Hereditary neuropathy and vocal cord paralysis in a man with childhood diphtheria.

We present the case of a 37-year-old Afghani man with a history of childhood diphtheria, who was diagnosed with bilateral vocal cord paralysis at age 15 years. At about this time he developed progressive muscular wasting and distally predominant weakness, and subsequently developed respiratory insufficiency, necessitating nocturnal ventilatory support. His examination suggested a distal symmetric sensorimotor neuropathy, and his brother was similarly affected, although to a lesser degree. Electromyography (EMG) and nerve conduction studies revealed this process to be purely axonal. A diagnosis of possible hereditary motor and sensory neuropathy (HMSN) type IIc, hereditary axonal polyneuropathy with vocal cord paralysis, is proposed, although the question of early diphtheritic involvement of the vocal cords and peripheral nerves is also considered.

Treatment of mononeuropathy multiplex in hepatitis C virus and cryoglobulinemia.

Recent reports advocate alpha-interferon (alpha-Ifn) treatment for mononeuropathy multiplex in hepatitis C virus-associated cryoglobulinemia. We report 2 patients with this disorder to describe two underrecognized treatment outcomes--worsening of polyneuropathy with initiation of alpha-Ifn, in the absence of immunosuppression, and deterioration of liver function with prednisone, despite improvement of polyneuropathy.

Clozapine-induced myotoxicity in patients with chronic psychotic disorders.

Muscle dysfunction related to clozapine treatment is largely unrecognized. We evaluated weekly creatine kinase (CK) levels in 37 consecutive clozapine-treated outpatients with chronic psychotic disorders. Those with CK elevations underwent clinical neurologic evaluation, electromyography (EMG), and nerve conduction studies. Patients with probable myopathy had a quadriceps muscle biopsy. Twenty control patients had a single CK level determination. Twenty-nine of 37 clozapine-treated patients had CK elevations. Three patients had extreme CK elevations (> 20,000 IU/L), without myoglobinuria. Mean CK levels were significantly greater in clozapine patients (194 IU/L) than in control patients (142.3, p = 0.033). Of 18 clozapine-treated patients evaluated clinically, 6 had mild proximal weakness. EMG in 13 patients was myopathic in 5, normal in 5, and neurogenic in 3. Muscle biopsy in 5 patients showed rare regenerating myofibers and mild acute denervation (1), mild type II fiber atrophy (1), minimal acute denervation (1), and normal muscle (2). In conclusion, clozapine therapy may be associated with CK elevations and, rarely, mild myopathy.

Extraocular muscle involvement in Becker muscular dystrophy.

We report limitation of gaze and slow saccadic eye movements by clinical examination and video-oculography in a patient with Becker muscular dystrophy. This rare association suggests that a dystrophinopathy should be considered in a patient with features characteristic of Becker muscular dystrophy even when mild impairment of eye movements is present.

Histopathology of cerebral toxoplasmosis in human immunodeficiency virus infection: a comparison between patients with early-onset and late-onset acquired immunodeficiency syndrome.

We reviewed the histological features of untreated toxoplasmosis in 18 cases with the acquired immunodeficiency syndrome (AIDS), eight of which were surgical biopsies and 10 of which were autopsy specimens. The results were compared according to the clinical status of the patient at the time the diagnosis of toxoplasmosis was made (early-onset v late-onset AIDS) and according to the source of the specimen (surgical biopsy specimen v autopsy specimen). Cerebral toxoplasmosis was the AIDS-defining illness in half of the cases (six surgical biopsy specimens and three autopsy specimens). Inflammation in these cases was moderate in 44% and severe in 56%. Fibrous capsules were found in five cases. Lymphocytes and plasma cells were more prominent than neutrophils. Cerebral toxoplasmosis developed in or was part of the terminal AIDS illness in the remaining nine cases (two surgical biopsy specimens and seven autopsy specimens). In this group inflammation was sparse in 44%, moderate in 55%, and severe in only 11%. Fibrous capsules were usually absent and neutrophils were the predominant cell type. Comparisons between surgical biopsy specimens and autopsy specimens showed moderate to severe inflammation and frequent fibrous encapsulation in all of the former specimens but only in those autopsy specimens in which toxoplasmosis was the initial manifestation of AIDS. Thus, this study demonstrates varied neuropathological patterns of untreated cerebral toxoplasmosis in patients with AIDS and correlates the inflammatory response in the brain with the clinical stage of the patient's human immunodeficiency syndrome (HIV) infection. Inflammation and fibrous encapsulation were common only in patients with early-onset AIDS in whom cerebral toxoplasmosis was the first manifestation of the illness. This study highlights important differences between the histology of this infection at surgical biopsy and at autopsy, and stresses the need to consider toxoplasma as a potential cause of encapsulated brain abscesses.

