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Biomedical subjects

M Donaghy

Publications and source records attributed to M Donaghy.

At least 19 recordsLinked to original sources
PubMed2007

Treatment of vasculitic peripheral neuropathy: a retrospective analysis of outcome.

BACKGROUND: Vasculitis of the peripheral nervous system (PNS) is rare. There are no controlled treatment trials, and clinical practice is guided by experience from case series and indirectly by analogy with systemic vasculitis. METHODS: We identified patients (n=212) with possible vasculitic peripheral neuropathy (VPN) from the neuropathology and neurophysiology records of two centres over 28 years. Case-notes were available for 181, from which, 106 cases of clinicopathological VPN were identified. Adequate treatment data were available in 100; follow-up data, in 93. RESULTS: Of 106 cases, 95 had systemic vasculitis and 11 had vasculitis confined to the PNS. Pharmacological treatment (94/100 cases) was corticosteroid-based, and included cyclophosphamide in 54; 17 received additional agents. Initial stabilization was achieved in all but six. One-year survival was 90.3%. Of the nine who died in the first years (mean age 73 years), seven had received cyclophosphamide, and all but two had severe, multisystem vasculitis. The neurological relapse rate was 10%. Only one relapse occurred after cyclophosphamide treatment. Outcome was reported as good in 72% (78% in those who relapsed). DISCUSSION: Death and relapse were infrequent in treated patients. Relapse occurred almost exclusively in patients treated with prednisolone alone. Aggressive early treatment with cyclophosphamide may prevent relapse. The current management approach to VPN appears largely effective, especially if cyclophosphamide is used.

Adult↗
PubMed2006

Clinical features of double infection with tick-borne encephalitis and Lyme borreliosis transmitted by tick bite.

BACKGROUND: In Latvia and other endemic regions, a single tick bite has the potential to transmit both tick-borne encephalitis (TBE) and Lyme borreliosis. OBJECTIVE: To analyse both the clinical features and differential diagnosis of combined tick-borne infection with TBE and Lyme borreliosis, in 51 patients with serological evidence, of whom 69% had tick bites. RESULTS: Biphasic fever suggestive of TBE occurred in 55% of the patients. Meningitis occurred in 92%, with painful radicular symptoms in 39%. Muscle weakness occurred in 41%; in 29% the flaccid paralysis was compatible with TBE. Only two patients presented with the bulbar palsy typical of TBE. Typical Lyme borreliosis facial palsy occurred in three patients. Typical TBE oculomotor disturbances occurred in two. Other features typical of Lyme borreliosis detected in our patients were distal peripheral neuropathy (n = 4), arthralgia (n = 9), local erythema 1-12 days after tick bite (n = 7) and erythema chronicum migrans (n = 1). Echocardiogram abnormalities occurred in 15. CONCLUSIONS: Patients with double infection with TBE and Lyme borreliosis fell into three main clinical groups: febrile illness, 3 (6%); meningitis, 15 (30%); central or peripheral neurological deficit (meningoencephalitis, meningomyelitis, meningoradiculitis and polyradiculoneuritis), 33 (65%). Systemic features pointing to Lyme borreliosis were found in 25 patients (49%); immunoglobulin (Ig)M antibodies to borreliosis were present in 18 of them. The clinical occurrence of both Lyme borreliosis and TBE vary after exposure to tick bite, and the neurological manifestations of each disorder vary widely, with considerable overlap. This observational study provides no evidence that co-infection produces unusual manifestations due to unpredicted interaction between the two diseases. Patients with tick exposure presenting with acute neurological symptoms in areas endemic for both Lyme borreliosis and TBE should be investigated for both conditions. The threshold for simultaneous treatment of both conditions should be low, given the possibility of co-occurrence and the difficulty in ascribing individual neurological manifestations to one condition or the other.

Bites and Stings↗
PubMed2006

Causes of lumbosacral plexopathy.

The lumbosacral plexus represents the nerve supply to the lower back, pelvis and legs. This review will focus on diseases and disorders affecting the pathway as demonstrated by magnetic resonance imaging (MRI) and computed tomography (CT). We stress the need to review the lumbosacral plexus in patients with non-specific symptoms such as back, hip, pelvic pain, and in those who present with sciatica unaccompanied by demonstrable intervertebral disc prolapse. We illustrate that the imaging appearances may be non-specific and re-inforce the importance of the clinical history and the use of tissue sampling to achieve an accurate diagnosis.

Abscess↗
PubMed2005

A practical definition of conduction block in IvIg responsive multifocal motor neuropathy.

