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Biomedical subjects

Z Kamieniecka

Publications and source records attributed to Z Kamieniecka.

At least 19 recordsLinked to original sources
PubMed1991

A new mouse mutant with progressive motor neuronopathy.

A new autosomal-recessive mouse mutant with progressive motor neuronopathy (pmn) is described. Homozygotes develop paralysis of the hindlimbs during the third week of life. Soon thereafter the forelimbs also become weak, and all mice die six to seven weeks after birth. Heterozygotes are normal. Skeletal muscles show neurogenic atrophy without histological signs of reinnervation. Axonal degeneration apparently starts at the endplates and is prominent in the sciatic nerve and its branches and the phrenic nerve. Axonal sprouts are abundant. There is no evidence of demyelination, and unaffected nerve fibers are normally myelinated. Sensory axons are spared. Almost all distal motor axons have disappeared in four to five weeks after birth. Ventral nerve roots show a reduced diameter of the largest fibers but no fiber deficits. The ventral horn cells show slight chromatolysis. The corticospinal tract is normal, but in terminally ill animals the fasciculus gracilis, the rubrospinal tract and possibly also reticulospinal fibers degenerate. The brain is histologically normal. The disease manifests itself in a dying-back fashion in the distal portion of the motor neurons and may represent an animal model of hereditary motor neuron diseases in man.

Animals↗
PubMed1989

[Plasmapheresis in the treatment of peripheral HIV neuropathy].

Peripheral neuropathy following HIV infection is frequently seen among HIV-positive-, ARC- and AIDS patients who often complain of debilitating paresthesias. Indirect immunofluorescence technique showed antibodies directed either to myelin and/or axons and to endothelial cells of the blood vessels within the nerve tissue. Sural nerve biopsies from five patients showed demyelination, axonal degeneration and perivascular cellular infiltration. Two of the patients had nuclear inclusions and high anti-CMV titer. Plasma exchange was undertaken on five HIV infected patients which resulted in complete disappearance of symptoms in three, incomplete remission in one and no effect in one patient. The finding of autoantibodies against different nerve tissue components, cellular infiltration, positive effect of the plasma exchange and relapse suggests that autoimmunity may play a pathogenetic role in some of the HIV infected patients with peripheral neuropathy.

AIDS-Related Complex↗
PubMed1988

IgM monoclonal gammopathy and neuropathy in two siblings.

A sister and a brother with a progressive mixed axonal and demyelinating polyneuropathy were found to have a monoclonal IgM gammopathy of kappa and lambda type, respectively. Sural nerve and cutaneous nerve specimens obtained by biopsy showed deposits of IgM on myelin sheets. Sera from both patients contained antibodies directed to bovine peripheral nerve myelin as determined by ELISA technique and to normal human peripheral nerve myelin as demonstrated by indirect immunofluorescence histochemistry. These siblings may have a genetic predisposition to the formation of autoantibodies with peripheral nerve myelin as the target for the immune attack.

Aged↗
PubMed1987

Benign congenital muscular dystrophy with autosomal dominant heredity: problems of classification.

A family with autosomal dominant congenital muscular dystrophy affecting members of both sexes in three generations is described; a father and his two sons were studied. The onset of symptoms was in early childhood and progression, if any, was slow. The proximal limb muscles, the sternocleidomastoid and anterior tibial muscles were affected. One patient had torticollis and all had heel-cord shortening. An electrophysiological examination showed myopathy. There was no cardiomyopathy. Creatine kinase (CK) was elevated, and a histological study revealed a necrotizing myopathy with pronounced regeneration and formation of aberrant myofibrils (ringbinden) and fibrosis.

Adolescent↗
PubMed1987

Early fatal nemaline myopathy: case report and review.

A newborn girl with atonia and arthrogryposis multiplex required mechanical ventilation; she died on the 14th day. Postmortem muscle histology disclosed nemaline myopathy with a lack of myofibrils. Peripheral nerves appeared to be normal. The parents are first cousins. The findings for 13 other patients who died from nemaline myopathy within the first year of life are reviewed. It is suggested that early fatal cases, in contrast to patients with the 'benign' childhood form, are homozygotic for the disease gene, and that the myopathy results from abnormal myosin synthesis.

Biopsy↗
PubMed1984

Mitochondrial myopathy as a cause of ptosis and ophthalmoplegia in elderly females.

Of 27 patients with ptosis and ophthalmoplegia or both, and abnormal mitochondria in proximal limb muscles, 13 were elderly females, in whom symptoms began late in life. Clinical, electrophysiological, and morphological findings are presented, and possible etiological factors are discussed. The ocular mitochondriopathy in post-menopausal females seems to represent a separate, previously not described syndrome.

Age Factors↗
PubMed1984

Misonidazole neuropathy.

Neurotoxic side effects of misonidazole with peripheral neuropathy was investigated in two series of patients. The first series consisted of eight patients with carcinoma of the pharynx, larynx or lung who, during treatment with misonidazole, developed peripheral neuropathy dominated by severe sensory symptoms and signs localized mainly to the lower extremities. Misonidazole was given for three to seven weeks in a total dose of 9.6 - 12.6 g/m2 (11 g/m2 or more in four of the patients). The symptoms subsided partially within a few months after cessation of the therapy. Electrophysiological and histological findings indicated axonal neuropathy with loss of large fibres and secondary demyelination. The second series consisted of 70 patients with carcinoma of the pharynx or larynx who, in addition to radiotherapy, were given either placebo or misonidazole over four weeks in a total dose of 11 g/m2. Fourteen patients out of 36 receiving misonidazole (38%) developed peripheral polyneuropathy, mostly in the feet, while this occurred in only two of the 34 patient placebo group.

