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Microsoft word - abstracts march 2011.doc
Infantile Spasms and Treatment. J.
Martinez, S. Penfold, Z. Agirre-Arrizubieta.
conditioning stimuli, however, indicate that in
(Department of Clinical Neurophysiology, East
some groups of fibres accumulation of potassium
Kent Hospitals University NHS Foundation
may also play a significant role. This may have
implications when interpreting results in muscle
children with infantile spasms (IS) during a three-
year-period (2007-2010) at East Kent Hospitals.
Corneal Confocal Microscopy: A
Novel Means to Detect Nerve Fibre Damage in
obtain aetiology, neurological findings, MRI,
video-EEG studies, treatment and follow-up.
Marshall¹, M. Tavakoli², M. Roberts¹, N.
Efron³, A.J. Boulton AJ ², R.A. Mallik² .
studied. Aetiology: symptomatic in 9 patients and
(Salford Royal NHS Foundation Trust, UK¹,
unknown in 1. Most common IS: flexion in
Central Manchester Foundation Trust and
43.6% and mixed in 36.4%. Therapeutic response
was broadly satisfactory: 60% seizure free; 20%
partial response, 10% with more than 50%
decrease and 10% with no IS but other seizures.
Idiopathic small fibre neuropathy (ISFN)
Polytherapy: noted in 70% of patients. At follow-
is associated with intraepidermal nerve fibre loss
up, deve1opment was normal in 1 patient and
and an increased prevalence of impaired glucose
delayed in 9 (2 mild, 3 moderate and 2 profound).
tolerance (IGT). It has been suggested that the
dysglycaemia of IGT and additional metabolic
challenging. In East Kent Hospitals, the
risk factors contribute to small nerve fibre damage
commonest current therapy is a combination of 2
of the following: ACTH, steroids, vigabatrin and
topiramate. The outcome and prognosis of IS
matched control subjects underwent detailed
appeared similar or mildly improved compared
evaluation of neuropathic symptoms, neurological
with previous experience. However, safer and
deficits (Neuropathy deficit score (NDS); Nerve
more effective therapies are needed to improve
Conduction Studies (NCS); Quantitative Sensory
Testing (QST) and Corneal Confocal Microscopy
(CCM)) to quantify small nerve fibre pathology.
Velocity Recovery Cycles in Biceps
patients with ISFN had significant neuropathic
Brachii: New Insights Into The Origin of The
symptoms, NDS, NCS and QST except for warm
Early Supernormality. C.E.G Moore1,3, R.
thresholds were normal. Corneal sensitivity was
Arunachalam2 and D.C. Allen.2,4 (Departments
reduced and CCM demonstrated significant
of Clinical Neurophysiology Portsmouth NHS
reductions in corneal nerve fibre density
Trust1 and Southampton University Hospital
(P<0.0001), nerve branch density (P<0.0001),
NHS Trust2 and Universities of Portsmouth3
nerve fibre length (P<0.0001) and an increase in
and Southampton4, UK).
nerve fibre tortuosity (P<0.0001). These
parameters did not differ between ISFN patients
conduction velocity recovery cycles (VRCs) using
with and without IGT or correlate with BMI,
paired stimuli are technically demanding and time
consuming. We have previously reported the use
of an automated computer driven methods to
means to detect early small nerve fibre damage in
investigate VRCs in tibialis anterior (TA). This
patients with ISFN and metabolic abnormalities
has shown promise in the investigation of muscle
disease, in particular critical illness myopathy.
Are There Differences Between Han
investigation of biceps. Muscle fibre potentials
Chinese and Caucasians in Transcranial
were recorded using standard technique for direct
Magnetic Stimulation (TMS) Parameters?
muscle stimulation. VRCs were recorded with 1,
Xiang Yi¹, K. Fisher², K. Mansoor³, Ming Lai³,
2 and 5 conditioning stimuli (10ms apart) prior to
the test stimulus. The interstimulus intervals (ISI)
between the last conditioning stimulus and the test
Infirmary³, Newcastle, UK).
biceps was greater than in TA. In addition there
studying a range of TMS parameters in Chinese
were distinct differences in the amount of early
and Caucasian subjects. Sixteen subjects were
supernormality after 1, 2 or 5 conditioning
studied in each group. A circular coil at the vertex
stimuli. The early supernormality has hitherto
was used for stimulation, whilst recording surface
been explained purely in terms of membrane
electromyograms (EMG) from right first dorsal
capacitance and should be independent of the
interosseous. In the passive state we measured
number of conditioning pulses. Our findings of
motor evoked potential (MEP) threshold, MEP
recruitment, short interval intracortical inhibition
like Angelman syndrome , brain malformation or
(SICI) and intracortical facilitation (ICF). The
MEP threshold, recruitment and silent period
features of two adult patients with MSNE and
discuss diagnostic and management challenges in
higher passive thresholds (p<0.01), less inhibition
this difficult to treat epilepsy syndrome. The
in of the motor response (SICI p< 0.01 ) and the
recognition of MSNE resulted in the correct
silent period was shorter (p< 0.05 ).
identification of Angelman syndrome in one
patient who was otherwise considered to have
Cortical Reorganisation in MND? A
learning disability of undetermined aetiology.
