficits were stabilized in 5% of the patients. Spasticity improved in 12 out of 18 patients, but weakness only in 7 out of 17 patients. Motor disturbances were always detected by MEP-recording. MEP-characteristics were not specific, resembling those of patients with other intra-, extramedullary myelopathies. Functional recovery was not matched by an improvement of the MEP parameters. MEP may be used as tool for surveillance of patients whose clinical findings are not progressive and are not at first surgical candidates.

Adult↗
PubMed1985

Lipolysis: pitfalls and problems in a series of 1,246 procedures.

In a large series of lipolysis procedures, followed for 2 years, there have been no serious major and few minor complications. Selection of patients is important and for the best results patients should be under 30 years of age. Many areas of lipodystrophy may be dealt with at one session in most cases. However, postoperative circulatory control, especially during the first 24 hours, is very important and always requires vigilance to avoid the tendency to hypotension. Early local problems include tenderness, bruising, and swelling, and the management of these is discussed. Later complications include skin flaccidity, dimpling, and localized areas of pigmentation and hypesthesia. The prevention and management of all these sequelae and others are discussed.

Adipose Tissue↗
PubMed1982

[Results after surgical treatment of acute injuries to the lateral ligament of the ankle (author's transl)].

105 patients with acute injuries of the fibular collateral ligament were treated surgically. Diagnoses and following treatment were based on X-rays in stressed positions (talar tilt and anterior drawer sign) compared with the other side. More than fifty percent were athletic injuries. After surgical treatment patients wore a below-knee plaster cast for 6 weeks. 90 patients were seen after an average interval of 24 months; another 7 were assessed on the basis of patient's judgement. In 7 cases the X-ray control showed some instability (talar tilt greater than 5 degree and anterior drawer sign greater than 2mm). At physical examination we found in some cases minor differences in mobility, swelling and pain. 14 patients showed hypesthesia on the lateral side of the dorsum of the foot. No patient had to give up his sport activities due to the ligamentous injury, 89 (97 were asked) were content with the result.

Adult↗
PubMed2001

Spinal intramedullary metastases. Report of two cases and review of the literature.

Two cases of intramedullary metastasis were observed in our department within a short period of time that change the picture of uncommon locations of spinal metastases as described in literature. The patients presented primarily with rapidly progressing flaccid paraparesis of the lower extremities. Both additionally described diffusely located hypesthesia and pallhypesthesia of the limbs. One complained about sphincter disturbance. Both were treated by laminectomy and microsurgical extirpation of the tumor. In the first case reported here, an intramedullary metastasis of an epithelioid sarcoma in the conus medullaris region was ascertained which may be regarded as the first such reported case. In the second case report, histologic findings confirmed a thoracic intramedullary metastasis of a mastocarcinoma. At follow-up 6 and 8 months postoperatively, we observed full recovery of neurologic function in one patient and partial recovery in the other. Intramedullary metastases are extremely rare. Clinical presentation may show rapid onset and outcome is related to rapid resection and adjuvant therapy. In contrast to intracerebral metastases, this is a remarkable incidence of sarcomatous intramedullary metastatic disease.

Breast Neoplasms↗
PubMed2002

[Extramedullary hematopoiesis in the paravertebral space].

BACKGROUND: Extramedullary hematopoiesis is a response of the organism to a deficient production of blood-forming cells within the bone marrow. It may coincide with some hematologic diseases. Two patients with paravertebral mass lesions representing extramedullary hematopoiesis are discussed. Characteristic findings of extramedullary hematopoiesis are presented along with a review of the literature on this topic. CASE REPORTS: A 76-year-old male with a known myelodysplastic syndrome presented with pneumonia. In addition, he had symptoms of a cauda equina syndrome with complaints of pain and hypesthesia of the lower limbs as well as urinary retention. A 63-year-old female presented with aggravated complaints of the lower thorax, low back pain radiating to the upper left leg, and dysesthesia of both feet. In her past medical history, she had polycythemia vera and a splenectomy. Both patients showed paravertebral and intraspinal lesions located in the thoracic and sacral spine which were regarded as extramedullary hematopoiesis according to the imaging findings in MRI and CT. Radiation therapy showed marked improvement in their neurologic complaints following the initial sessions. CONCLUSION: Clinical presentation, knowledge of the underlying disease and of imaging findings are essential in the diagnosis of extramedullary hematopoiesis. MRI is the imaging modality of choice in the primary diagnosis of extramedullary hematopoiesis. Possible extension of the disease into the intraspinal space can be evaluated with high accuracy and differential diagnosis can be facilitated. In addition, MRI is of use in the accurate planning of radiation fields as well as during follow-up of extramedullary hematopoiesis.

