PubMed Health⌕ Search

Biomedical subjects

E Ellie

Publications and source records attributed to E Ellie.

At least 37 records · Page 2Linked to original sources
PubMed1994

Peripheral neuropathy after autologous blood stem cell transplantation for multiple myeloma.

We report a case of peripheral neuropathy occurring after autologous blood stem cell transplantation (ABSCT) for multiple myeloma. The patient, free of neurological symptoms, was transplanted in partial remission, and achieved a complete remission after transplantation. A severe peripheral, symmetric, distal sensori-motor polyneuropathy appeared at day 25 and worsened progressively until commencement of corticosteroid therapy. A peripheral nerve biopsy showed endoneurial cellular infiltrates which were predominantly composed of T cells identified by immunocytochemistry. Ultrastructural examination showed acute axonal damage. Electrophysiologic studies performed before and during the treatment were consistent with a severe axonal degeneration and showed a marked improvement, concomitant with the favorable clinical outcome. This is the first report of peripheral neuropathy after ABSCT.

Axons↗
PubMed1993

Malignant infiltration of peripheral nerves in the course of acute myelomonoblastic leukaemia: neuropathological study of two cases.

Two patients suffering from acute myelomonoblastic leukaemia developed clinical evidence of peripheral nerve involvement. In both cases, a peripheral nerve biopsy revealed endoneurial cellular infiltrates which were identified as leukaemic cells by immunocytochemistry. Ultrastructural studies showed mainly axonal damage. Moreover, in one patient, a few features of active demyelination were also observed.

Action Potentials↗
PubMed1993

Late central demyelination after Fischer's syndrome: MRI studies.

The case of a patient who presented with clinical, electrophysiological, and MRI evidence of central demyelination is described. The patient had been admitted to hospital for Fischer's syndrome a few years previously. The association of these two events suggests that central and peripheral myelinopathy may be related in Fischer's syndrome.

Ataxia↗
PubMed1993

Isolated peripheral nerve relapse masquerading as Guillain-Barré syndrome in a patient with acute lymphoblastic leukemia.

We report a 30 year old patient with acute lymphoblastic leukemia (ALL) whose leukemic relapse presented as an isolated symmetrical peripheral neuropathy with facial diplegia. Initially, this was consistent with a Guillain-Barré syndrome but the peripheral nerve biopsy revealed leukaemic infiltration. This was followed by a systemic relapse. Reports of peripheral nerve infiltration are scarce and to the best of our knowledge this is the first documented case of peripheral polyneuropathy as a presenting manifestation of ALL relapse.

Adult↗
PubMed1993

[Limb-girdle syndrome. A study of 46 cases].

We report a series of 46 patients (32 women and 14 men) with limb-girdle syndrome. After reappraisal, another diagnosis was made in 10 of them. Becker's muscular dystrophy was the most frequent cause among men (near 50 p. 100). A Duchenne muscular dystrophy manifesting carrier was discovered among 13 reevaluated women. Among the 36 cases (29 women and 7 men) without any defined etiology, 29 were without any other known familial history. Fifteen of these women had similar clinical findings: incipient weakness in the pelvic girdle and onset of symptoms most often in the forties. In these cases serum creatine kinase activity was normal or slightly elevated, and muscle biopsy showed non-specific patterns. "Late onset muscular dystrophy in females" should be reevaluated.

Adult↗
PubMed1993

[Paraneoplastic cerebral venous thrombosis].

A 45 year-old patient presented with an epidermoid carcinoma of the tongue and cranial hypertension. MRI showed dural venous sinus occlusion, without infiltration or compression by a metastatic tumor. Anticoagulation led to recanalization of the occluded sinuses, as shown by the follow-up MRI, three months later. The pathophysiology of these rare paraneoplastic cerebral venous thrombosis is discussed.

Carcinoma, Squamous Cell↗
PubMed1992

[Sensory neuropathies].

This review paper analyzes the different steps in the diagnosis of peripheral sensory neuropathies. Although electrodiagnostic tests are almost invariably needed, other investigations should be performed according to an accurate prior clinical evaluation. Some causes are frequent and easy to discover, such as diabetes and chronic renal failure. On the opposite, genetically determined and dysimmune neuropathies are less frequent, and may go unrecognized for a long time. A careful survey of the clinical, biological and electrophysiological features sometimes discloses a specific aetiology of an initially unclassified neuropathy.

Adult↗
PubMed1992

Unilateral diaphragmatic paralysis: an electrophysiological study.

