PMCC PMCC

Search tips
Search criteria

Advanced
Results 1-16 (16)
 

Clipboard (0)
None

Select a Filter Below

Journals
Authors
more »
Year of Publication
1.  Sensory neuropathy as part of the cerebellar ataxia neuropathy vestibular areflexia syndrome 
Neurology  2011;76(22):1903-1910.
Objective:
The syndrome of cerebellar ataxia with bilateral vestibulopathy was delineated in 2004. Sensory neuropathy was mentioned in 3 of the 4 patients described. We aimed to characterize and estimate the frequency of neuropathy in this condition, and determine its typical MRI features.
Methods:
Retrospective review of 18 subjects (including 4 from the original description) who met the criteria for bilateral vestibulopathy with cerebellar ataxia.
Results:
The reported age at onset range was 39–71 years, and symptom duration was 3–38 years. The syndrome was identified in one sibling pair, suggesting that this may be a late-onset recessive disorder, although the other 16 cases were apparently sporadic. All 18 had sensory neuropathy with absent sensory nerve action potentials, although this was not apparent clinically in 2, and the presence of neuropathy was not a selection criterion. In 5, the loss of pinprick sensation was virtually global, mimicking a neuronopathy. However, findings in the other 11 with clinically manifest neuropathy suggested a length-dependent neuropathy. MRI scans showed cerebellar atrophy in 16, involving anterior and dorsal vermis, and hemispheric crus I, while 2 were normal. The inferior vermis and brainstem were spared.
Conclusions:
Sensory neuropathy is an integral component of this syndrome. It may result in severe sensory loss, which contributes significantly to the disability. The MRI changes are nonspecific, but, coupled with loss of sensory nerve action potentials, may aid diagnosis. We propose a new name for the condition: cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS). Neurology® 2011;76:1903–1910
doi:10.1212/WNL.0b013e31821d746e
PMCID: PMC3115806  PMID: 21624989
2.  Relationships between paediatricians and infant formula milk companies 
Archives of Disease in Childhood  2006;91(5):383-385.
Paediatricians should recognise the influence of infant formula milk companies and avoid intentionally or inadvertently promoting them
doi:10.1136/adc.2005.072892
PMCID: PMC2082719  PMID: 16632663
breast feeding; conflict of interest; infant formula; nutrition
3.  Influence of sensory manipulation on postural control in Parkinson's disease. 
Postural control was assessed on a tilting platform system in 20 patients with idiopathic Parkinson's disease and 20 age-matched controls. The amount of information provided by vision and lower limb proprioception was varied during the experiment to investigate the influence of changes in sensory cues on postural control. The patient group with clinical evidence of impaired postural control (Hoehn and Yahr III) had significantly higher sway scores over all sensory conditions than either the Hoehn and Yahr II group or controls. The pattern of sway scores indicated that no obvious deficit in the quality, or processing, of sensory information was responsible for the postural instability observed in this group. The patients in both Hoehn and Yahr groups were also able to respond appropriately to potentially destabilising sensory conflict situations and significantly improved their sway scores when provided with visual feedback of body sway. The results indicate that in Parkinson's disease, the main site of dysfunction in postural control is likely to be at a central motor level.
PMCID: PMC1015374  PMID: 8270927
4.  Coordination of eye and head movements during smooth pursuit in patients with vestibular failure. 
During pursuit of smoothly moving targets with combined eye and head movements in normal subjects, accurate gaze control depends on successful interaction of the vestibular and head movement signals with the ocular pursuit mechanisms. To investigate compensation for loss of the vestibulo-ocular reflex during head-free pursuit in labyrinthine-deficient patients, pursuit performance was assessed and compared under head-fixed and head-free conditions in five patients with isolated bilateral loss of vestibular function. Target motion consisted of predictable and unpredictable pseudo-random waveforms containing the sum of three or four sinusoids. Comparison of slow-phase gaze velocity gains under head-free and head-fixed conditions revealed no significant differences during pursuit of any of the three pseudo-random waveforms. The finding of significant compensatory eye movement during active head movements in darkness in labyrinthine-deficient patients, which were comparable in character and gain to the vestibular eye movement elicited in normal subjects, probably explains the similarity of the head-fixed and head-free responses. In two additional patients with cerebellar degeneration and vestibular failure, no compensatory eye movement response was observed, implying that the cerebellum is necessary for the generation of such responses in labyrinthine-deficient patients.
PMCID: PMC1015324  PMID: 1479390
7.  Recognising failure to thrive in early childhood. 
Archives of Disease in Childhood  1990;65(11):1263-1265.
The maximum weight centile achieved by a child between 4 and 8 weeks of age was found to be a better predictor of the centile at 12 months than the birth weight centile. Children whose weight deviated two or more major centiles below this maximum weight centile for a month or more showed significant anthropometric differences during the second year of life from those who showed no such deviation. It is suggested that this leads to a logical and practical definition of failure to thrive.
PMCID: PMC1792592  PMID: 2131802
9.  Auditing community screening for undescended testes. 
Archives of Disease in Childhood  1990;65(8):888-890.
Different ways of auditing screening for undescended testes, using Hospital Activity Analysis data, hospital case notes, and community/general practice records are described. The cumulative orchidopexy rate per 1000 male births in successive birth cohorts is a simple tool for monitoring trends within a health district. Information gained from community/general practice records is valuable in highlighting problem areas in screening, such as the lack of clear diagnostic criteria and referral pathways.
PMCID: PMC1792507  PMID: 1976004
11.  Feasibility of percutaneous transluminal angioplasty for carotid artery stenosis. 
Percutaneous transluminal balloon angioplasty was attempted in seven patients with internal carotid artery stenosis, including one patient who had two procedures. All had recurrent, carotid territory, neurological symptoms considered haemodynamic in origin. Six had occlusion of the contralateral internal carotid artery. Cerebral blood flow studies confirmed diminished cerebrovascular reserve in six patients studied. In five patients (six procedures) angioplasty of the stenosed internal carotid artery was carried out successfully. With two patients technical difficulty in crossing the stenosis prevented angioplasty and in one patient with bilateral stenosis the procedure was not attempted on the second side because of the severity of the stenosis. In two patients transient aphasia developed during cannulation of the carotid artery and in another a transient monoparesis developed during the procedure. Both these haemodynamic complications recovered within ten minutes. No other complications occurred. Our experience suggests that balloon angioplasty is technically feasible in the management of stenotic carotid disease associated with haemodynamic stroke. It is a technically simple procedure requiring only a brief admission to hospital. However, its general application to patients with thromboembolic carotid-territory stroke will depend on the risk/benefit ratio compared to carotid endarterectomy or to conventional medical treatment.
Images
PMCID: PMC1014136  PMID: 2139110
12.  Idiopathic dystonia and cervical spondylotic myelopathy. 
Cervical myelopathy developed in two patients with idiopathic torsion dystonia. There were marked spondylotic changes in both patients, probably attributable to the incessant dystonic movements of the neck. Previous cervical spine surgery may have exacerbated the myelopathy in one of the patients. Cervical myelopathy complicating idiopathic dystonia must be distinguished from other causes of neurological deterioration, since it may be improved by appropriate neurosurgical treatment.
Images
PMCID: PMC1031604  PMID: 2614440
15.  Lomotil in diarrhoeal illnesses. 
Archives of Disease in Childhood  1980;55(7):577-578.
PMCID: PMC1626793  PMID: 7436512

Results 1-16 (16)