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G I Wolfe

Publications and source records attributed to G I Wolfe.

At least 19 recordsLinked to original sources
PubMed2005

Multifocal motor sensory demyelinating neuropathy: inflammatory demyelinating polyradiculoneuropathy.

The authors present two cases that provide the first autopsy findings in multifocal acquired demyelinating sensory and motor neuropathy (MADSAMN). Both cases documented multifocal but asymmetric demyelinating neuropathy with rare axonal degeneration. One case clearly documented an inflammatory polyradiculoplexoneuropathy, confirming the inflammatory nature of this neuropathy. This study showed that MADSAMN is an inflammatory demyelinating polyradiculoneuropathy that shares histologic features observed in chronic inflammatory demyelinating polyradiculoneuropathy and multifocal motor neuropathy (MMN), suggesting a similar immunopathogenesis for these entities.

Anti-Inflammatory Agents↗
PubMed2002

Axonal multifocal motor neuropathy without conduction block or other features of demyelination.

BACKGROUND: Conduction block is considered an essential finding for the distinction between motor neuropathies and lower motor neuron disorders. Only a small number of reports describe patients with multifocal motor neuropathies who lack overt conduction block, although in these cases other features of demyelination still suggest the presence of a demyelinating disorder. In contrast, a purely axonal multifocal motor neuropathy has not been described. METHODS: This report describes nine patients with slowly or nonprogressive multifocal motor neuropathies who had purely axonal electrodiagnostic features. RESULTS: GM1 antibodies titers were normal in all nine cases. Six patients were treated with either prednisone or IV immunoglobulin and three showed convincing improvement. CONCLUSIONS: These findings suggest an immune-mediated motor neuropathy with axonal electrophysiologic features that appears to be distinct from both multifocal motor neuropathy and established motor neuron disorders.

Adolescent↗
PubMed2000

McArdle's disease presenting with asymmetric, late-onset arm weakness.

McArdle's disease or myophosphorylase deficiency is one of the most common muscle glycogenoses and typically presents in childhood or adolescence with exercise intolerance, myalgia, myoglobinuria, and cramps in exercising muscle. We describe an elderly man who developed asymmetric proximal arm weakness at age 73. He had no history of exercise-induced cramps, myalgias, or myoglobinuria. Creatine kinase levels were elevated, serum lactate did not rise on ischemic exercise testing, and muscle biopsy showed a vacuolar myopathy with absent myophosphorylase activity. This unusual case demonstrates that McArdle's disease may present with fixed, asymmetric proximal weakness at an advanced age and should be considered in this clinical setting, especially when a history of poor exercise tolerance can be elicited.

Aged↗
PubMed2000

Perineurial cell hyperplasia in early-onset polyneuropathy with multiple cranial neuropathies.

We describe a patient who as a teenager developed a sensory-motor polyneuropathy with optic atrophy. Over the next three decades, multiple cranial neuropathies appeared. Striking areas of subperineurial cellular proliferation were observed on sural nerve biopsy. The ultrastructural and immunohistochemical characteristics of these aggregates were those of perineurial cell hyperplasia. To our knowledge, this is the second full report associating perineurial cell hyperplasia with a sensory-motor polyneuropathy and the first in the English literature.

Adolescent↗
PubMed2000

Abnormal sudomotor function in the hypomelanotic macules of tuberous sclerosis complex.

To investigate the integrity of sympathetic innervation in the hypomelanotic macules of tuberous sclerosis complex, we studied sudomotor function in nine patients with tuberous sclerosis complex. Postganglionic sudomotor function was assessed using the Silastic imprint test in nine patients with tuberous sclerosis complex who have at least one hypomelanotic macule greater than 2 cm in diameter. Sweating was induced by iontophoresis with 0.5% pilocarpine nitrate and sweat droplets were counted under a microscope using a 1 x 1 cm grid. Silastic imprint testing of an analogous skin area contralateral to the hypomelanotic macule was measured as a control. Sweat volume quantitation using sweat collectors was performed in five of the subjects. The sweat volume collected from the hypomelanotic macule was reduced compared to the control skin in four of the five subjects. Sweat droplet counts from the hypomelanotic macule were significantly reduced in only one of nine subjects. These data suggest that, although there is no difference in the number of functioning sweat glands in most hypomelanotic macules, the sweat glands produce less sweat (ie, decreased sweat volume) than in normal skin. We hypothesize that focal abnormalities of sympathetic innervation might be responsible for the hypomelanotic macules of tuberous sclerosis complex.

Adolescent↗
PubMed1999

Brachial amyotrophic diplegia: a slowly progressive motor neuron disorder.

