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Biomedical subjects

M Donaghy

Publications and source records attributed to M Donaghy.

At least 55 records · Page 3Linked to original sources
PubMed1994

Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin.

Within one month of starting oral prednisolone treatment weakness unexpectedly increased in four patients aged 34 to 75 years with purely motor forms of acquired chronic demyelinating neuropathy. By contrast, steroids produced the expected improvement in 11 other patients with symmetric sensorimotor chronic inflammatory demyelinating polyneuropathy. Two of the patients with purely motor demyelinating neuropathy were subsequently treated with high dose IVIg (0.4 g/kg/day for five days) with prompt improvements in strength measurements and motor nerve conduction. Thus IVIg seems to be the treatment of choice and steroids should be used with extreme caution, if at all, in patients with purely motor forms of acquired demyelinating polyneuropathy.

Adolescent↗
PubMed1993

Progressive sensory nerve dysfunction in amyotrophic lateral sclerosis: a prospective clinical and neurophysiological study.

Sensory nerve function was determined in 19 patients with amyotrophic lateral sclerosis (ALS), using a battery of clinical and neurophysiological tests. This assessment was repeated on 12 patients after intervals of 6-18 months. Twelve controls were also studied. In the ALS group, only 2 patients had noticed mild sensory symptoms and none had sensory signs. Between successive studies the vibration thresholds increased, but not to a significant degree. ALS patients showed a significant fall in amplitude of the sensory nerve action potentials in the median, radial, and sural nerves (P < 0.04); sensory nerve conduction velocity did not alter. The median nerve somatosensory evoked potential N19 latency showed a highly significant increase (P < 0.008). Significant subclinical deterioration in sensory nerve function occurs in ALS, and parallels the progressive motor decline. Neuronal degeneration in ALS is not restricted to motor neurons.

Action Potentials↗
PubMed1993

Thoracic outlet syndromes and magnetic resonance imaging.

The thoracic outlet syndromes encompass the diverse clinical entities affecting the branchial plexus or subclavian artery including cervical ribs or bands. Thoracic outlet syndrome are often difficult to diagnose on existing clinical and electrophysiological criteria and new diagnostic methods are necessary. This study reports our experience with magnetic resonance imaging (MRI) of the brachial plexus in 20 patients with suspected thoracic outlet syndrome. The distribution of pain and sensory disturbance varied widely, weakness and wasting usually affected C8/T1 innervated muscles, and electrophysiology showed combinations of reduced sensory nerve action potentials from the fourth and fifth digits, and prolonged F-responses or tendon reflex latencies. The MRI study was interpreted blind. Deviation of the brachial plexus was recorded in 19 out of the 24 symptomatic sides (sensitivity 79%). Absence of distortion was correctly identified in 14 out of 16 asymptomatic sides (specificity 87.5%). The false positive rate was 9.5%. Magnetic resonance imaging demonstrated all seven cervical ribs visible on plain cervical spine radiographs. Magnetic resonance imaging also showed a band-like structure extending from the C7 transverse process in 25 out of 33 sides; similar structures were detected in three out of 18 sides in control subjects. These MRI bands often underlay the brachial plexus distortion observed in our patients. We also observed instances of plexus distortion by post-traumatic callus of the first rib, and by a hypertrophied serratus anterior muscle. If they did not demonstrate a cervical rib, plain cervical spine radiographs had no value in predicting brachial plexus distortion. We believe MRI to be of potential value in the diagnosis of thoracic outlet syndrome by: (i) demonstrating deviation or distortion of nerves or blood vessels; (ii) suggesting the presence of radiographically invisible bands; (iii) disclosing other causes of thoracic outlet syndrome apart from ribs or bands.

Adult↗
PubMed1992

Multifocal peripheral neuropathy in eosinophilic fasciitis.

Polyneuropathy may complicate eosinophilia-associated connective tissue disease. We report a multi-focal neuropathy in a patient with eosinophilic fasciitis proven by demonstrating an eosinophilic cellular infiltrate in a fascial biopsy specimen from the forearm. Sural nerve biopsy revealed lymphocytic cuffing of epineural arterioles. Although described in the L-tryptophan-related eosinophilia-myalgia syndrome, peripheral neuropathy with these features has not been previously noted in a patient with eosinophilic fasciitis who had not consumed L-tryptophan.

Adult↗
PubMed1991

Acute infection with human immunodeficiency virus presenting with neurogenic urinary retention.