[Detectability of pulmonary coin lesions: a comparative assessment of the image quality of a storage phosphor system and a conventional film screen system]. / Erkennbarkeit von Lungenrundherden: Vergleichende Beurteilung der Bildqualität von Speicherleucht-stoffolien mit einem konventionellen Film-Foliensystem.

The object of this study was to compare the prototype of a storage phosphor digital radiography system (Siemens) which allows postprocessing image data adjustment with the stored information, with an optimised conventional film system with respect of image quality and diagnostic accuracy. The test objects were pulmonary coin lesions as the search for primary or secondary lung tumours is one of the most frequent clinical requests and the detectability of coin lesions depends very much on their relative contrast against the surrounding area. In order to achieve reproducible results, an anthropomorphic lung phantom was used. Besides the conventional film radiograms the unprocessed digital radiograms and twelve series with variations of the processing parameters were evaluated. To achieve a homogeneous radiation image an anatomically shaped lead-loaded acrylic glass compensation filter was used. With regard to the true-positive demonstration of coin lesions there were no significant differences between the chosen film system and all digital radiograms. Image processing had no recognisable influence on the demonstration of the findings. Coin lesions of 3 mm diameter were detected only by chance; the detectability of lesions of 5 mm and 10 mm in diameter respectively depended mainly on their localisation. Lesions of 15 mm diameter were detected independent of their localisation. The results indicate that the storage phosphorus radiography system is already comparable to an optimised conventional film system concerning the conspicuity of coin lesions. If the image is homogenised by means of an anatomically shaped lead-loaded acrylic glass compensation filter, selective postprocessing adjustment of the digital radiograms for the evaluation of the mediastinum or the lung parenchyma is unnecessary.

[Roentgen findings in T-drainage--results of 311 patients]. / Röntgenbefunde bei T-Drainagen--Resultate von 311 Patienten.

Postoperative T Tube cholangiographies of 311 patients from 1980 to February 1992 were evaluated by studying the patient histories and x-ray examinations. Most frequent questions concerned residual stones and papillary occlusion. Most frequent pathological findings were dilatation of the choledochus and of the bile ducts, residual stones and paravasations. Fistulas, abscesses and signs of cholangitis rarely occurred. Dilatation of the choledochus alone did not lead to further treatment. Papillary occlusion was treated by leaving the T drain in situ, patients with occlusion and residual stones were treated by papillotomy, litholysis or lithotripsy. Only 5 patients with necrotizing pancreatitis and abscess had to be reoperated. T tube cholangiography, which at the beginning of the study was a routine postoperative control with minor complications, will surely be applied less frequently in the future due to advances in endoscopy.

[Results of computerized tomography after Whipple operation of chronic pancreatitis]. / Ergebnisse der Computertomographie nach Whipplescher Operation bei chronischer Pankreatitis.

53 patients were controlled with CT after Whipple resection. The median age of the patients was 49 years, the median time after surgery nine years. Diagnostic criteria were the identification of the residual pancreas, signs of pancreatic atrophy and radiological signs of chronic pancreatitis. CT was performed with slice thickness of 4 mm and steps of 4 mm. The residual pancreas was visible in all 53 cases. Pancreatic atrophy was found in 26 cases, in four of these the residual pancreas was only 2 mm in size. Signs of chronic pancreatitis were found in 16 cases. By comparing preoperative and postoperative CT-scans, seven progresses of pancreatitis were detected. Signs of acute pancreatitis were not found in the 53 patients. Fatty liver degeneration was found in 7.5%. We found that CT is the most reliable technique to visualize the pancreas after Whipple resection. CT should be performed in all patients with clinical symptoms of an acute pancreatitis. CT should also be performed in patients with residual pancreas pseudocysts. There is no indication for CT in the routine examination of asymptomatic patients after Whipple resection.

[Is the use of hyperosmolar oral contrast media still justifiable today?]. / Ist der Einsatz hyperosmolarer oraler Kontrastmittel heute noch gerechtfertigt?

The application of oral hyperosmolar contrast media, as they are used for the control of anastomoses after operations on the upper intestinal tract and for the diagnosis of ileus, is still controversial today because of possible side effects. We examined 53 patients postoperatively, after abdominal operations and within the framework of the diagnosis of ileus, to find out whether there were any problems with the fluid and electrolyte balance, and whether side-effects such as nausea, vomiting, pain and diarrhoea were still tolerable. The patients did not classify their subjective complaints as very serious. None of our patients showed clinically manifest disorders of the fluid and electrolyte balance. The increased frequency of bowel movement, caused by the contrast agent, was required for the treatment of postoperative bowel atonia and the condition of ileus or sub-ileus, if present. We still think the application of hyperosmolar contrast media can be justified, and hence we restrict the use of the much more expensive iso-osmolar contrast agents to patients who are at risk in respect to aspiration and unfit to cooperate.

