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Biomedical subjects

B Mamoli

Publications and source records attributed to B Mamoli.

At least 19 recordsLinked to original sources

EEG, pattern-evoked potentials and nerve conduction velocity in a family with adrenoleucodystrophy.

In a family with 8 children one case of adrenoleucodystrophy (ALD), verified by autopsy, 2 cases with clinical signs of ALD and four other clinically healthy subjects with pathological ACTH tests were found. As an initial sign in the EEG, temporo-occipital slowing occurred. Further EEG studies showed spread of the primary local abnormalities, indicating diffuse brain dysfunction during progression of the disease. Terminal EEG flattening may be characteristic for late stages of the disease. No correlation could be established between lateralization, paroxysms and actual stage of the disease. Abnormal EEGs occurred not only in clinically and subclinically affected male patients, but also in a girl, who might be a carrier of ALD. Visual evoked potentials showed an increased latency of the P2 wave in the clinically, and in one subclinically, affected subjects. Motor nerve conduction velocity was reduced in only 2 patients. Antidromic sensory nerve conduction velocity of the median nerve was normal in all patients.

Addison Disease

Electroneurographic investigations of misonidazole polyneuropathy.

13 patients with malignant tumors were treated by the radiosensitizer misonidazole (Ro 07-0582), total dosage 20-29 g. The electrophysiological investigations showed (1) an early increase of distal latency, the motor nerve conduction velocity (NCV) of the peroneal nerve and the NCV of the sural nerve remaining normal or only signlty reduced, and in a few cases a marked reduction of the compound action potential or of the nerve action potential (NAP), indicating a primary axonal neuropathy; (2) greater changes in the parameters of sensory nerves (n. suralis) than of motor nerves; (3) the distal latency is a good indicator of subclinical neuropathies; (4) the electrophysiological parameters showed a normalization 6 months after the end of therapy. The mechanisms possibly responsible for the misonidazole neuropathy are discussed.

Action Potentials

Further clinical experiences of a phase I study with the hypoxic cell radiosensitizer misonidazole.

Since April 1976 we have performed clinical investigations with multiple doses of the hypoxic cell radiosensitizer misonidazole in 21 patients. A significant side effect of the drug was the development of peripheral sensory neuropathies in 13 patients (8 mild, 5 severe) and of a transient acute organic psychosyndrome in 2 of the 5 patients with a severe polyneuropathy. The severity of the polyneuropathies is related to the total dose of misonidazole and the overall time of drug administration. Treatment schedules designed to obtain the desired sensitizing effect without neurological side effects are under investigation.

Adolescent

Electrophysiological studies on the "stiff-man" syndrome.

In addition to anamnestic and clinical data electrophysiological and pharmacokinetic investigations may be necessary for the diagnosis of stiff man syndrome. Continuous activity of motor units with superimposed bursts during muscular spasms was found by electromyography in the two patients reported. Rigidity and continuous activity disappears during sleep, after i.v. application of Tubocurarine and Diazepam, during Thiopenal anesthesia and after neural block with Procaine. Dipropylacetate and Baclofen improved the condition but did not lead to complete relaxation. Biperidenlactat and Magnesiumlaevulinat have only a temporary effect on rigidity. Neostigmine, Phenytoine, Glycine, Dopa and 5-Hydroxy-Tryptophan had no effect. Passive shortening or stretching of the m. biceps brachii as well as touching the skin increased motor activity which spread to other segments and to the contralateral side. The H/M ratio was increased but the silent period was normal. A combination of Diazepam and Dipropylacetate or Clonazepam was therapeutically effective in the cases reported. A central genesis, of the pathogenetic mechanisms discussed, is the most probable in our cases.

Brain

Recurrent Bell's palsy. Etiology, frequency, prognosis.

The etiology, frequency and prognosis of recurrent Bell's palsy were studied in patients with peripheral paresis of n.facialis of various etiology. Fourteen (11.9%) of 117 patients with Bell's palsy had a recurrent paresis of n.facialis. Nine of these were of homolateral and five contralateral type. Seven recurrent facial paralyses in idiopathic Bell's palsy (i.e. 10.4% of the patients with idiopathic facial palsy), 3 facial palsies of viral origin (i.e. 8.8% of the patients with viral facial palsy), one in association with diabetes mellitus, one during pregnancy, one combined with positive rheumatic serological tests, and one in a case of Melkersson-Rosenthal syndrome were found. The frequency and heterogenity of etiology of recurrent facial palsies suggest a predisposing factor or immune mechanisms. In eight patients there was within 6 to 8 weeks a good, in two patients a moderate and in four patients a poor recovery of function. The greater reduction of the compound action potential of the m.orbicularis oris in recurrent homolateral facial palsy in relation to patients with single manifestation point out the greater denervation and therefore the poorer prognosis of recurrent palsies. A prophylactic decompression to prevent a third attack of recurrent facial paresis is considered.