Study Using Pharmacological fMRI. S. Azam.
(Royal Free Hospital. London, UK).
Examining the Reproducibility of
Median Motor Nerve Excitability Testing. J.C.
(MND) progresses there may be pathological or
compensatory changes in cortical motor network
(Department of Clinical Neurophysiology, St.
Vincent’s Hospital Group, Dublin, Ireland1
representation, which may be due to excitotoxic
and Department of Neural Engineering,
damage of local inhibitory circuits leading to
Trinity College Dublin, Ireland2).
inappropriate activation of the motor pathway or
due to increased peripheral motor effort leading to
functional assessment of axonal membrane
function in peripheral nerve, and the technique
has potential for use in longitudinal studies.
imaging of the blood oxygentation response
(BOLD-fMRI) during a calibrated handgrip task
reliability of nerve excitability in a healthy cohort.
of 5-30% maximum grip strength was performed
in 12 healthy controls, 12 MND subjects and 12
motor excitability studies performed by the same
subjects with multifocal motor neuropathy
operator on three occasions (twice on the same
(MMN), under placebo (i.v. saline) or midazolam
day, and once after one week). ANOVA was
conditions and images analysed with statistical
used to analyse and compare the within-subject
and between-subject variances for different
fMRI signal increases were seen in all groups in
contralateral primary sensorimotor cortex and
testing had excellent reproducibility. The
ipsilateral cerebellum, and statistically similar
parameters with the greatest reproducibility were
decreases in BOLD activation of the cortical
superexcitability and minimum I/V (current
threshold) slope. F-ratio of between-subject
versus within-subject variance 7.30 (p < 5x10-6)
motor cortex reorganisation in patients with
for superexcitability and 7.0 (p < 5x10-5) for
MND, using this calibrated motor paradigm; the
minimum I/V slope). The least reproducible
parameters were strength-duration time constant,
following midazolam in all groups suggests that
and threshold parameters such as rheobase, in
neuronal inhibition via GABA potentiation is not
which the between-subject and within-subject
differences were not significantly different.
Conclusion: Nerve excitability testing is
Myoclonic Status in Nonprogressive
a reliable test over time, and should be suitable
Encephalopathies (MSNE) – Presentation in
for use in longitudinal patient studies.
Two Adult Cases. R. Ramdass, M. Jones, R.
MacDonagh, H. Kargwell, A. Marshall, R.
Mohanraj. (Departments of Neurology and
Excitability Demonstrate Rapid And Sustained
Neurophysiology, Royal Salford Hospitals
Improvement in Nerve Function Following
NHS Trust, Salford, UK).
Renal Transplantation. C.E.G. Moore1, 3, M.
Todd2, J. Mason2, M. Connell2. (Departments
of Clinical Neurophysiology1 and Renal
syndrome in development characterized by
diffuse EEG abnormalities associated with
positive and/or negative phenomena correlated
with transient or recurring motor,cognitive or
behavioural disturbances. MSNE is now included
important feature of advanced chronic kidney
in the ILAE classification of electroclinical
disease. The pathophysiology of ‘uraemic
neuropathy’ remains ill understood. Measures of
axon excitability have implied that chronic
diagnostic workup and suggest an underlying
depolarisation, secondary to hyperkalaemia, may
cause such as the presence of a genetic syndrome
be the major pathological factor. The ability to reduce potassium levels with dialysis is however,
short lived and a more prolonged normalisation of the associated depolarisation may be required (Ref).
receiving a living-related renal transplant. Testing was performed 8 months, and 1 day pre-transplant and at 1 day, and 3 months post-transplantation. 8 months prior to transplantation peripheral nerve excitability was significantly diminished, with threshold
hyperpolarising stimuli (‘fanning-in’) (p<0.005) and reduced sub- and superexcitability in the recovery cycle (p<0.02). These abnormalities worsened up until transplantation.
marked recovery towards normal. This was too rapid to be explained by changes in neuronal cytostructure or remyelination. By three months post-transplantation all measures of axonal excitability had normalised. The neuropathy symptom score had improved and there was no further deterioration in ‘routine’ nerve conduction studies.
These findings show ‘proof of principle’
that decreasing the amount of hyperkalaemia related depolarisation improves nerve membrane function with a potential reversal of neuropathy.
Ref: Krishnan et al. Brain 2005;128:2164-74
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