[Comparison of CT and plain tomography in hilar and lung processes]. / Vergleich von CT und Verwischungstomographie bei Hilus- und Lungenprozessen.

A group of 100 patients including 46 patients with suspected bronchogenic carcinoma and 54 patients with suspected lung metastases was evaluated by conventional plain tomography. Patients with preoperatively suspected T4- or N3-categories were excluded. The diagnosis was confirmed in all cases by surgery and histology. In addition, patients with lung metastases were controlled by follow-up chest x-rays (median follow-up time 1.5 years). The diagnosis of plain tomography was true positive in 70% and false in 30%. CT was true positive in 57% and incorrect in 43%. Tumors or metastases were detected with both methods when more than 5 mm in diameter, lesions smaller than 5 mm were overlooked with CT and tomography. Plain tomography an CT did not detect lymph nodes metastases smaller than 1.0 cm.

[A comparison of arthrography and magnetic resonance tomography in painful limited movements of the hand]. / Vergleich Arthrographie--Kernspintomographie bei schmerzhaften Bewegungseinschränkungen der Hand.

In 18 patients with unclear pain in the wrist limiting hand movement, arthrography and magnetic resonance imaging (MRI) were compared. The FISP gradient echo sequence turned out to be superior to the T1 weighted spin-echo sequence in the imaging of the triangular fibrocartilage complex. MRI is very sensitive in detecting triangular fibrocartilage lesions and therefore represents a suitable non-invasive screening method. It is, however, not possible to assess the degree of the lesion. Ruptures in carpal tendons could only be diagnosed by means of arthrography.

[Adaptability of fine-needle biopsy for preoperative and postoperative studies in femoro-crural vascular reconstruction]. / Eignung der Feinnadelpunktion (FNP) zur prä- und postoperativen Darstellung bei femoro-kruralen Gefäss-Rekonstruktionen.

During two years we performed 199 pre- and postoperative angiograms on 99 patients with femoro-crural reconstruction. 133 of these were by FNP. In 4.2% of postoperative FNPs (fully anticoagulated patients), there was significant bleeding from the puncture or the operative scar. It was not necessary to reoperate on any bypasses and there were no late complications. As regards the demonstration of the calf vessels. FNP proved itself superior as a pre- and postoperative method. Because of the rapidity of the method, the small amount of contrast required and the minimal trauma, FNP is the method of choice for pre- and post-operative studies of the calf vessels for femoro-crural reconstructions.

Smooth muscle cells can express cytokeratins of "simple" epithelium. Immunocytochemical and biochemical studies in vitro and in vivo.

Cytokeratins are a set of 19 proteins that together constitute the class of intermediate filament protein expressed by epithelial cells and tumors. Using a panel of 9 different monoclonal anti-cytokeratin antibodies, the authors have performed immunocytochemistry on methanol-fixed, frozen sections and methacarn-fixed, paraffin-embedded tissue of human myometrial specimens. Anomalous cytokeratin expression (ACE) by smooth muscle cells was found in all specimens. Immunoblots of this tissue confirmed the presence of cytokeratin 19, and possibly 8. In addition, immunocytochemical studies demonstrated ACE in human fetal tissues within the intestinal muscularis and the heart, especially in the region of the aortic outflow tract, and in 8 of 19 cases of leiomyosarcoma from adults. Indirect immunofluorescence studies were also performed on cells explanted from myometrial tissue; the overwhelming majority of cells derived from these cultures were smooth muscle cells as verified by expression of muscle actins, and a subpopulation of these cells was found to be cytokeratin-positive. ACE was confirmed in vitro by double labeling experiments demonstrating simultaneous expression of muscle actins and cytokeratins within the same cell. The significance of this smooth muscle cell ACE is unknown, but it may be a phenotypic marker of smooth muscle in a proliferative state. ACE could be a source of confusion in the immunocytochemical analysis of poorly differentiated malignancies if a complete panel of antibodies is not employed.

Chart review of alcohol problems in adolescent psychiatric patients in an emergency room.

Of 459 consecutive teenage patients seen for psychiatric evaluations in a general hospital emergency room during a 1-year period, 76 (17%) had blood alcohol levels over 100 mg/dl. Males and females were represented equally in this group. The most common DSM-III diagnoses in these patients were affective disorders and conduct disorders.