OBJECTIVE: To describe a sporadic motor neuron disorder that remains largely restricted to the upper limbs over time. BACKGROUND: Progressive amyotrophy that is isolated to the upper limbs in an adult often suggests ALS. The fact that weakness can remain largely confined to the arms for long periods of time in individuals presenting with this phenotype has not been emphasized. METHODS: We reviewed the records of patients who had a neurogenic "man-in-the-barrel" phenotype documented by examination at least 18 months after onset. These patients had severe bilateral upper-extremity neurogenic atrophy that spared lower-extremity, respiratory, and bulbar musculature. RESULTS: Nine of 10 patients meeting these criteria had a purely lower motor neuron disorder. During follow-up periods ranging from 3 to 11 years from onset, only three patients developed lower-extremity weakness, and none developed respiratory or bulbar dysfunction or lost the ability to ambulate. CONCLUSION: Patients presenting with severe weakness that is fully isolated to the upper limbs, without pyramidal signs, may have a relatively stable variant of motor neuron disease.

Adult↗
PubMed1999

Myasthenia gravis activities of daily living profile.

The authors have developed an MG activities of daily living (ADL) profile (MG-ADL)-a simple eight-question survey of MG symptoms. In 254 consecutive encounters with established MG patients, the authors compared scores from the MG-ADL to the quantitative MG score (QMG)-a standardized, reliable scale used in clinical trials. The mean MG-ADL score was 4.89+/-3.63. The mean QMG score was 10.80+/-5.70. Pearson's correlation coefficient was 0.583 (p < 0.001). The MG-ADL is an easy-to-administer survey of MG that correlates well with the QMG and can serve as a secondary efficacy measurement in clinical trials.

Activities of Daily Living↗
PubMed1999

The exercise test in Andersen syndrome.

BACKGROUND: Andersen syndrome is a rare form of periodic paralysis (PP) associated with dysmorphic features and potentially fatal cardiac dysrhythmias. To date, no electrodiagnostic abnormalities have been reported that can be used to confirm the presence of PP in this condition. OBJECTIVES: To determine if the exercise test could be used to confirm the diagnosis of PP in Andersen syndrome. To evaluate the exercise test as a means to assess neuromuscular status during treatment. METHODS: We performed the exercise test on 2 patients with Andersen syndrome. In 1 patient, we used a modified version of the test to document responsiveness to treatment with tocainide. RESULTS: Studies in both patients demonstrated a progressive decline in the compound muscle action potential amplitude after exercise that was characteristic of the phenomenon seen in other forms of PP. In 1 patient, improvement in interattack strength and a reduction in the number of attacks of weakness correlated with improvement in the test results. CONCLUSIONS: Our cases demonstrate that the exercise test can confirm the diagnosis of PP in Andersen syndrome. A modified version of exercise testing may also be considered as an objective method for documenting treatment responses in PP.

Abnormalities, Multiple↗
PubMed1999

Chronic cryptogenic sensory polyneuropathy: clinical and laboratory characteristics.

BACKGROUND: Chronic sensory-predominant polyneuropathy (PN) is a common clinical problem confronting neurologists. Even with modern diagnostic approaches, many of these PNs remain unclassified. OBJECTIVE: To better define the clinical and laboratory characteristics of a large group of patients with cryptogenic sensory polyneuropathy (CSPN) evaluated in 2 university-based neuromuscular clinics. DESIGN: Medical record review of patients evaluated for PN during a 2-year period. We defined CSPN on the basis of pain, numbness, and tingling in the distal extremities without symptoms of weakness. Sensory symptoms and signs had to evolve for at least 3 months in a roughly symmetrical pattern. Identifiable causes of PN were excluded by history, physical examination findings, and results of laboratory studies. We analyzed clinical and laboratory data from patients with CSPN and compared findings in patients with and without pain. RESULTS: Of 402 patients with PN, 93 (23.1%) had CSPN and stable to slowly progressive PN syndrome. These patients presented with a mean age of 63.2 years and a mean duration of symptoms of 62.9 months. Symptoms almost always started in the feet and included distal numbness or tingling in 86% of patients and pain in 72% of patients. Despite the absence of motor symptoms at presentation, results of motor nerve conduction studies were abnormal in 60% of patients, and electromyographic evidence of denervation was observed in 70% of patients. Results of laboratory studies were consistent with axonal degeneration. Patients with and without pain were similar regarding physical findings and laboratory test abnormalities. Only a few patients (<5%) had no evidence of large-fiber dysfunction on physical examination or electrophysiologic studies. All 66 patients who had follow-up examinations (mean, 12.5 months) remained ambulatory. CONCLUSIONS: Cryptogenic sensory polyneuropathy is a common, slowly progressive neuropathy that begins in late adulthood and causes limited motor impairment. Isolated small-fiber involvement is uncommon in this group of patients. Management should focus on rational pharmacotherapy of neuropathic pain combined with reassurance of CSPN's benign clinical course.

