PubMed Health⌕ Search

Biomedical subjects

T Kuntzer

Publications and source records attributed to T Kuntzer.

At least 73 records · Page 4Linked to original sources

Quantitative EMG analysis and longitudinal nerve conduction studies in a Refsum's disease patient.

A patient with aosmia and night blindness had repeated clinical and electrophysiological examinations during a 21-year period. Within the first 11 years, he experienced two subacute episodes of numbness with weakness and ataxia. Over the 10 ensuing years, no additional functional disability appeared. We looked retrospectively for a correlation between repeated manual muscle scoring and different nerve conduction parameters. Further studies were done in order to estimate reinnervation in proximal and distal muscles and to assess autonomic functions. A direct relationship was found between the magnitude of muscle weakness and the amplitude of compound muscle action potentials, whereas nerve conduction velocities were inhomogeneously reduced but almost unchanged over time. Parasympathetic tests were normal but sympathetic skin responses were absent. As estimated by macro-EMG and turns-amplitude analysis the compensatory reinnervation was very high. We conclude that, in Refsum's disease, recurrent segmental demyelination of a significant portion of the motor units can occur in parallel with exacerbations of weakness, and that a considerable degree of progressive motor and sudoromotor axonal loss can be found although there is an apparent long-term clinical stabilization of the neuropathy.

Action Potentials↗

Early symptoms and outcome of Listeria monocytogenes rhombencephalitis: 14 adult cases.

Listeria monocytogenes rhombencephalitis has never been studied in a significant group of patients. We describe 14 adult cases who were seen over a 10-year period. A biphasic illness was characteristic: (1) prodromes (5-15 days) with malaise, fatigue, headache, nausea or vomiting, and fever; (2) cranial nerve palsy with facial palsy, diplopia, dysphagia, dysarthria, usually multiple. Meningism and hemi- or tetraparesis were present in 11 patients and cerebellar dysfunction in 9 patients. In 4 cases, CT showed widening of the brain stem with disappearance of the surrounding cisterns. The cerebrospinal fluid was abnormal in all patients in whom this investigation was done (pleocytosis, elevation in protein content). The patients received antibiotic therapy for 2-6 weeks. In the 9 patients who recovered, the neurological dysfunction improved within 2 days to 1 week of the initiation of therapy. There were 5 deaths. At autopsy in 2 cases, there was severe purulent meningitis and rhombencephalitis with predominantly polymorphonuclear cellular infiltration in 1 case, while numerous microabscesses in the midbrain, pons and medulla were observed in the other. We conclude that L. monocytogenes infection should be considered in patients who develop fever and focal neurological signs particularly localized to the brain stem.

Adult↗

Motor conduction block and high titres of anti-GM1 ganglioside antibodies: pathological evidence of a motor neuropathy in a patient with lower motor neuron syndrome.

A patient with a progressive lower motor neuron syndrome and neurophysiological evidence of motor axon loss, multifocal proximal motor nerve conduction block, and high titres of anti-ganglioside GM1 antibodies. Neuropathological findings included a predominantly proximal motor radiculoneuropathy with multifocal IgG and IgM deposits on nerve fibres associated with a loss of spinal motor neurons. These findings support an autoimmune origin of this lower motor neuron syndrome with retrograde degeneration of spinal motor neurons and severe neurogenic muscular atrophy.

Aged↗

Birth trauma: its effect on the urine continence mechanisms.

Birth trauma is the most important etiological factor in the genesis of stress urinary incontinence in women (SUI). There is a high incidence of SUI during pregnancy, and after delivery SUI persists in a small percentage of women (2-3%). Almost all studies on perineal muscle function reveal decreasing intravaginal pressures in the days after delivery which rarely return to predelivery levels. A few urodynamics studies have demonstrated reduced urethral closure pressures and functional length after vaginal delivery, but the importance of such findings in the genesis of SUI is controversial. Better consensus has been found when pelvic floor neurophysiology was carried out: there is electromyographic evidence of a denervation-reinnervation pattern in the striated urethral sphincter muscle and occasionally prolonged pudendal conduction times when the pudendal nerves are directly stimulated. Histomorphologic studies of the pelvic floor have demonstrated that, in some women, abnormal collagen types are responsible for vaginal prolapse and accompanying SUI. Finally, the great importance of perineal reeducation by electromyostimulation and biofeedback in patients with traumatic pelvic floor pathology may be emphasized, but the importance of its role in the prevention of late SUI development remains to be established by more prospective studies.

