Neurophysiological assessment of children with obstetric brachial plexus palsy]

INTRODUCTION: The objectives of the neurophysiological evaluation of infants with brachial plexus palsy are to determine the time of occurrence of the lesion, to locate the lesion and to determine its course. METHODS AND CONCLUSIONS: These objectives are achieved by studying affected upper extremity muscles by needle electromiography (EMG) and affected nerves by motor and sensory conduction studies. EMG is performed in the first week of life in those patients with brachial plexus palsy of unknown etiology to determine the age of the lesion for medico-legal reasons. EMG is performed before surgery for tendon transfer in the selected muscles to assure that they are normal. EMG and motor and sensory conduction studies are performed at the age of 3 and 6 months in infants with less than 4 muscle weakness to determine candidates for surgical exploration. Motor and sensory nerve conduction studies are performed intraoperative to determine the functional status of the affected axons and the best surgical procedure (neurotization, neurolysis and/or neuroma resection and homologous nerve graft).     

A new evaluation system to predict the sequelae of late obstetric brachial plexus palsy

Xklp2 is a plus end-directed Xenopus kinesin-like protein localized at spindle poles and required for centrosome separation during spindle assembly in Xenopus egg extracts. A glutathione-S-transferase fusion protein containing the COOH-terminal domain of Xklp2 (GST-Xklp2-Tail) was previously found to localize to spindle poles (Boleti, H., E. Karsenti, and I. Vernos. 1996. Cell. 84:49-59). Now, we have examined the mechanism of localization of GST-Xklp2-Tail. Immunofluorescence and electron microscopy showed that Xklp2 and GST-Xklp2-Tail localize specifically to the minus ends of spindle pole and aster microtubules in mitotic, but not in interphase, Xenopus egg extracts. We found that dimerization and a COOH-terminal leucine zipper are required for this localization: a single point mutation in the leucine zipper prevented targeting. The mechanism of localization is complex and two additional factors in mitotic egg extracts are required for the targeting of GST-Xklp2-Tail to microtubule minus ends: (a) a novel 100-kD microtubule-associated protein that we named TPX2 (Targeting protein for Xklp2) that mediates the binding of GST-Xklp2-Tail to microtubules and (b) the dynein-dynactin complex that is required for the accumulation of GST-Xklp2-Tail at microtubule minus ends. We propose two molecular mechanisms that could account for the localization of Xklp2 to microtubule minus ends.     

A new strategy of muscle transposition for treatment of shoulder deformity caused by obstetric brachial plexus palsy

Cross-innervation (caused by misdirection of regenerated axons), muscular imbalance (caused by muscle paresis or earlier recovery), and growth are the three main causes of shoulder deformity due to obstetric brachial plexus palsy. If perioperative studies demonstrate the existence of muscle recovery by cross-innervation, a new strategy of muscle transposition to minimize the influence of cross-innervation is used. Release of antagonistic muscles (pectoralis major and teres major muscles) and augmentation of paretic muscles (transferring teres major to the infraspinatus muscle, reinserting both ends of the clavicular part of the pectoralis major muscle laterally) are performed for reconstruction. Since 1993, 29 patients having shoulder deformity caused by obstetric brachial plexus palsy underwent reconstruction utilizing this strategy of muscle transposition. The timing for the reconstruction was at an average of 8.5 years (range, 4 to 21 years). The average shoulder abduction following the muscle transposition was 151 degrees (i.e., average gain 104 percent, or 77 degrees) and that of external rotation was 72 degrees (average gain 200 percent, or 48 degrees). Compared with the patients who had no surgery for shoulder deformity caused by obstetric brachial plexus palsy and early nerve surgery for the infant obstetric brachial plexus palsy, the results of the strategy seem to be significantly impressive.     

Fetal inguinoscrotal hernia: sonographic diagnosis and obstetric management

Fetal inguinal hernia is a rare antenatal diagnosis. We present the sonographic features and outcome of a fetus diagnosed at 36 weeks' gestation as having an (indirect) inguinoscrotal hernia. Sonographic criteria for antenatal diagnosis are discussed within the context of differential diagnoses raised by the presence of a perineal mass. Guidelines for obstetric management and counselling are suggested.     

Prenatal diagnosis of a fetal head and neck neoplasm: differential diagnosis, management, and histology

A fetal head and neck malignancy was prenatally diagnosed. The parents allowed the fetus to die during labour, due to the poor prognosis. We discuss the corresponding pathology findings, differential diagnosis, and management of this rare entity. Prenatal diagnosis of fetal neoplasms theoretically improves outcome, although this was not true in our case.     

Clinical picture and differential diagnosis of cardiomyopathy and myocarditis

BACKGROUND: The purpose of this prospectively randomized study was to evaluate intraoperative as well as long-term performance of different coating materials for knitted Dacron aortobifemoral prostheses. PATIENTS AND METHODS: Between 1989 and 1992 a total of 150 consecutive patients who underwent aortobifemoral bypass were randomized for three different coating materials: gelatine (Unigraft; n = 49), collagen (Hemashield; n = 50) and human albumin (USCI; n = 51). Intra- and perioperative data such as duration of operation, clamping time, blood loss, and early complications were obtained as well as yearly follow-up examinations up to five years. Frequency of late complications, graft patency, and patient survival were observed. RESULTS: No statistically significant difference of intraoperative data could be obtained. In every group 6% of patients presented with anastomotic aneurysm in the groin. Primary patency rates were Unigraft 92%, Hemashield 91%, USCI 92% (no significant difference, n.s.), secondary patency rates after 5 years were Unigraft 98%, Hemashield 95%, USCI 94% (n.s.). CONCLUSIONS: Overall superiority of one particular type of prosthesis could not be stated. In order to prevent sudden graft failure associated with progressive arteriosclerotic disease or from late anastomotic aneurysm regular follow-up examinations should follow aorto-bifemoral bypass.     