Several neurological presentations of acute primary infection with HIV have recently been described. A previously unrecognised presentation with neurogenic retention of urine and sacral sensory loss is reported. The case is discussed in the context of other neurological syndromes associated with seroconversion to HIV, and of other viral causes of acute retention of urine. The importance of considering the possibility of primary HIV infection in a wide range of self-limiting neurological disorders is emphasised.

Acute Disease↗
PubMed1991

Generalized peripheral neuropathy in a dental technician exposed to methyl methacrylate monomer.

A 58-year-old dental prosthetic technician developed generalized sensorimotor peripheral neuropathy. Neurophysiologic studies showed a generalized sensorimotor neuropathy of axonal degeneration type. Examination of a sural nerve biopsy showed a moderately severe axonal neuropathy with loss of large myelinated fibers and unmyelinated axons. There was evidence of slow ongoing degeneration and considerable fiber regeneration. Electron microscopy showed increased numbers of filaments in a few fibers. These findings show resemblances to the nerve changes caused by another acrylic resin, acrylamide. We suggest that the neuropathy may have been caused by 30 years of occupational cutaneous and inhalational exposure to methyl methacrylate monomer since we excluded other recognized causes of neuropathy.

Dental Technicians↗
PubMed1990

Cerebrotendinous xanthomatosis: clinical, electrophysiological and nerve biopsy findings, and response to treatment with chenodeoxycholic acid.

A 30-year-old patient with cerebrotendinous xanthomatosis was studied over a 6-year period. The clinical manifestations were cataracts, intellectual deterioration, ataxia, palatal and pharyngeal myoclonus, corticospinal tract damage and an electrophysiologically demonstrated sensorimotor peripheral neuropathy. Peripheral motor and sensory nerve conduction velocity was slowed. Sural nerve biopsy revealed reduced densities of both myelinated and unmyelinated axons and teased fibres showed evidence of axonal regeneration and some remyelination. The loss of myelinated nerve fibres particularly affected those of larger diameter, thus contributing to the slowing of nerve conduction. Chenodeoxycholic acid treatment for two separate periods of 10 and 6 months each increased nerve conduction velocity. This electrophysiological improvement was not matched by detectable clinical neurological improvement.

Adult↗
PubMed1990

Familial multiple symmetric lipomatosis with peripheral neuropathy.

We describe coexisting peripheral neuropathy and multiple symmetric lipomatosis in 4 of 7 siblings. The absence of either condition in 3 other generations of this family suggests autosomal recessive inheritance. None of the affected siblings were alcoholic, a factor some have proposed to explain the frequent occurrence of peripheral neuropathy in sporadic multiple symmetric lipomatosis. Serum lipid studies, including apoprotein A levels, were normal. Sural nerve biopsy from 1 patient showed nerve fiber loss, predominantly affecting large myelinated fibers. The relationship between myelin sheath thickness and axon diameter was normal, arguing that this neuropathy is not due to primary axonal atrophy.

Adult↗
PubMed1989

Peripheral neuropathy associated with Castleman's disease.

Four patients with polyneuropathy complicating the plasma cell variant of Castleman's disease (angiofollicular lymph node hyperplasia) are described. The neuropathy was predominantly motor and severely disabling. Vasculopathy, papilloedema, organomegaly, endocrinopathy, oedema and paraproteinaemia were variably present in these patients. Sural nerve biopsy showed changes of both demyelination and axonal loss. Capillary proliferation and endothelial hypertrophy in the epineurium and endoneurium, similar to that seen in affected lymph nodes, suggested that a diffuse vasculopathy may contribute to the neuropathy. Serum antibody activity against a variety of neural antigen preparations was not detected in any of the patients. Two untreated patients died. Substantial improvement in the neuropathy occurred in the two patients treated with cyclophosphamide and prednisolone.

Adult↗
PubMed1989

An immunohistological and genotypic study of the plasma cell form of Castleman's disease.

The plasma cell variant of Castleman's disease (angiofollicular hyperplasia) is an uncommon and poorly understood lymphoproliferative disorder which may be associated with a wide variety of systemic features. The clinical, pathological, immunophenotypic and genotypic features of a series of five cases are presented. The clinical manifestations were variable but systemic features were present in four cases. Histologically, all five cases were similar. The characteristic follicle-like structures are composed of concentric rings of B-lymphocytes with a mantle zone phenotype, surrounding an inner core of dendritic reticulum cells. The central regions are composed of plump cells that express Factor VIII related antigen, stain with Ulex europaeus I and are associated with laminin immunoreactivity. Many of these cells stain with Ki67 indicating cellular proliferation. Between these 'follicles', plasma cells are present interspersed between high endothelial venules. Using immunohistochemical techniques two cases were polyclonal, whilst in two others unequivocal lambda/IgA restriction was present; in the other case an intermediate picture of lambda/IgA predominance was apparent. Genotypic studies in three cases indicated immunoglobulin gene rearrangements but germ line T-cell receptor genes. The significance of the apparent clonal proliferation of plasma cells is discussed and a unifying model of Castleman's disease is proposed.