Aged↗
PubMed1997

[The differential indication for "open" or "endoscopic" carpal tunnel release].

Carpal tunnel syndrome is one of the most common nerve compression syndromes. Nocturnal paresthesia and pain are early symptoms, and hypesthesia and thenar atrophy are late symptoms. These days endoscopic and open operation techniques are available. Minimal trauma after endoscopic release allows early return to work. The endoscopic technique should be performed by experienced surgeons to avoid severe complications.

Adult↗
PubMed2001

[Minimally invasive surgical therapy of gynecomastia: liposuction and exeresis technique].

INTRODUCTION: A number of techniques are available for the correction of gynecomastia. Nonscarring sparing methods are preferred, and the minimally invasive technique is to use liposuction for the gland and the fatty tissue exclusively. In this retrospective study we present our experience with a combination of liposuction and subsequent resection of the remaining gland. METHODS: Sixty-two patients (112 breasts) were surgically treated for gynecomastia from January 1996 and September 2000. From 1996 to 1997 all patients suffering from gynecomastia grade Simon I-II were treated by the method described by Rosenberg and Stark, which is exclusively suction of the fatty and glandular tissue. In a retrospective chart study a high recurrence rate was found in these patients. Subsequently we changed our technique to liposuction of the fatty tissue followed by sharp excision of the glandular tissue through the incision made for the liposuction cannula in the submammary fold. RESULTS: Suction alone was not sufficient to remove the glandular tissue; the rate of recurrence after suction was 35%. When sharp resection of the glandular tissue was carried out after the liposuction the recurrence rate dropped to under 10%. In total our complication rate was 50% including minor sequelae. The most frequent complication was unacceptable scarring of the nipple-areola complex. Hypesthesia of the nipple-areola occurred in 13.4% of the patients. CONCLUSION: The combination of liposuction and resection of the glandular tissue is a minimally invasive correction that can be used in all cases of gynecomastia grade Simon I-II.

Adult↗
PubMed2005

[Minimally invasive otoplasty].

OBJECTIVE: We have further developed a method, first published in 1967 by Kaye but not widely used later, to correct protruding ears. To our knowledge, clinical experience with a closed otoplasty technique based on a large number of patients has not yet been published. SUBJECTS AND METHODS: Of 442 ears in 4.5 years, 385 (200 patients, 15 one sided) were corrected using the minimally invasive technique. The key part of this technique to such otoplasty is the creation of a naturally appearing antihelix by combining permanent buried sutures and a conservative cartilage-breaking technique, both applied without extensive skin incisions and excisions. RESULTS: A total of 154 of the patients (i.e., with six one sided corrections, 302 ears) could be examined at least 3 months after surgery. Complications were revisions for either a partial recurrence or a still insufficient correction in the opinion of the patient (30/302, 9.9%), reactions to the suture material (26/302, 9.3%), early infections (2/385, 0.7%), prolonged sensitivity (2/302, 0.7%) and one hypertrophic scar in the lobule (1/302, 0.3%). There was no bleeding, hematoma, hypesthesia, sensitivity to temperature or skin reactions in the auricle. CONCLUSIONS: Minimally invasive otoplasty is a surgical technique that allows the correction of most protruding ears. Very strong cartilage or a very high lateral conchal wall set the limits to such an approach. We found the cosmetic results and complication rates to match those of open methods. Since patient comfort seems to be higher, the method is better accepted.

Adolescent↗