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Biomedical subjects

Richard A Grünewald

Publications and source records attributed to Richard A Grünewald.

7 recordsLinked to original sources
PubMed2007

Myopathy associated with gluten sensitivity.

Ataxia and peripheral neuropathy are the most common neurological manifestations of gluten sensitivity. Myopathy is a less common and poorly characterized additional neurological manifestation of gluten sensitivity. We present our experience with 13 patients who presented with symptoms and signs suggestive of a myopathy and in whom investigation led to the diagnosis of gluten sensitivity. Three of these patients had a neuropathy with or without ataxia in addition to the myopathy. The mean age at onset of the myopathic symptoms was 54 years. Ten patients had neurophysiological evidence of myopathy. Inflammatory myopathy was the most common finding on neuropathological examination. One patient had basophilic rimmed vacuoles suggestive of inclusion-body myositis. Six patients received immunosuppressive treatment in addition to starting on a gluten-free diet; five improved and one remained unchanged. Among seven patients not on immunosuppressive treatment, four showed clinical improvement of the myopathy with a gluten-free diet. The improvement was also associated with reduction or normalization of serum creatine kinase level. The myopathy progressed in one patient who refused the gluten-free diet. Myopathy may be another manifestation of gluten sensitivity and is likely to have an immune-mediated pathogenesis. A gluten-free diet may be a useful therapeutic intervention.

Adolescent↗
PubMed2006

Dietary treatment of gluten neuropathy.

We studied the effect of a gluten-free diet in patients with idiopathic sensorimotor axonal neuropathy and circulating antigliadin antibodies. Consecutive patients underwent baseline neurophysiological assessment and were offered a gluten-free diet. Those who went on the diet formed the intention-to-treat group and those who did not were the control group. Repeat neurophysiological assessment and subjective evaluation of neuropathy symptoms were performed at 1 year. A total of 35 patients participated in the study, with 25 patients going on the diet and 10 not doing so. There was a significant difference in the change of sural sensory action potentials (pre-defined primary endpoint), with evidence of improvement in the intention-to-treat group and deterioration in the control group. Subjective change in neuropathy symptoms also showed significant differences, with patients in the intention-to-treat group reporting improvement and those in the control group reporting deterioration. Gluten-free diet may thus be a useful therapeutic intervention for patients with gluten neuropathy.

Aged↗
PubMed2006

A six-year follow-up MRI study of complicated early childhood convulsion.

We have previously reported a greater than 50% prevalence of structural brain abnormalities in children within 2 weeks of first complicated early childhood convulsion, including significant hippocampal asymmetry unrelated to edema. In a 6-year follow-up study, 8 of the original 17 subjects were reimaged to determine any developments in hippocampal pathology. Three subjects manifested significantly greater asymmetry, and two exhibited a modest increase in asymmetry at follow-up compared with that present at initial magnetic resonance imaging. Two of the remaining three subjects manifested no change in the degree of hippocampal asymmetry that was already evident at initial scan, whereas in the final case, apparent resolution of previously identified (slight) hippocampal asymmetry had occurred. Elevated hippocampal T2 relaxation times did not consistently accord with smaller hippocampal volumes. These results suggest that complicated early childhood convulsion may be less benign than often supposed.

Brain Damage, Chronic↗
PubMed2004

Somatic symptom progression in idiopathic Parkinson's disease.

The substantia nigra (SN) pars compacta is arranged somatotopically. Symptoms of idiopathic Parkinson's disease (IPD) are caused by a lesion in this nucleus, which spreads in a stereotyped spatio-temporal pattern during the course of the disease. We investigated the order of somatic symptom progression in a group of 30 patients with IPD to determine if progression of symptoms was consistent with ordered spread of pathology through the SN pars compacta. Thirty outpatients with IPD were interviewed retrospectively about the progression of their symptoms using a semi-structured questionnaire. All the patients experienced somatic symptom progression in one of two distinct patterns, which was dependent on the location of the initial symptom. The typical pattern of progression for patients with lower limb onset was: (i) foot, (ii) leg, (iii) arm, (iv) hand, (v) face, (vi) voice and (vii) swallowing. For patients whose first symptom was in the upper limb progression to the face and lower limbs occurred roughly simultaneously or sequentially, with the typical pattern of progression: (i) hand/arm, (ii) foot/leg and face, (iii) speech and (iv) swallowing. These patterns of disease progression may reflect two distinct patterns of neuropathology within the SN pars compacta. Although the sample size was relatively small, this is the first time this phenomenon has been described in a full-length article.

Adult↗
PubMedDate not supplied

A double-blind, placebo-controlled, crossover trial of topiramate in essential tremor.

The antiepileptic drug topiramate has been reported to improve essential tremor, but clinical trials have been small and of unsatisfactory design. We undertook a double-blind, placebo-controlled crossover study of topiramate in essential tremor, using accelerometry, spirography, and an activities of daily living questionnaire. Sixteen subjects were recruited between January and June 2002. Three withdrew consent, 13 commenced the study, and 10 finished it. Subjects were assessed at baseline and after 2, 4, and 6 weeks of treatment (placebo or topiramate doses 25, 50, and 100 mg daily). Following a washout period of 1 week, patients crossed over to the alternate arm of the study. Statistical analysis followed the method described by Altman. There was no period effect or treatment-period interaction. No outcome measure improved significantly in the active treatment period as compared with the placebo control period, although 4 of the 10 patients improved in at least 2 of the 3 outcome measures during the active treatment phase, and none during the control treatment phase. This study provided no evidence of therapeutic benefit of topiramate in essential tremor. A marginally larger study would be required formally to exclude a useful therapeutic effect. To achieve a study with a power of 80% to exclude the null hypothesis, at least 17 patients would be required using accelerometry, 19 using spirography, and 2,00 using an activities of daily living questionnaire.

Activities of Daily Living↗