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Biomedical subjects

R Rohkamm

Publications and source records attributed to R Rohkamm.

17 recordsLinked to original sources
PubMed1997

Solitary Langerhans cell histiocytosis lesion of the parieto-occipital lobe: a case report and review of the literature.

A 38 year-old woman with a solitary intracerebral Langerhans cell histocytosis (LCH) lesion is presented, in whom, cerebral magnetic resonance imaging (MRI) revealed a non-enhancing mass in the right parieto-occipital lobe. The surgical specimen consisted of a nodular polymorphic infiltrate of mononuclear histiocytic cells, macrophages, eosinophilic granulocytes, lymphocytes and Langerhans giant cells involving leptomeninges, cerebral cortex and white matter. The histiocytes displayed features of Langerhans cells such as CD1a and S-100 immunopositivity, and of reticulum cells such as Ki-M4P and X-12 immunopositivity. This case as well as ten other cases from the literature are reviewed.

Adult↗
PubMed1989

Rippling muscle disease.

Six patients from two families with an autosomal dominantly inherited disease, apparently a myopathy, are described. Their major complaint was muscle stiffness, primarily in the legs. The muscles displayed an unusual sensitivity to stretch, manifested by rippling waves of muscle contraction. These rippling contractions were not accompanied by muscle fiber action potentials. Nonspecific, mild abnormalities were seen on muscle biopsy. These findings raise the possibility that there is an intracellular derangement in the muscle fiber responsible for the muscle rippling; further studies are necessary to establish the underlying pathophysiologic condition.

Adult↗
PubMed1988

Hypertrophy of the calf with S-1 radiculopathy.

Occasionally, chronic denervation is associated with enlargement rather than atrophy of muscle tissue. We studied seven patients with S-1 radiculopathy who developed ipsilateral enlargement of the calf. On the basis of results of calf muscle biopsies, it seems likely that the enlargement was due to muscle fiber hypertrophy and atrophy combined with an increase in connective tissue. Computed tomograms of the legs revealed no evidence of tumor. Surgical decompression of the involved S-1 nerve root had no obviously beneficial effect in reducing the calf enlargement. More information is needed to define the natural course of the calf enlargement and to clarify the pathophysiologic processes involved.

Adult↗
PubMed1988

The use of endomyocardial biopsy in heart failure.

Endomyocardial biopsy in this study of 1250 biopsied patients (mean of five samples/patient) proved to be a remarkably safe technique with no lethal complications. It may help to detect the underlying cause of heart failure but is handicapped by sampling error in focal disease processes (such as myocarditis and sarcoid heart disease) when conventional light and electron microscopy are used. In this biopsy series 123 patients (9.8%) suffered from severe heart failure; lymphocytic infiltrates were found in only 10 (8%). Immunohistological data suggested a secondary humoral immunopathogenesis in all patients with myocarditis and perimyocarditis, in 75% of patients with postmyocarditic heart muscle disease and in 48% of patients with primary dilated cardiomyopathy. There may thus be a need for a new classification of heart muscle diseases that includes immunological parameters of humoral and cellular autoreactivity.

Biopsy↗
PubMed1987

Infantile glycerol kinase deficiency--a condition requiring prompt identification. Clinical, biochemical, and morphological findings in two cases.

Infantile glycerol kinase deficiency (GKD) is an X-linked genetic disease characterized clinically by adrenal insufficiency and muscular dystrophy. The enzyme defect leads to increased levels of glycerol in blood and urine, which can be used for diagnosis. Without recognition of this condition, the chances for life-saving steroid treatment and for genetic counselling are missed. We report clinical, endocrinological, biochemical, and morphological findings in two non-related boys. One of them died in early infancy. The other is thriving at the age of 2 years although he is suffering from a myopathy not distinguishable from Duchenne muscular dystrophy. We discuss when to suspect and how to confirm the diagnosis of infantile GKD, and under what precautions the condition is detectable by commonly used screening procedures for inborn errors of metabolism.

Adrenal Insufficiency↗
PubMed1987

Fixation of neural tissue for electron microscopy with an electronically controlled perfusion pump.