An electrophysiological study was carried out on four patients with unilateral diaphragmatic paralysis. Whereas neurogenic involvement of the paralysed hemidiaphragm was roughly similar in all cases, neurogenic patterns could be detected in the normally moving contralateral hemidiaphragm in three cases, and the degree of involvement could be correlated with the respiratory state of the patients. EMG also showed that the neuropathic process affected the limb muscles. Thus unilateral diaphragmatic paralysis may be, at least in some cases, the localised expression of a more diffuse neuropathy, perhaps a peculiar form of neuralgic amyotrophy.

Adult↗
PubMed1992

[Autosomal dominant centronuclear myopathy].

In a family 6 members in 3 generations were affected by centronuclear myopathy (CNM) of autosomal dominant inheritance. The apparent onset was in the early forties and the disease progressed slowly. Limb weakness was predominant. Strabismus was present in 5 cases and calves hypertrophy in 3. Serum creatinine kinase was always within the normal range. In one case myotonic bursts were found at electromyography. In 2 cases brain stem auditory evoked potential studies demonstrated abnormal prolongation of interpeak latencies I-III and favoured subclinical nervous system involvement. Muscular biopsies showed typical features of centronuclear myopathy with 50 to 80% central nuclei. In two cases immunocytochemical labelling of dystrophin showed staining in the sarcoplasm in favour of an arrest in the morphogenesis of developing myofiber. Others families with autosomal dominant CNM in the literature and also some sporadic adult cases had similar clinical features.

Adult↗
PubMed1991

Voltage-dependent calcium and potassium channels in Schwann cells cultured from dorsal root ganglia of the mouse.

1. Whole-cell patch clamp studies were carried out on Schwann cells in organotypic cultures of dorsal root ganglia (DRG) from OF1 mice embryos (18-19 days). 2. In standard external solution, from a holding potential of -70 mV, two types of voltage-dependent K+ currents were recorded: a fast transient current and a delayed sustained current. With a holding potential of -30 mV, only the delayed sustained current could be evoked. 3. Both K+ currents were inhibited by tetraethylammonium chloride (TEA) and 4-aminopyridine (4-AP) in a dose-dependent manner. For the transient current the half-maximal effective dose was 100 mM for TEA and 1.3 mM for 4-AP. For the delayed sustained current the half-maximal effective dose was 11 mM for TEA and 4 mM for 4-AP. Both currents were insensitive to external Ca2+. 4. The delayed sustained current, isolated by use of a holding potential of -30 mV displayed a 'cumulative inactivation' which was removed by hyperpolarizing the membrane to -70 mV between each test pulse. 5. In K(+)-free external and pipette solutions, with 10 mM-external Ca2+, from a holding potential of -70 mV voltage-dependent Ca2+ channel currents were recorded. The threshold for activation was -45.3 +/- 5.4 mV (mean +/- S.D., n = 5) and the current inactivated fully at the end of the test potential. The current was unaffected by 2 microM-tetrodotoxin (TTX) and totally blocked by 5 mM-Co2+. 6. Equimolar replacement of external Ca2+ by Ba2+ did not significantly modify the voltage dependence (threshold for activation -42.8 +/- 6.4 mV, n = 7) or the magnitude of the inward current. Ca2+ and Ba2+ were equally permeant. The fully inactivating current was insensitive to both nifedipine and Bay K 8644 (1 microM each). Increasing the external Ba2+ concentration from 10 to 89 mM enhanced the Ba2+ current and shifted the voltage dependence of the current (threshold for activation, -30.5 +/- 7.3 mV, n = 9) along the voltage axis as expected for altered external surface potential. 7. In 89 mM-external Ba2+ solution, some cells displayed an additional slowly decaying current which was totally blocked by nifedipine (1 microM). 8. Ca2+ channel currents were recorded only when DRG neurons were present in the culture, as excision of explants and subsequent axonal degeneration led to loss of detectable Ca2+ channel currents. This phenomenon was never observed for K+ currents. 9. We conclude that mouse Schwann cells in organotypic culture possess voltage-dependent K+ and Ca2+ channels.(ABSTRACT TRUNCATED AT 400 WORDS)

4-Aminopyridine↗
PubMed1991

[Sensory neuropathy and subacute dysautonomia. Clinical and pathological study].

We report a case of severe subacute autonomic and sensory neuropathy in a 52 year-old man. Cerebrospinal fluid protein was 275 mg/dl. Electrophysiological data were consistent with an axonal sensory neuropathy. Nerve biopsy showed a severe decrease in myelinated fibers, and a less severe loss of unmyelinated fibers. No cause was found and recovery was almost complete over 4 years, with minimal persistent dysautonomia. This case and 4 similar reported cases are compared with pure dysautonomia and with sensorimotor dysautonomic neuropathy. The site of damage is discussed and it is suggested that these cases are axonal forms of inflammatory polyneuropathy.

Autonomic Nervous System Diseases↗