BACKGROUND: Multifocal motor neuropathy with conduction block (MMN) can be mistaken for motor neurone disease or other lower motor neurone syndromes, but is treatable with intravenous immunoglobulin (IvIg). Formal electrophysiological criteria for conduction block (CB) are so stringent that substantial numbers of patients may miss out on appropriate treatment. METHODS: Electrophysiological data were collected from 10 healthy volunteers and compared to data from 10 patients who satisfied the clinical criteria for MMN and who responded to IvIg. This produced a definition of CB in MMN patients which was compared with existing definitions to assess "miss rates". RESULTS: Mean values for compound muscle action potential area, amplitude, and duration were calculated in normal subjects. Results beyond 3 SD of their respective means were considered abnormal. Using these criteria, CB in the context of MMN was defined as a reduction in negative peak area >23% along a distal nerve segment or >29% across a proximal segment; or a reduction in amplitude >32% across a distal segment or >33% across a proximal segment. All IvIg responsive patients had at least one nerve segment showing such CB. Employing some criteria from the literature would have denied treatment to over 30% of responsive patients. CONCLUSION: In the clinical setting of suspected MMN, less stringent criteria for CB can improve the diagnosis of this treatable disorder. Exclusions on grounds of temporal dispersion may be over-restrictive. A little over one third of CBs occur proximally.

Action Potentials↗
PubMed2005

The effect of treatment upon temporal dispersion in IvIg responsive multifocal motor neuropathy.

BACKGROUND: Multifocal motor neuropathy with conduction block (MMN) is a treatable disorder that can be mistaken for other lower motor neurone syndromes. Existing electrophysiological diagnostic criteria for MMN are restrictive. In particular, many are cautious about diagnosing conduction block (CB) in the presence of abnormal temporal dispersion (TD). OBJECTIVE: To study the significance of TD in MMN, its relationship to CB in intravenous immunoglobulin (IvIg) responsive patients, and its utility in detecting a treatment response. METHODS: We compared pre- and post-treatment changes in CB and TD in nine patients who satisfied clinical and electrophysiological criteria for MMN and responded to IvIg. RESULTS: TD improved in one or more nerve segments in eight of nine patients tested. There was marked improvement in 65% of all nerve segments, and 60% of those segments with CB. By comparison, significant improvement in CB occurred in only 33% of segments. Of segments with significantly better CB after treatment, all but one showed similar improvements in TD. Such changes were not related to the degree of TD before treatment, being seen in segments with abnormal as well as normal TD. There was no correlation between improvements seen in TD and CB. CONCLUSION: We believe that TD should be considered an inherent feature of MMN. Improvement in TD is an independent marker of electrophysiological improvement in this disorder and is likely to be more useful than CB. When MMN is clinically suspected, the use of stringent criteria for CB in the presence of TD should be avoided.

Action Potentials↗
PubMed2005

Diphtheria with polyneuropathy in a closed community despite receiving recent booster vaccination.

INTRODUCTION AND METHODS: We report 20 patients aged 18-24 years from Latvia with diphtheritic polyneuropathy. All lived in a closed community and 80% were known to have been fully vaccinated against diphtheria until at least 14 years old. Diphtheria antitoxin had been administered within 3 days of the onset of upper respiratory tract infection in 16 patients and 15 received antibiotics. RESULTS: Neurological symptoms developed after a median of 43 days (range 35-58) compared to only 10 days in previous studies of unvaccinated patients. All showed evidence of mild limb polyneuropathy with electrophysiological evidence of polyneuropathy. Only 30% showed early bulbar abnormalities compared to the usual rate of over 95% in diphtheritic polyneuropathy. However, 45% had later bulbar deterioration coinciding with the limb polyneuropathy. CONCLUSIONS: These patients show that an attenuated form of polyneuropathy of later onset, with less prominent early bulbar features, can occur in patients vaccinated against diphtheria according to schedule but living in a closed community in a country where diphtheria remains endemic.

Adolescent↗
PubMed2002

An expanded cortical representation for hand movement after peripheral motor denervation.

