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Biomedical subjects

X Ferrer

Publications and source records attributed to X Ferrer.

At least 37 records · Page 2Linked to original sources
PubMed1989

Jaw closing spasm--a form of focal dystonia? An electrophysiological study.

The case of a 36 year old man suffering from unilateral right jaw closing spasms over two years is reported. Permanent spasm with trismus severely impeding mouth-opening was combined with paroxysms triggered by various sensory stimuli. The diagnosis of temporo-mandibular joint syndrome was considered but treatment failed to improve the symptoms. Neurological investigation two years after onset of the spasms showed by electrophysiological studies excessive co-contraction of the antagonistic jaw-closers, mainly the right masseter during attempts at jaw opening and absence of the silent period in the right masseter and anterior temporalis following jaw tap and trigeminal exteroceptive stimulation. Jaw dystonia was therefore considered and Botulinum A toxin was injected into the right masseter and temporalis which dramatically improved the patient's condition.

Adult↗
PubMed1989

Chronic relapsing idiopathic polyneuropathy with primary axonal lesions.

Idiopathic polyradiculoneuropathy with primary axonal lesions is rarely encountered. Two cases are reported with a chronic relapsing course and a fatal outcome. Neuropathological examination of biopsied peripheral nerve in the two patients and in a necropsy case showed loss of myelinated fibres, but neither active demyelination nor inflammatory cells were observed. Acute and chronic relapsing axonal polyradiculoneuropathies appear to be two clinical forms of a peculiar entity different from GBS.

Axons↗
PubMed1989

An inhibitor of cellular cytotoxicity is present in multiple sclerosis.

A blocking factor is present in the serum of patients with multiple sclerosis (MS). It is able to inhibit a cellular cytotoxicity reaction in vitro. This substance has characteristics similar to those of the 'P4' protein described in pregnant women and patients bearing tumors. Its possible contribution to MS pathogenesis is discussed.

Cytotoxicity, Immunologic↗
PubMed1989

Familial idiopathic striopallidodentate calcifications.

We report a father and son with striopallidodentate calcifications. Metabolic studies excluded calcium/phosphorus metabolism disturbances and no specific etiology was found. The structure of the calcified areas differed, on magnetic resonance imaging, depending on location and, probably, age. There are nine families with similar clinical and radiologic backgrounds and no evident etiology in the literature. Transmission is most often autosomal dominant, and in contrast with physiologic senescent basal ganglia calcification, the prognosis appears to be poor.

Basal Ganglia Diseases↗
PubMed1988

Booster effect of tuberculin testing in healthy 6-year-old school children vaccinated with Bacillus Calmette-Guérin at birth in Santiago, Chile.

In order to determine whether tuberculin testing caused a booster effect in children vaccinated with Bacillus Calmette-Guérin (BCG) at birth, we studied forty 6-year-olds by repeat tuberculin testing 2 weeks later on the contralateral forearm. All children were healthy and had no known exposure to tuberculosis. None of the children had a history of mycobacteriosis other than tuberculosis. The mean induration was 2.3 +/- 1.8 mm for the first tuberculin reaction and 7.6 +/- 3.3 mm for the second tuberculin reaction (P less than 0.005). Four children had positive reactions (greater than or equal to 10 mm) to the first purified protein derivative test; 18 children were positive upon retesting. Eleven of these latter children had increases of at least 6 mm from reactions less than 10 mm to greater than or equal to 10 mm. The size of the BCG scar was significantly correlated to the size of both the first and second purified protein derivative reactions (P less than 0.01), suggesting that the increased reactivity upon retesting was a consequence of sensitization induced by BCG vaccination 6 years earlier. All children remained healthy after this study was completed. Retesting of tuberculin reactivity within 2 weeks in BCG-vaccinated children with reactions less than 10 mm will produce reactions greater than 10 mm in some healthy children who may not require antituberculosis treatment.

Antibodies, Bacterial↗
PubMed1987

[Divry-Van Bogaert cortico-meningeal angiomatosis and Sneddon's syndrome. Nosological study. Apropos of 4 cases].

Four patients with idiopathic livedo reticularis and multiple cerebral ischemic events leading to a severe dementia are reported. Imaging (CT, MRI, angiograms) and pathological features (in one case) are presented. Similar clinical features have been described previously in Divry-van Bogaert's disease and Sneddon's syndrome. Data from our cases and a review of the literature did not find distinctive characteristic between these entities. Thus, for practical purposes, considering them as one group appears at present warranted.

Adult↗
PubMed1987

[Neurogenic muscular hypertrophy. Association with abnormal electrophysiological activities].

Muscle hypertrophy in neurogenic disorders is an uncommon phenomenon which has been reported in various conditions: hereditary or acquired anterior horn cell diseases, essentially S1 radicular compressions, and polyneuritis generally of a demyelinating type. We report two cases of denervating disease with muscle hypertrophy. The first was an S1 radiculopathy, and the second a compression with ischemia of the spinal cord by herniation of the D11-D12 disc, and showing partial improvement after surgery. In both cases, electrophysiological examination of the hypertrophic muscles revealed abnormal activities identified as complex repetitive discharges. In the second case they were associated with a syndrome of continuous motor unit activity. Such activities are rare in peripheral nerve involvement. However they have quite often been recorded in cases of neurogenic muscle hypertrophy and may therefore be partly responsible for the development of the hypertrophy.