Adult↗

[Chin neuropathy of neoplastic origin].

Three patients with mental neuropathy ("numb chin syndrome") secondary to systemic cancer are described. Two patients had malignant lymphoma and one a probable hypernephroma. In 2 patients, facial numbness was a presenting symptom of the malignancy. Investigations disclosed metastatic lesions of the mandible in one patient and cerebrospinal fluid was normal in 2 patients. Two patients died at 1 and 8 months respectively after onset of the neuropathy. Although benign in appearance, numb chin syndrome is most often, and sometimes initially, associated with visceral neoplasia or malignant hemopathy. It is a factor of poor prognosis. The pathogenesis is related either to metastasis of mandible, or neoplastic infiltration of the nerve itself.

Aged↗

Herald facial numbness.

Three unusual patients who developed subacute facial numbness as the heralding symptom of an expanding tumor that involved the trigeminal nerve fibers are reported. The first patient had clinical and electrophysiological evidence of an isolated mental neuropathy as a result of metastatic lesions with bone destruction from a renal cell carcinoma. The second patient had a sensorimotor trigeminal neuropathy caused by a direct compression of the semilunar ganglion by a cavernous hemangioma of Meckel's cave. The last patient experienced facial numbness as the unusual presenting manifestation of a primary brainstem lymphoma. Patients 1 and 3 died a few weeks after the admission, whereas patient 2 poorly recovered. Despite the availability of new techniques for early diagnosis, this report demonstrates how difficult it can initially be to differentiate a 'benign' trigeminal neuropathy from serious conditions and underscores the poor prognosis of fifth nerve fibers involvement by an expanding mass. Early referral with clinical and electrophysiological evaluation appears to be of crucial importance.

Adult↗

[Monomelic ischemic neuropathy caused by subclavian artery obstruction].

A case study of acute ischemic monomelic neuropathy due to occlusion of the right subclavian artery is reported. The occlusion was embolic. Clinical examination showed sensory loss and severe distal weakness of the limb without obvious muscle necrosis. EMG showed motor and sensory nerve axon loss, chiefly distally. Despite surgical intervention, the neurological damage was irreversible.

Aged↗

Borrelia rhombencephalomyelopathy.

Three patients, in whom the diagnosis of Borrelia burgdorferi infection was unknown for several years, developed a biphasic involvement of the central nervous system: an acute brain-stem dysfunction was followed up, in two patients, by a progressive, disabling myelitis and, in one patient, by further relapsing-remitting episodes of severe multifocal rhombencephalitis. The most consistent cerebrospinal fluid abnormalities in the analysis of sequential specimens were elevated total IgM levels that normalized after penicillin therapy. The neuropathologic findings in one patient showed microgliosis and meningovascular involvement of the central nervous system, resulting in two ischemic infarcts in the myelencephalon. Few spirochetes were localized in the leptomeninges and around subependymal vessels of the fourth ventricle. The vascular element consisted of an obliterative inflammatory vasculopathy in the medullary parenchyma. This study (1) provides pathologic evidence that a vascular disease induced by B burgdorferi is a pathogenetic mechanism for cerebrovascular diseases, and (2) emphasizes the similarities between neuroborreliosis and neurosyphilis.

Adult↗

Predictive value of anti-GM1 ganglioside antibodies in neuromuscular diseases: a study of 180 sera.