Aged↗
PubMed1982

Misonidazole Neuropathy: a clinical, electrophysiological, and histological study.

We studied eight patients with carcinoma of the pharynx and larynx (five cases) or lungs (three cases) who, during treatment with the radiosensitizing drug misonidazole, developed peripheral neuropathy dominated by severe sensory symptoms and signs mainly localized to the lower extremities. The symptoms partially subsided within months after cessation of therapy. Electrophysiological and histological findings indicated an axonal neuropathy with loss of large fibers and secondary demyelination. The neurotoxic property of misonidazole limits its therapeutic use.

Aged↗
PubMed1982

The diagnostic yield of quantified electromyography and quantified muscle biopsy in neuromuscular disorders.

Electromyography (EMG), histology, and histochemistry were related in 264 patients with neuromuscular disorders classified according to history and clinical and other laboratory findings. Electromyography and histological and histochemical abnormalities were divided in specific and nonspecific criteria. Specific histochemical criteria alone identified 28% of neurogenic lesions. Criteria of myopathy, obtained from the pattern of electrical activity during 30% of maximal effort, helped to delineate a myopathy when the only abnormality was an increased incidence of polyphasic potentials together with a pattern of full recruitment during maximal effort. Histology, histochemistry, or both, and EMG were concordant with clinical findings in 77% of 188 patients with myopathy and in 91% of 64 patients with neurogenic lesions. The electromyogram was concordant with the clinical classification in 87% of patients with myopathy and in 91% of patients with neurogenic impairment. The biopsy was in agreement with or contributed to the classification in 79% of patients with myopathy and in 92% of patients with neuropathy.

Adolescent↗
PubMed1979

Familial amyloidosis with cranial neuropathy and corneal lattice dystrophy.

Five siblings of a Danish family with slowly progressive involvement of the trigeminal, facial, glossopharyngeal, accessory, and hypoglossal nerves beginning at the age of 55-65 years were examined. All had asymptomatic corneal lattice dystrophy. Clinical and electrophysiological investigations also showed evidence of slight neurogenic involvement of the limbs. Conduction velocity along sensory nerves was normal but amplitude of sensory potentials was severely reduced suggesting an axonal affection which was confirmed by sural nerve biopsy. The neuropathy was secondary to amyloidosis revealed by skin and sural nerve biopsies.

Aged↗
PubMed1977

Myopathies with abnormal mitochondria. A clinical, histological, and electrophysiological study.

Among 135 consecutive patients with myopathy, 17 showed abnormalities in the mitochondria of muscle. In eight of these patients the abnormalities were demonstrated by electron microscopy. In all patients Gömöri trichome stain showed red masses at the sites of the mitochondria and diformazan deposits when stained for mitochondrial enzymes. The patients could be divided into three groups: i) Nine patients had weakness of the extra-ocular muscles, either alone or associated with weakness of skeletal muscles and non-muscular signs. One patient with the oculo-cranio-somatic syndrome had two affected sibs. ii) Four patients had a facioscapulohumeral distribution of weakness. Three (two sisters and a maternal aunt) were in the same family, and the fourth patient had an affected sister. iii) Four patients were clinically heterogeneous. When mitochondrial alterations were the only pathological change in many fibers in a muscle, they were most often associated with an ocular or oculo-cranio-somatic syndrome. Electromyography showed changes indicating or suggestive of myopathy in all but one patient. There was a discrepancy between severe changes in the EMG and nearly normal fibers by hematoxylin and eosin and van Gieson stains. Serum creatine kinase was increased in 10 of 17 patients, in most 2-4 times the upper limit of normal.

Action Potentials↗
PubMed1977

Post-tetanic mechanical tension and evoked action potentials in McArdle's disease.

The tension produced by the cramp evoked in the adductor pollicis muscle by repetitive stimuli to the nerve (20/s for 50 s) and by full voluntary effort in the brachial biceps was measured in a patient with McArdle's disease. The contracture was 17% of the peaktetanic tension, and was not associated with action potentials. Twitches superimposed on the contracture were at most diminished to half, as were their action potentials. Both slow and fast muscle fibres participated in the contracture. The contraction time of the twitches elicited after the tetanus was prolonged more in the patient than in a normal subject of the same age. There was evidence of delayed firing, first observed 90 seconds after the peak of the contracture. The patient had electromyographic and histological signs of myopathy.

Action Potentials↗
PubMed1975

Histochemical fiber typing and staining intensity in cat and rat muscles.

In the gastrocnemius muscle of cat and rat, staining for oxidative enzymes differentiated three fiber types (A,B,C) and staining for adenosine triphosphate at pH 9.4 differentiated two fiber types (I, II) with a reliability of 90% and 98%, respectively. In cat 96% and in rat 90% of the fibers were typed identically after staining for nicotinamide adenine dinucleotidelinked lactic dehydrogenase (LDH) and succinic dehydrogenase (SDH). When differentiated by staining for LDH, A and B fibers were of type I. IN RAT, 80-90% OF ALL FIBERS WERE OF TYPE 22, COMPPRISING A, B and C fibers. Type I fibers stained for LDH intensely as did C fibers of type II, but stained intermediately for SDH. The degree of staining was measured by photometry. When fibers were stained for LDH, histograms of density showed three peaks corresponding to A, B and C fibers in cat, but only two peaks corresponding to A and C fibers in rat, In cat and rat, the densities of A, B and C fibers belonged to different populations. In soleus muscle of cat and rat stained for LDH, menadione-linked alpha-glycerophosphate dehydrogenase and adenosine triphosphatase at pH 9.4, the degree of staining differed from thatin any type of fiber in gastrocnemius muscle

Adenosine Triphosphatases↗