Action Potentials

Nervous lesions in a case of local gigantism.

A case of local gigantism of the thumb was studied by light and electron microscopy. In the lesion, proliferation of fibroadipose tissue and typical alterations of the nerves were found. The alterations in the nerve were isolated hypertrophy of the perineural cells. The relation of the changes to other peripheral nerve tumours especially in neurofibromatosis, is discussed.

Adult

Electrophysiological investigations in a case of cephalic tetanus.

In a case of cephalic tetanus the amplitudes and the latencies of the compound action potentials recorded from the facial muscles after stimulation of the facial nerve at the mastoid were not significantly different on the two sides. With repetitive stimulation at 30/sec a pathological facilitation was observed on both sides. The blink reflex bilaterally had normal latencies but the amplitudes of the reflex potentials were always significantly lower on the paretic side. It is concluded that facial palsy in cephalic tetanus is mainly due to a functional block of conduction in the course of the peripheral nerve whereas the disturbance of neuromuscular transmission probably has little importance in these cases.

Action Potentials

[Comparative bioptic and electrophysiological investigations in polyneuropathies (author's transl)].

Comparative bioptic and electrophysiological investigations were performed in 30 cases of polyneuropathy of varying aetiology. Axonal and demyelinating neuropathies were distinguishable concordantly on the basis of both investigations. Maximum motor nerve conduction velocity (NCV) and compound potential (CP) were regularly examined in several nerves of each patient. Low (NCV) values (critical limit 25 m/sec) and long-standing high CP values were characteristic of demyelinating processes, whereas axonal neuropathies showed a slight to moderate decrease in NCV with early decrease in CP, often rapidly followed by loss of nerve excitability. Nonclassifiable stages and diseases are discussed. In biopsies, ultrastructural examination in particular, not only allowed the differentiation of primary axonal and primary demyelinating lesions and the respective neuropathic syndromes, but permitted moreover, further classification of these heterogenous groups of neuropathies. According to the sequence of lesions of the nerve fibre classes on the one hand, and to the nature of the primary lesion of the nerve fibres on the other hand, the following types of neuropathies were distinguished: in axonal neuropathies of the myelinated fibres, dystrophic and disintegrative forms were differentiated; axonal neuropathies of unmyelinated fibres correlated with sensory neuropathies. Demyelinating neuropathies were represented by hypertrophic, leucodystrophic and paraproteinaemic forms. The correlation of these types of neuropathies to the different clinical and aetiological forms of polyneuropathies is diagrammatically shown. The connection between the electrophysiological data and the basic nerve fibre alterations of the different neuropathies is discussed.

Demyelinating Diseases

Initial clinical experience with the radiosensitizing nitroimidazole Ro 07-0582.

The 2-nitroimidazole derivate Ro 07-0582 is known from experimental studies to be a very efficient radiosensitizer of hypoxic cells. Experiences with its clinical use are very limited so far. This study reports the side effects observed in the use of this drug in 12 patients. Nine of these patients were suffering from brain tumors, 3 from extracerebral tumors. In 8 of our 12 patients a sensoric polyneuropathy occurred which was strongly related to the total dose of the drug administered. Polyneuropathy was observed on the average after a total dose of 26 grams, but was already noticed at doses as low as 22 grams. It showed a good tendency for remission after termination of the drug treatment and was reversible in all patients within 4 weeks. In one patient with a brain tumor a severe organic psychosyndrome occurred which is possibly related to the drug. This side effect was also reversible. In this small group of patients the critical dose limit seems to be 29 grams. This dose can be divided into 6 fractions of 60-80 mg/kg. Up to the present knowledge this single dose is likely to give an enhancement ratio of 1,5, which would be extremly valuable for the treatment of hypoxic tumor cells.

Adolescent

[Motor nerve conduction velocity in uraemic polyneuropathy: correlation with metabolic factors (author's transl)].

The following parameters have been examined in twenty-one patients suffering from chronic renal failure (creatinine level between 4.5 and 18.8 mg/100 ml serum): maximum motor nerve conduction of the peroneal nerve, amplitude of the compound muscle action potential of the extensor digitorum brevis muscle, serum creatiine, total protein, serum globulins, serum albumins, alkali reserve, time of increase of serum creatinine above 4 mg/100 ml up to time of determination of the maximum motor nerve conduction, daily urinary excretion, mean blood pressure, (p less than 0.01) was found between maximum motor nerve conduction, as well as amplitude of the compound muscle action potential, and the serum albumin level only. Decreased levels of serum albumin, is correlated with diminished nerve conduction and a lower amplitude. The relationship between the electrophysiological data and serum albumin levels maybe explained on the basis of progression of a pre-existing polyneuropathy due to additional dietary malnutrition. A different interpretation is the assumption of an inactivation of neurotoxin on binding by albumins. A decrease in the albumin level would, therefore, result in an increased amount of unbound toxic agent. The values of the maximum motor nerve conduction were between 16 m/sec and 51 m/sec (mean value 42.2 m/sec), pointing to a polyneuropathy of primary axonal type rather that to primary demyelinization. The amplitudes of the compound muscle action potentials were not greatly reduced and thus the uraemic polyneuropathy seems to be of mixed type. In uraemic polyneuropathy different aetiological factors have to assumed. According to the prevalent factor a polyneuropathy of predominantly axonal or predominantly demyelinizing type may result.