The incidence of anti-GM1 antibodies in the serum of 104 patients with neurological diseases, 35 patients with non-neurological diseases (NND) and 41 normal controls was determined by enzyme-linked immunosorbent assay (ELISA). Anti-GM1 antibodies were found in 90% of patients presenting with a motor neuropathy (all except one had multifocal conduction blocks). A large proportion (60%) of these patients displayed high antibody titer ranging from 101 to 788. A low incidence of anti-GM1 antibodies was found in the other groups of patients, i.e. 21% of amyotrophic lateral sclerosis (ALS), 26% of other neurological diseases (OND) and 23% of NND. High antibody titers ranging from 106 to 260 were found in two (5%) ALS patients, one (2%) OND patient (myasthenia gravis), and one (3%) NND patient (Waldenström's disease). This study shows that high titers of anti-GM1 antibodies are found in a large proportion of patients with motor neuropathy with multifocal conduction blocks. This argues for a possible autoimmune origin of this neuropathy. We suggest that anti-GM1 antibody determination should be included systematically in the evaluation of all patients with motor neuron diseases and predominantly motor neuropathies.

Adult↗

[Lambert-Eaton myasthenic syndrome. Physiopathological aspects and therapeutic modalities].

A 68-year old man experienced a progressive proximal tetraparesis with anhidrosis and a single episode of horizontal diplopia before presenting exertional dyspnea; pulmonary investigations revealed a small cell carcinoma of the lung. Clinical and electrophysiological investigations with abnormal SFEMG, repetitive stimulations and autonomic assessment pointed to a pre-synaptic neuromuscular dysfunction compatible with a Lambert-Eaton syndrome. Antibodies to acetylcholine receptors and calcium channels were negative. Thoracic radiotherapy combined with chemotherapy produced marked improvement: repeated electrophysiological evaluations showed a strong correlation between median nerve CMAP amplitude and clinical course. This case prompted us to discuss current concepts of pre-synaptic dysfunction, and paraneoplastic syndrome, and to review therapeutic strategies, in the light of recent studies of Lambert-Eaton syndrome.

Aged↗

Correlation between sensory loss, functional disability and short-latency somatosensory evoked potentials in strokes.

Short-latency somatosensory evoked potentials (SEPs) were reviewed for their correlation with the clinical features and functional deficit of 64 patients with supratentorial deep or superficial ischemic strokes. Abnormal SEPs correlated with lesions of proprioceptive pathways and with clinical sensory impairment. 87% of patients with abnormal SEPs had sensory loss and 88% of those with normal SEPs had normal sensation. The reliability of the SEPs made them useful in an objective demonstration of an abnormality in sensory system function. However, they are not sensitive enough to be used as a method of prognostication because 1) the SEPs abnormalities depend on the involvement of their generators and therefore may be normal in some thalamic or cortical lesions associated with severe disability, 2) 48.4% of the patients studied had normal sensation, and 3) in the group with absent SEPs, the functional disability was poorer than in the group with normal SEPs, not in relation to the type of the sensory loss but to the volume and location of the infarcted area.

Activities of Daily Living↗

Meningovascular form of neuroborreliosis: similarities between neuropathological findings in a case of Lyme disease and those occurring in tertiary neurosyphilis.

Recent observations have delineated the neurological manifestations of Lyme disease, but, to our knowledge, no detailed neuropathological study from autopsy cases has been reported. In this report we describe the neuropathological findings in a case of Lyme neuroborreliosis. The chronic meningitis, the occlusive meningovascular and secondary parenchymal changes that we found are similar to those occurring in the meningovascular form of neurosyphilis. Thus, we suggest that the case described here represents the meningovascular form of tertiary Lyme neuroborreliosis.

Humans↗

[Focal mononeuropathies of the lower limbs].

The various clinical pictures of lower limb's mononeuropathies are described. An essential step in the diagnosis of pressure and entrapment neuropathies is to obtain an accurate description of the patient's symptoms and to ascertain whether the sensory and motor symptoms and signs correspond to the distribution of a single peripheral nerve. EMG is a useful adjunct to the physical examination and nerve conduction studies. Computer tomogram scanning may reveal bone, joint or soft tissues abnormalities responsible for the neuropathy. The attitude and the prognosis depend on the physical and functional state of damaged nerve trunks, mainly based on the classification of neuropraxia, axonotmesis and neurotmesis.

Humans↗