Adult↗
PubMed1999

Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome.

We report 11 patients with multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, defined clinically by a multifocal pattern of motor and sensory loss, with nerve conduction studies showing conduction block and other features of demyelination. The clinical, laboratory, and histological features of these patients were contrasted with those of 16 patients with multifocal motor neuropathy (MMN). Eighty-two percent of MADSAM neuropathy patients had elevated protein concentrations in the cerebrospinal fluid, compared with 9% of the MMN patients (P < 0.001). No MADSAM neuropathy patient had elevated anti-GM1 antibody titers, compared with 56% of MMN patients (P < 0.01). In contrast to the subtle abnormalities described for MMN, MADSAM neuropathy patients had prominent demyelination on sensory nerve biopsies. Response to intravenous immunoglobulin treatment was similar in both groups (P = 1.0). Multifocal motor neuropathy patients typically do not respond to prednisone, but 3 of 6 MADSAM neuropathy patients improved with prednisone. MADSAM neuropathy more closely resembles chronic inflammatory demyelinating polyneuropathy and probably represents an asymmetrical variant. Given their different clinical patterns and responses to treatment, it is important to distinguish between MADSAM neuropathy and MMN.

Adult↗
PubMed1999

Distal myasthenia gravis.

Myasthenia gravis (MG) characteristically involves ocular, bulbar, and proximal extremity muscles. Distal extremity muscles are typically spared or less prominently involved. The authors performed a retrospective chart review of MG patients treated at two university-based neuromuscular clinics. From a total population of 236, nine patients (3%) had distal extremity weakness exceeding proximal weakness by at least one Medical Research Council grade during their illness. Hand muscles, particularly finger extensors, were involved more frequently than were distal leg and foot muscles.

Adolescent↗
PubMed1998

Cryptogenic sensory and sensorimotor polyneuropathies.

Chronic sensory or sensorimotor polyneuropathy is a common clinical problem referred to neurologists. Even with modern diagnostic approaches, up to one-third of them will remain unclassified. Recent studies have provided insight into their clinical spectrum and course. The age of onset tends to be in the sixth to seventh decade of life. Symptoms progress slowly, and most patients present with paresthesias or pain. Cryptogenic polyneuropathies are almost exclusively axonal on clinical, electrophysiologic, and histologic grounds. Overall, the prognosis is favorable, and only a small minority of patients develop significant motor disability or physical incapacitation. Progression to a nonambulatory state essentially never occurs. Successful management should focus on rational pharmacotherapy for painful paresthesias combined with patient education and reassurance.

Education, Medical, Continuing↗
PubMed1998

Acetylcholine receptor antibodies in the Lambert-Eaton myasthenic syndrome.

Two patients were initially diagnosed with myasthenia gravis with elevated titers of acetylcholine receptor antibodies. Features including weakness that normalized with sustained contraction, areflexia, autonomic symptoms, and low-amplitude baseline compound muscle action potentials with abnormal increments following brief exercise and high-frequency repetitive stimulation, however, suggested that these patients had Lambert-Eaton myasthenic syndrome. One patient had antibodies directed against presynaptic calcium channels, confirming the diagnosis. The second patient was seronegative for these antibodies but had elevated titers of antistriated muscle antibodies. This shows that serologic studies can conflict with clinical and electrodiagnostic findings in patients with Lambert-Eaton syndrome. These cases also point out that acetylcholine receptor antibodies are not necessarily diagnostic of myasthenia gravis in patients with Lambert-Eaton syndrome. Instead, these antibodies could represent a nonpathogenic epiphenomenon.

Action Potentials↗
PubMed1998

Primary hyperparathyroidism and ALS: is there a relation?

BACKGROUND: An association between primary hyperparathyroidism (PHP) and amyotrophic lateral sclerosis (ALS) has been noted; however, a causal relation between these disorders has not been confirmed. PATIENTS/METHODS: We report five patients (three men, two women) meeting El Escorial criteria for ALS who also had PHP. In three patients, the diagnosis of PHP was made during the laboratory evaluation for motor neuron disease, and in one patient, the diagnosis of PHP preceded the onset of weakness by 5 months and in another by 2 years. Serum calcium levels in all five patients were elevated, ranging from 11.2 to 12.8 mg/dL (normal, <10.4 mg/dL), as were levels of parathyroid hormone (PTH). RESULTS: All five patients underwent parathyroid adenoma resection with subsequent normalization of serum calcium and PTH levels. Each patient had progressive weakness resulting in death 1 to 3 years following parathyroidectomy. CONCLUSION: Resection of parathyroid adenomas in patients meeting El Escorial criteria for ALS did not alter the course of ALS. PHP and ALS appear to be coexisting but unrelated disorders.

Adenoma↗