Action Potentials

[The effect of dipropyl acetic acid (Convules) in epileptic adults with a high frequency of seizures].

The anti-convulsive action of dipropyl acetic acid (Convulex) was tested in 21 patients with grandmal seizures (GM) and/or temporal lobe attacks (TL), or with Jacksonian epilepsy. Patients were chosen according to a negative selection system. Results showed that in GM and TL seizures, Convulex constitutes an important addition to the battery of anticonvulsives available today. Action was more potent in GM than in TL attacks. Toxic effects are slight. Treatment had to be discontinued in two patients -- once because of cerebellar symptoms and once because of crowded incidence of seizures. In two cases the patients gained weight. Occasional gastrointestinal complaints disappeared when antacids were administered. No changes were observed with reference to blood count, or to hepatic or renal function. Fatigue set in only when treatment was combined with barbiturates and disappeared again when pre-medication was reduced. Five patients showed an improved state of mind (activation and better communication with others); this psychological action may be considered an additional advantage. Since the action of barbiturates and hydantoins is potentiated by Convulex, pre-medication may be reduced --provided Convulex therapy alone does not seem adequate. Hence, trial treatment with Convulex may also be recommended in those patients who are successfully controlled with barbiturates and hydantoins,but whose daily work schedule is impaired due to fatigue.

Adult

[The severest form of CO poisoning (author's transl)].

The case histories of four patients with severe cerebral manifestations as a result of CO poisoning are reported. All patients displayed an acute mesencephalic syndrome in the initial stage. One case developed a fatal bulbar syndrome following a transitional phase. Autopsy showed cerebral oedema, in addition to necrotic areas in the basal ganglia and cerebellar haemorrhage. The other 3 patients developed an apallic syndrome with all the typical features and symptoms. Two of these patients died without showing any improvement, displaying not only necroses in the basal ganglia, but also diffuse destruction of the white matter and, in one case, even of the cortex. The third patient recovered. His recovery took a similar course to that of a case of traumatic apallic syndrome and he was subsequently able to take up normal activities. It is concluded from these four cases that secondary brain oedema precedes the development of the most serious form of CO poisoning and is of grave prognostic significance.

Adolescent

[Finger Microtremor--an experimental study (author's transl)].

This paper deals with the recording of finger microtremor using a self-constructed apparatus (amplitudes 0.5 to 0.05 mm). 32 healthy subjects aged 7 to 60 years were examined by means of a test set permitting the exact recording of the amplitudes and frequencies of the right middle finger movement. Special attention was given to the vertical plane of the tremor in order to permit more exact demonstration. Frequencies of 5 to 12 cps. were recorded, the mean frequency being 7.55+/-1.12 cps. The minimum amplitude was 0.02 and the maximum recorded amplitude amounted to 1.4 mm, with a mean amplitude of 0.12+/- 0.07 mm. No significant differences in amplitude or frequency were noted with respect to age or sex. This method is useful in the accurate quantitative assessment of every kind of tremor.

Action Potentials

[Intracranial and spinal hemorrhage in haemophilia (author's transl)].

Among 234 hemophiliacs, 14 patients (5.9%) with intracranial and 1 patient (0.4%) with intramedullary hemorrhage were observed and examined. The average age at the time of hemorrhage was 17 years in patients with severe hemophilia and 43 years in patients with moderate hemophilia. The patients with mild hemophilia and intracranial bleeding were 5 and 58 years old, respectively. Eight intracerebral, 3 subdural and 4 subarachnoid hemorrhages were encountered. In 7 cases bleeding was posttraumatic and in 5 it occurred spontaneously. In 2 patients hemorrhage followed a sudden increase of intracranial pressure. In 1 patient reliable data could not be obtained. Four of the 8 patients with intracerebral bleeding died; all survivors displayed some residual neurological abnormalities. All patients suffering from subarachnoid bleeding recovered completely. No serious complications were encountered after 3 lumbar punctures, 5 cerebral angiographies and after 1 myelography. Only in 1 case with an acquired inhibitor did a local hematoma develop at the site of puncture for the angiography. The same diagnostic procedures should be performed after substitution therapy with anti-hemophiliac plasma in a hemophiliac without inhibitor as in non-hemophiliacs if intracranial or intramedullar bleeding is suspected. In hemophiliacs with an anticoagulant, angiography or lumbar puncture should be performed only in exceptional cases.

Adolescent