OBJECTIVES: Functional reorganisation of the motor or sensory cortex has been demonstrated in animals after section of mixed peripheral nerves. Here functional changes in the motor cortex specifically after peripheral motor denervation in humans are investigated. METHODS: Functional MRI (fMRI) was used to study brain activation during a finger flexion-extension task in patients with a late onset, acquired pure motor neuropathy (n=6), contrasting results with those from patients with pure sensory neuropathies (n=4) or healthy controls (n=7). RESULTS: Increases in the extent of activation in the motor cortex both ipsilateral and contralateral to the hand moved were found in the patients with motor neuropathy. The neuroanatomical localisation of the mixed contralateral sensorimotor cortex activation volume was more posterior for the patients with motor neuropathy than for the healthy controls (mean difference, 12 mm, p<0.05). The pure sensory neuropathy group by contrast showed no change in the extent of activation relative to healthy controls and a trend for more anterior primary sensorimotor cortex activation (p<0.06). To test whether the increased activation volumes found in patients with motor neuropathy were a result simply of factors such as increased effort with movement rather than the motor denervation, patients with hand weakness from inclusion body myositis (n=4) were studied while making similar hand movements. No differences in either the numbers of significantly activated voxels or in their localisation were found relative to healthy controls (n=10). CONCLUSIONS: These results provide a novel demonstration that peripheral denervation (as distinguished from factors related to weakness) leads to functional reorganisation of the sensorimotor cortex in the adult brain. This suggests that adaptive responses to motor denervation involve the central as well as the peripheral nervous system.

Adult↗
PubMed2002

Duration, frequency, recency, and type of migraine and the risk of ischaemic stroke in women of childbearing age.

BACKGROUND: Migraine is recognised increasingly as a risk factor for ischaemic stroke in women of childbearing age. Migraine with aura poses a higher risk than migraine without aura. OBJECTIVE: To investigate further the effect of duration, frequency, recency, and type of migraine on the risk of ischaemic stroke. METHODS: Additional analyses of a previously reported multicentre case-control study of the relation between stroke and migraine in women aged 20-44 years. RESULTS: Among 86 cases of ischaemic stroke and 214 controls, the adjusted risk of ischaemic stroke was significantly associated with: (1) migraine of more than 12 years duration, odds ratio (OR) 4.61 (1.27-16.8); (2) initial migraine with aura, OR 8.37 (2.33-30.1); (3) particularly if attacks were more frequent than 12 times per year, OR 10.4 (2.18-49.4). In no case did correction for oral contraception usage significantly alter these odds ratios. Increasing risk of ischaemic stroke was related to a change to increased frequency of headaches (trend p <or= 0.03). CONCLUSIONS: These data support earlier reports of a relation between ischaemic stroke and migraine with aura. The risk seems particularly high in those whose initial migraine type involved aura occurring more than 12 times per year.

Adult↗
PubMed2002

Clinical and genetic heterogeneity in peroneal muscular atrophy associated with vocal cord weakness.

BACKGROUND: The peroneal muscular atrophy syndrome is the most common inherited disorder of the peripheral nervous system and has extensive clinical and genetic heterogeneity. Cranial nerve involvement is rare, though there are distinct peroneal muscular atrophy syndromes in which vocal cord paralysis is a characteristic feature. Among these dHMN-VII and HMSN-IIC are clinically similar but are differentiated by sensory involvement in HMSN-IIC. The gene for dHMN-VII, designated DHMNVP, has been localised to chromosome 2q14, but the location of the gene for HMSN-IIC is currently unknown. It has been suggested that dHMN-VII and HMSN II-C are allelic disorders. OBJECTIVE: To assess the contribution of the dHMN-VII predisposition gene to peroneal muscular atrophy syndromes associated with vocal cord weakness. METHODS: Linkage analysis of microsatellite markers at chromosome 2q14 was undertaken on two families, one affected by HMSN-IIC and a second manifesting vocal cord paralysis and sensorineural deafness in addition to distal muscular atrophy. RESULTS: Two-point LOD scores at chromosome 2q14 markers encompassing the DHMNVP gene were negative in both families. CONCLUSIONS: These results suggest that at least one further gene predisposing to distal muscular weakness in association with vocal cord paralysis is likely to exist, and that dHMN-VII and HMSN-IIC are unlikely to be allelic disorders. Analyses of further HMSN-IIC families are required to confirm this.

Charcot-Marie-Tooth Disease↗
PubMed2001

Altered cortical activation with finger movement after peripheral denervation: comparison of active and passive tasks.