Adult↗
PubMed1988

Abnormalities of the axonal cytoskeleton in giant axonal neuropathy.

Intermediate filaments accumulate abnormally in a variety of cell types in individuals with human inherited giant axonal neuropathy (GAN). A characteristic feature of this disorder is the occurrence of focal axonal enlargements filled with accumulations of neurofilaments. The minimum separations between neurofilaments in sural nerve axons of a patient with GAN were 12-30 nm compared with 24-60 nm in controls. The normal sidearm protrusions which cross-bridge adjacent filaments were rare in GAN. Average minimum neurofilament diameter was 12.4 nm in GAN compared with 10.1 nm in controls. Many axons were devoid of neurofilaments and contained an increased density of microtubules, many of which did not run longitudinally. This disorganization of microtubule alignment may reflect the lack of an associated neurofilament lattice. It is concluded that GAN involves abnormalities of neurofilament cross-linkage to one another and to adjacent microtubules. Mechanisms are discussed which could account for this inherited disorder of intermediate filament organization affecting various cell types.

Adult↗
PubMed1988

Giant axonal neuropathy: observations on a further patient.

A further child with giant axonal neuropathy (GAN), abnormally curly hair and consanguineous parents is described. Of the 19 patients with GAN so far reported in the literature, six, including the present patient, have resulted from consanguineous marriages. This makes autosomal recessive inheritance of GAN highly probable. Our patient also exhibited cerebellar ataxia and signs of pyramidal tract damage; magnetic resonance brain imaging demonstrated abnormalities within the cerebellar and cerebral white matter. Myelinated nerve fibre density in the sural nerve was reduced to 6790/mm2 at age 8 years and had fallen to 3812/mm2 16 months later, indicating that progressive axonal loss occurs in GAN.

Axons↗
PubMed1987

Hereditary sensory neuropathy with neurotrophic keratitis. Description of an autosomal recessive disorder with a selective reduction of small myelinated nerve fibres and a discussion of the classification of the hereditary sensory neuropathies.

A Kashmiri family with 3 members affected by a congenital sensory and autonomic neuropathy and corneal opacification is described. The 3 affected cases were offspring of consanguinous marriages in two generations; autosomal recessive inheritance is therefore probable. Pain and temperature sensation was lost in the limbs with a resulting mutilating acropathy. Sudomotor function was also impaired. Motor function, tendon reflexes, kinaesthetic sensation and sensory nerve action potentials were normal. Sural nerve biopsy showed a selectively reduced small myelinated nerve fibre population. Corneal histology revealed neurotrophic keratitis. The classification of the hereditary sensory and autonomic neuropathies is discussed. This family represents a previously unrecognized variant.

Adult↗
PubMed1986

Sera from patients with motor neuron disease and associated paraproteinaemia fail to inhibit experimentally induced sprouting of motor nerve terminals.

IgG kappa paraproteinaemia was discovered in one patient with amyotrophic lateral sclerosis and one with chronic spinal muscular atrophy. Serum from these patients was injected into mouse muscles paralysed by botulinum toxin which is known to induce sprouting from motor nerve terminals. Daily injection of serum for 7 days failed to inhibit terminal sprouting. It is concluded that the paraproteins did not recognise a growth factor postulated as being implicated in the pathogenesis of motor neuron disease.

Aged↗
PubMed1985

Ocular palsy preceding chronic relapsing polyneuropathy by several weeks.

Three patients exhibited an unusual variant of chronic relapsing polyneuritis in which extraocular muscle palsies developed 19 days, 20 days, and 3 1/2 months, respectively, before the neuropathy affected the limbs. The peripheral neuropathy evolved over 7 to 12 weeks, lasted 15 months to 13 years, and relapsed in every case. Each patient had dysphagia, areflexia, sensory loss, and weakness of all four limbs, which in two was asymmetrical. Slowed motor nerve conduction and prolonged F wave latencies were present, and the cerebrospinal fluid protein level was elevated without increased cell count. No patient recovered fully despite treatment with immunosuppressant drugs.

Adult↗