Oral manifestations and differential diagnosis of isolated hypoglossal nerve palsy: report of two cases

Isolated hypoglossal nerve palsy is rare, but occasionally it appears as the initial or solitary sign of an intracranial or extracranial space-occupying lesion, a head and neck injury, or a vascular abnormality of the internal carotid artery. Therefore it should be considered in differential diagnosis. We report two cases of isolated unilateral hypoglossal nerve palsy. In Case 1 the cause of the palsy appeared to be hypoglossal nerve neurilemmoma within the hypoglossal canal, whereas in Case 2 the cause could not be identified. Neither patient complained of any disability other than slight dysarthria. The tongue deviated toward the healthy side at rest and toward the affected side on protrusion. Hemiatrophy of the tongue with fatty displacement was demonstrated by means of T1-weighted magnetic resonance imaging. Dentists who might at times see patients with isolated hypoglossal nerve palsy should be aware of the significance of its oral manifestation, and they should be able to perform differential diagnosis of patients with the condition who appear for treatment.     

Dementias--diagnosis, differential diagnosis and therapy

The most common cause of primary (degenerative) dementia in old age is Alzheimer's disease, with vascular forms of dementia taking second place. Endocrinopathies, normal pressure hydrocephalus and space-occupying lesions are frequent causes of secondary dementia. A multitude of further, more rare clinical presentations results in a wide differential diagnostic spectrum necessitating a careful diagnostic work-up of the demential syndrome before considering treatment. This work-up must include clinical, laboratory and equipment-based investigations. The multimodal therapeutic approach to dementia covers not only the elimination of treatable underlying causal conditions and the medical treatment of cognitive symptoms and psychiatric accompanying symptoms, but also non-medicamentous aspects.     

Migraine--diagnosis, differential diagnosis and therapy

Migraine is caused by intermittent brain dysfunction. Attacks result in severe unilateral headache with nausea, vomiting, photophobia, phonophobia and general weakness. The prevalence of migraine is 12 to 20% in women and 8 to 12% in man. Treatment of an acute attack is done by antiemetics in combination with analgesics. Severe migraine attacks are treated with ergotamine or sumatriptan. Parenteral treatment is performed most efficiently and safely with i.v. ASA. Frequent and severe attacks require prophylaxis. Drugs of first choice are metoprolol, propranolol, flunarizine and cyclandelate. Substances of second choice are valproic acid, DHE, pizotifen, methysergide and magnesium. Homeopathic remedies are not superior to placebo. Nonpharmacological treatment consists of sport therapy and muscle relaxation techniques.     

Oral ulcerations in individuals infected with human immunodeficiency virus: clinical presentations, diagnosis, management, and relevance to disease progression

Oral ulcerations can be both a localized disease entity and a manifestation of an underlying systemic condition. In individuals infected with the human immunodeficiency virus, oral ulceration may serve as a marker for a specific systemic illness, early immunosuppression, and disease progression. This article reviews clinical manifestations, diagnosis, and management of oral ulcerations in individuals with the human immunodeficiency virus.     

Common presentations and management of leukaemia in childhood

The curability of childhood leukaemia (acute lymphoblastic, acute non lymphoblastic and chronic myeloid) has improved dramatically over the past 25 years. The author reviews the clinical presentations and current therapeutic approaches in broad principle. The importance of supportive care (both at the time of diagnosis and during therapy) and the potential for long-term side effects relevant to adult life are discussed.     

Typical and atypical presentations of gastroesophageal reflux disease. The role of esophageal testing in diagnosis and management

Gastroesophageal reflux disease (GERD) is a common disease with many typical and atypical forms of presentation. In the classic presentations of GERD with heartburn and regurgitation, esophageal testing, except for endoscopy, is only required for poorly responding patients or prior to surgical therapy. The atypical presentations of GERD, including chest pain, asthma, and ear, nose, and throat complaints, frequently are not associated with heartburn or regurgitation. Esophageal testing, particularly 24-hour pH monitoring is key to making the diagnosis and ensuring adequate acid suppression.     

Dysphagia. Diagnosis, differential diagnosis

The wide scope of differential diagnosis in dysphagia makes interdisplinary team-work essential. The aim of this review therefore is to show the various specialties of medicine dealing with dysphagia and to point out possible causes and therapeutic measures.     

Obstetrical brachial plexus palsy: results following neurolysis of conducting neuromas-in-continuity

Sixteen infants with conducting neuromas-in-continuity at primary brachial plexus exploration underwent microsurgical neurolysis of their lesions. For each patient, the immediate preoperative scores for individual joint movements were compared with scores at the last examination. In the Erb's palsy group (n = 9), significant improvement was seen in shoulder movements, elbow flexion, supination, and wrist extension (paired t test, p < 0.05). Clinically useful improvements in function was seen at the shoulder and elbow (Fisher's exact test, p < 0.05). In the total palsy group (n = 7), significant improvement in shoulder abduction, shoulder adduction, elbow flexion, and extension of the wrist, fingers, and thumb was seen (paired t test, p < 0.05), but there was no significant improvement in the proportion of patients with useful functional outcomes. Neurolysis in Erb's palsy improves both muscle grade and the functional ability of patients. Neurolysis does not provide useful functional recovery in patients with total plexus palsy.     

Hypoglycemia. Symptoms, differential diagnosis, therapy

The article deals with epidemiologic evaluation of long-term chemical effects. The authors stress difficulties in setting the "cause-effect" relationships and define some typical mistakes in such research.