Many attempts to improve the perfusion of mammalian tissues aim at changes of the osmotic pressure. We describe a method for fixation of nervous tissues controlling both the hydrostatic pressure and the flow rate of a perfusion solution. The constancy of these parameters is guaranteed by an electronically controlled perfusion pump. Thus, a more uniform and complete preservation can be achieved. Further advantages of this method include provision for a rapid succession of rinsing and fixation solution and a continuous control of the hydrostatic pressure during perfusion.

Animals↗
PubMed1986

[Nuclear magnetic resonance tomography in the diagnosis of muscular diseases].

Forty-nine patients with various systemic muscle diseases were examined by MR using a 1 Tesla magnet and the appearances of different conditions are analysed. Emphasis was placed on the analysis of patients with progressive muscular dystrophies, myositis, myotonia dystrophica and other muscle diseases. The investigation was begun in March 1984 and was continued until September 1985. Certain characteristic patterns of selectively involved muscles could be recognised. The pattern corresponds to our present understanding of the early phases of muscle diseases, whether inflammatory or due to fatty degeneration. The T1 and T2 relaxation times in various patients were quantified and changes in the normal pattern were analysed. Attention is drawn to the value of MR when carrying out a biopsy and for treatment of muscle diseases.

Adult↗
PubMed1986

Adynamia episodica and paralysis periodica paramyotonica.

We studied hyperkalemic attacks in one family with adynamia episodica (AE) and one family with paralysis periodica paramyotonica (PPP). Under exercise, serum potassium increased as in healthy subjects. Thiazide did not affect this increase. Thirty minutes after exercise, a second potassium increase occurred, but could be prevented by thiazide and not by mexiletine. After cooling, muscle relaxation time was normal in AE but increased up to 100 times in PPP; this cooling effect was prevented by mexiletine. Although hyperkalemic attacks are similar in AE and in PPP, the membrane defect in PPP seems more complex.

Adult↗
PubMed1983

Different effectiveness of tocainide and hydrochlorothiazide in paramyotonia congenita with hyperkalemic episodic paralysis.

We investigated the effectiveness of tocainide and hydrochlorothiazide on muscular symptoms in a patient with paramyotonia congenita and episodic attacks of hyperkalemic paralysis. Generalized weakness was evoked by exercise and potassium loading. Myotonia and weakness were evoked by local muscle cooling. Tocainide prevented myotonia and weakness induced by cooling, but failed to prevent hyperkalemic weakness. Hydrochlorothiazide prevented hyperkalemic weakness, but did not influence symptoms evoked by cooling. These results suggest that, in this disorder, two different mechanisms cause muscular weakness.

Adult↗
PubMed1983

A dominantly inherited myopathy with excessive tubular aggregates.

We studied a family in which seven individuals in three generations had slowly progressive weakness without atrophy, myalgia, cramps, or episodic weakness. Creatine kinase was normal, and EMG showed only slight "myopathic" changes. Neuromuscular transmission was undisturbed. Muscle biopsies were performed in three patients. About 60 to 90% of all fibers contained tubular aggregates. There was a marked variation in fiber size and a marked type II fiber atrophy. Biopsy of an asymptomatic family member was normal. The nature of the underlying disease was obscure.

Adult↗
PubMed1983

A modified Bodian stain for routine quantitative assessment of axonal degeneration in teased nerve fibers.

We present a modified Bodian method for staining axons in teased peripheral nerves. For test materials, guinea pig sciatic nerves, intact and cut in situ, were removed 3, 4 and 5 days after surgery. Notable characteristics of the stain are a strong contrast between the axon and surrounding myelin and easy detection of nodes of Ranvier. The method is suitable for the quantitative assessment of axonal degeneration.

Animals↗
PubMed1983

[Correlation between CT findings and clinical course in viral and bacterial meningoencephalitis].

The computed axial tomograms (CAT) of 50 patients with viral or bacterial meningoencephalitis are correlated with the clinical course, the laboratory data, and the EEG changes. The diagnostic value of CAT in the early diagnosis of herpes simplex encephalitis is of specific importance. Hypodense regions are demonstrated after the fifth day of illness in the temporal lobe. All other viral or bacterial meningoencephalitis cases have no specific changes on CAT examination compared with clinical or laboratory data. However, in localised encephalitis CAT may reveal hypodense regions. For follow-up studies of meningoencephalitis CAT is of important diagnostic value in demonstrating complications such as abscess or occlusive hydrocephalus.

Aged↗