Adult↗
PubMed1986

[Epilepsy with continuous discharges during slow-wave sleep. Treatment with clobazam].

The authors describe the case of a 12 years old boy suffering from an epilepsy with complex partial seizures evolving to a syndrome of epilepsy with continuous spikes-waves during sleep. A dramatic improvement follows clobazam introduction in the treatment. The originality of this case report and the possible mechanism of action of clobazam are discussed.

Anti-Anxiety Agents↗
PubMed1985

[Tomaculous neuropathy. Electrophysiologic study].

In two cases with recurrent palsies, the results of electrophysiological studies led to nerve biopsy showing typical tomacula. The first case was an inherited neuropathy with liability to pressure palsies. The second case was an apparently sporadic painless recurrent brachial neuropathy. Electrophysiological alterations were diffuse and sensory fibres of the median nerve between index and wrist were the most involved. Conduction blocks were observed without palsy in narrow anatomical passageways where nerve compressions are frequent (ulnar nerve at the elbow, peroneal nerve at the fibula). A compression by a neighbouring anatomical structure could make the prognosis worse and justify nerve decompression. The nerves with slowest conduction have the most important risk of palsy and the patients should be given advice to avoid their compression.

Adolescent↗
PubMed1985

[Post-radiotherapy anterior horn cell syndrome].

Three patients developed a progressive flaccid paraparesis without sensory or sphincter disturbances, following radiotherapy for lymphoma in two cases and carcinoma of testis in one case. The course was progressive with stabilization between two and four years. Electrophysiological study suggested anterior horn cell damage the mechanism of which remains unclear.

Adolescent↗
PubMed1984

IgM demyelinative neuropathy with amyloidosis and biclonal gammopathy.

A 59-year-old man developed a sensorimotor neuropathy of the upper and lower limbs, associated with a biclonal gammopathy, within the space of a few months. Each of two paraproteins was coupled with a distinct IgM kappa IgG lambda light chain. Examination of a nerve biopsy specimen by electron microscopy revealed a demyelinative process with a widening of the interlaminar space in the myelin sheath, as well as deposits of amyloid substance between nerve fibers. Direct immunofluorescence revealed the presence of IgM and of the kappa light chain in certain Schwann cells, while the lambda IgG was fixed to the amyloid deposits. Immunoperoxidase histochemistry showed a positive reaction in normal human nerve tissue to the immune serum IgM and kappa light chain. The findings suggest that the widening of the interlaminar space of the diseased myelin corresponds to an active fixation of immunoglobulin on the sheath of the Schwann cell. The presence of two light chains in this patient's gammopathy caused a dual pathology: the kappa chain, a demyelinative neuropathy, and the lambda chain, a primary amyloidosis, with deposits in the peripheral nerve and in the kidney.

Amyloidosis↗
PubMed1984

Cavernous sinus syndrome due to lymphoma.

In both the cases described, painful ophthalmoplegia was the first indication of infiltration of the cavernous sinus by a lymphosarcoma. The onset of symptoms and the course of the disease were different in the two cases. CT scan which has been the crucial investigation for detecting lymphomas in the cavernous sinus, was normal in the early stages. Symptomatic remission could be obtained with treatment, although the prognosis remained poor.

Aged↗
PubMed1984

Chronic demyelinating neuropathy with IgM-producing lymphocytes in peripheral nerve and delayed appearance of "benign" monoclonal gammopathy.

A chronic demyelinating neuropathy with "benign" IgM gammopathy was followed for 6 years in a 63-year-old man. The clinical, biologic, and EMG aspects were similar to those already reported, but a lymphoplasmocytic infiltrate in the nerve connective tissue of this patient has only rarely been observed in benign IgM gammopathy. The paraprotein was not evident in the serum until 5 years after symptoms of the neuropathy started.

Demyelinating Diseases↗
PubMed1983

[Familial amyloid neuropathies in 3 families of French origin].

Clinical, electromyographic and neuropathological studies were carried out at different stages of evolution of a familial amyloid neuropathy in 6 members of 3 families of French origin. The clinical onset was marked by sensory symptoms and signs in limb extremities, primary manifestations being alterations in pain and thermal sensitivity. This was followed by motor and amyotrophic disorders predominant in the lower limbs. Autonomic nervous system disorders were frequent later. Early electromyographic signs were diminished amplitude and increased duration of sensory potentials. Progression of the disease is shown by the onset of signs resulting from severe axonomyelinic lesions. Neuromuscular biopsy demonstrated the presence of amyloid deposits in the endoneurium in 5 of the 6 cases. Transmission appeared to be dominant autosomal. These cases pertain to group I of the amyloid neuropathies. The axonal lesions marking the onset of the affection could be secondary to biochemical alterations in prealbumin, responsible for amyloid formation. This hypothesis affords a basis for plasmapheresis which has been used in 3 patients.

Adult↗