PubMed Health⌕ Search

Biomedical subjects

I Hagne

Publications and source records attributed to I Hagne.

13 recordsLinked to original sources
PubMed1991

EEG findings in children and adolescents with mitochondrial encephalomyopathies: a study of 25 cases.

EEG was studied in 25 children and adolescents with mitochondrial encephalomyopathies, defined on the basis of clinical, biochemical and morphological criteria. Twenty cases conformed to well-known mitochondrial syndromes: Alpers syndrome [6], Leigh syndrome [2], MERRF (myoclonus epilepsy and ragged red fibers) syndrome [3], MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) syndrome [5] and Kearns-Sayre syndrome [4]. Many patients were followed for several years with repeated EEG. In all, 112 EEG records were included in the study. A common feature of all the mitochondrial encephalomyopathic syndromes was slowing of the alpha rhythm. Epileptic discharges were seen in most syndromes. In spite of the small number of cases in each group, in Alpers, MERRF and MELAS syndromes we found sequential EEG patterns which seemed to be typical of the respective syndromes. In contrast, in Kearns-Sayre syndrome, a slow background rhythm was the only consistent finding. We conclude that EEG, especially repeated recordings, may be of help in the diagnostic evaluation of mitochondrial encephalomyopathies.

Acidosis, Lactic↗
PubMed1991

Muscle structure and function before and after surgery for primary hyperparathyroidism.

In 23 unselected cases of primary hyperparathyroidism (pHPT), muscle strength, morphology and enzymatic activities were studied and electromyography (EMG) performed before and 6 months after surgical treatment. Hypercalcemia was mostly mild or moderate. Nine women undergoing surgery for benign thyroid conditions served as controls regarding muscle strength, while muscle morphologic and enzymatic data were compared with findings in healthy persons of similar age. Only three pHPT patients reported muscle weakness preoperatively, and two were subjectively improved after surgery. Muscle strength did not differ significantly before or after operation between patients and controls: After surgery both groups showed increased isokinetic muscle strength at higher angular velocities. Nor did muscle morphology differ between pHPT patients and controls. No conclusive EMG changes were found before or after surgery for pHPT but postoperatively the pHPT patients showed significant increase in glycolytic but not oxidative muscle enzymes, possibly reflecting early effect of pHPT on especially type II fibers, which previously were shown to be most extensively involved in pHPT. Otherwise no measurable negative effects of mild or moderate pHPT were found on muscle strength or function.

Adult↗
PubMed1989

EEG development in Rett syndrome. A study of 30 cases.

EEG development through advancing clinical stages was studied in 30 cases of Rett syndrome, a progressive encephalopathy seen so far only in girls. Besides slowing of background activity and epileptic patterns, which are found in many types of 'degenerative' brain diseases, we observed in advanced stages of Rett syndrome unusual EEG patterns resembling those seen in slow virus encephalitis. On the other hand, the EEG was normal in one case of advanced Rett syndrome. The typical steps of EEG development were: initially normal EEG; as the first abnormality rolandic spikes, often followed by other epileptic patterns; and, in more advanced stages, a pseudoperiodic delta pattern and occasionally generalized periodic spike activity. The results motivate a discussion concerning other possible origins of Rett syndrome in addition to the supposed genetic one. The question is raised whether Rett syndrome might be etiologically heterogeneous.

Adolescent↗
PubMed1983

Hereditary motor and sensory neuropathies in Swedish children. III. De- and remyelinating type in 10 sporadic cases.

Clinical, neurophysiological and certain other laboratory data are given for 10 children considered to represent sporadic cases of de- and remyelinating hereditary motor and sensory neuropathies (HMSN). In all 10 families the parents had been found to be non-affected and said to be non-consanguineous. Nine of the 10 families originated from the two most northerly Swedish counties, but none from the Gothenburg area. The median age at clinical onset was 3 years. Gait abnormalities and/or foot deformities were common reasons for referral. Scoliosis developed in all 10 and hand atrophies in 7 children. In the majority there was neurological deterioration through childhood. Nerve conduction velocities, both motor and sensory, were consistently and markedly reduced. The protein level in cerebrospinal fluid (medium 657 mg/l) was slightly to moderately raised in 7 of 9 examined children and greater than or equal to 1 500 mg/l in the remaining two. The fatty acid pattern of serum lecithin was consistently normal in all 10.

Adolescent↗
PubMed1983

Hereditary motor and sensory neuropathies in Swedish children. II. Neuronal-axonal types.

Clinical, neurophysiological and laboratory data are given for 32 children (30 kinships) considered to represent hereditary motor and sensory neurophaties (HMSN) of neuronal-axonal types. In 25 families (27 cases) one of the parents was found to be affected. In one both parents were normal. The mode of inheritance in the 27 patients with familial neuronal-axonal HMSN was concluded to be autosomal dominant HMSN II (Lambert type). The disability was mild to moderate and, on an average, less pronounced than in de- and remyelinating types of HMSN. Seven out of 27 were early toe-walkers of the secondary type. Cavus feet were noted in 25, hand atrophies in eight and mild scoliosis in five. Sensory complaints were recorded in 21. The nerve conduction velocities (NCVm and NCVs) of children and parents were slightly subnormal in a few. EMG proved to be the most important parameter for identifying subclinically affected parents.

Axons↗