We wished to contrast cortical activation during hand movements in profoundly weak patients with motor neuropathy and in normal controls using a paradigm that is behaviourally matched between the two groups. Previous work has suggested that a passive movement task could be appropriate. Using functional magnetic resonance imaging (fMRI), we first characterised patterns of brain activation during active and passive index finger movements in healthy controls (n=10). Although the relative activation differences were highly variable, there was a trend for the mean number of significantly activated voxels in the primary motor cortex contralateral to the hand moved (CMC) to be lower for the passive than for the active task (40% relative decrease, P=0.09). There was a small posterior shift in the centre of mass of the CMC (mean, 8 mm, P<0.02) and of the ipsilateral sensorimotor cortex (IMC) (mean, 11 mm, P<0.05). No activation with passive movement was found in the patients with severe distal sensory neuropathy (n=2), suggesting that activation with passive movements is dependent on sensory feedback and unlikely to be due to mental imagery alone. In contrast, patients with severe pure motor neuropathies (MN, n=2) showed substantial increases in the volumes of activation compared to controls. The relative increases in numbers of voxels activated above threshold in different regions of interest for both the active (MN/controls: CMC, 2. 1; IMC, 8.1; supplementary motor area [SMA], 5.2) and passive (CMC, 2.6; IMC, 8.0; SMA, 5.1) tasks were similar. These results confirm expansion of cortical representation for finger movement in patients with motor neuropathy and demonstrate central reorganisation as a consequence of the motor nerve loss. An expanded representation for finger movement in the primary motor cortex with peripheral weakness suggests the possibility that the primary motor cortex may encode motor unit activation rather directly.

Adult↗
PubMed2001

Epidemiological investigation of the central Scotland outbreak of Escherichia coli O157 infection, November to December 1996.

On Friday, 22 November 1996, the microbiologist at a hospital in Lanarkshire, Scotland, identified presumptive Escherichia coli O157 in faecal specimens submitted by three patients with bloody diarrhoea, and confirmed its presence in one. Over the next 6 h, 12 more potential cases were identified. Investigations first indicated then confirmed a single food premises as the source of infection. Effective control measures were applied promptly. The outbreak was declared over on 20 January 1997, by which time 512 cases had been identified, and infection with the outbreak strain confirmed in 279. Twenty deaths occurred in cases during the outbreak and there were two more in cases during 1997. Seventeen of these deaths resulted from the outbreak. This paper describes the outbreak's epidemiological investigation, referring to other investigations, and control measures, where appropriate.

Adolescent↗
PubMed2001

Monitoring patients in the community with suspected Escherichia coli O157 infection during a large outbreak in Scotland in 1996.

During outbreaks of Escherichia coli O157 a minority of patients with suspected infection develop haemolytic uraemic syndrome (HUS). The ability to identify this subgroup at an early stage is beneficial as mortality from HUS is high and may be influenced by intervention. During the 1996 Central Scotland E. coli O157 outbreak, of 886 patients from the community with suspected infection monitored at an outbreak clinic, nine developed HUS. We assessed factors associated with the development of HUS in this group. Children and the elderly were at increased risk of HUS. However, high white cell count was as least as good a predictor of HUS as age. High white cell counts predicted development of HUS with a sensitivity of 89%, specificity of 87%, positive predictive value of 7% and a negative predictive value of over 99%. We have used the results from this study along with other currently available evidence to propose a monitoring protocol for patients from the community with suspected E. coli O157 infection.

Adolescent↗
PubMed2001

The clinical and laboratory features of chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies.

The clinical and laboratory phenotype of a paraproteinaemic neuropathy syndrome termed chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies is described in a series of 18 cases. Previous single case reports have outlined some features of this syndrome. All 18 cases were defined by the presence of serum IgM antibodies which react principally with NeuAc (alpha2-8)NeuAc(alpha2-3)Gal-configured disialosyl epitopes common to many gangliosides including GDlb, GD3, GTlb and GQlb. In 17 out of 18 cases, the serum contained benign IgM paraproteins, and in four of these cases at least two IgM paraproteins were present. The IgM antibodies were also cold agglutinins in 50% of cases. The clinical picture comprised a chronic neuropathy with marked sensory ataxia and areflexia, and with relatively preserved motor function in the limbs. In addition, 16 out of 18 cases had motor weakness affecting oculomotor and bulbar muscles as fixed or as relapsing-remitting features. When present in their entirety, these clinical features have been described previously under the acronym CANOMAD: chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies. This distribution of clinical features is reminiscent of Miller Fisher syndrome, in which acute-phase anti-disialylated ganglioside IgG antibodies are found. Clinical electrophysiology and nerve biopsy show both demyelinating and axonal features. A partial response to intravenous immunoglobulin and other treatments is reported in some cases.

Adult↗
PubMed2001

Distinct combinations of Borrelia burgdorferi sensu lato genospecies found in individual questing ticks from Europe.

The genetic diversity of Borrelia burgdorferi sensu lato was assessed in individual adult Ixodes ricinus ticks from Europe by direct PCR amplification of spirochetal DNA followed by genospecies-specific hybridization. Analysis of mixed infections in the ticks showed that B. garinii and B. valaisiana segregate from B. afzelii. This and previous findings suggest that host complement interacts with spirochetes in the tick, thereby playing an important role in the ecology of Lyme borreliosis.

Animals↗