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Biomedical subjects

A H Ropper

Publications and source records attributed to A H Ropper.

At least 37 records · Page 2Linked to original sources
PubMed1998

Comparison of IgM-MGUS and IgG-MGUS polyneuropathy.

OBJECTIVE: To compare the clinical and electrodiagnostic features and response to treatment in patients with IgM-MGUS and IgG-MGUS associated polyneuropathy. MATERIAL AND METHODS: Retrospective review of 34 consecutive patients with MGUS associated neuropathy evaluated over 5 years. RESULTS: There were 19 patients with IgM-MGUS and 15 with IgG-MGUS. There were no differences in age, duration of symptoms, or distribution of motor and sensory symptoms or signs. IgM-MGUS patients had prolonged distal latencies of the median and ulnar motor potentials, greater slowing of the peroneal nerve conduction velocity and more often absent ulnar sensory potentials. Half of the patients in both groups improved following immunotherapy. CONCLUSION: IgM-MGUS patients had more severe demyelination on the nerve conduction studies, but there were no clinical features that differentiated the 2 groups. IgM and IgG-MGUS patients improved with plasma exchange and other immune therapies. Anti-MAG antibodies failed to distinguish a subgroup of patients with IgM-MGUS neuropathy.

Aged↗
PubMed1998

Treatment of chronic inflammatory demyelinating polyneuropathy with interferon-alpha 2a.

We performed an open-label, prospective, pilot study of interferon (IFN)-alpha 2a treatment for 6 weeks in 16 patients with chronic inflammatory demyelinating polyneuropathy (CIDP). All patients had failed to improve or relapsed after treatment with at least one conventional therapy (steroids, IV gamma globulin, or plasma exchange). Assessment included MRC strength score, leg sensory score, grip dynanometry, Rankin disability score, electrodiagnostic studies, and serum concentration of tumor necrosis factor-alpha. Nine (56%) improved after IFN-alpha therapy. Mean MRC score increased by 4.2 points (p = 0.01), and mean sensory score improved by 2.3 points (p = 0.02). Five patients improved five or more points on the MRC score, nine had slight improvement or were unchanged, and two worsened. We conclude that IFN-alpha may be effective in some patients with CIDP who relapse or fail to respond to conventional immunomodulating therapy.

Adult↗
PubMed1997

Axonal neuropathy associated with monoclonal gammopathy of undetermined significance.

OBJECTIVE: The neuropathy associated with monoclonal gammopathy of undetermined significance (MGUS) is typically a predominantly demyelinating process that may have additional features of axonal degeneration. Sixteen patients with MGUS and a pure or predominantly axonal neuropathy are reported and compared with 20 consecutive patients with demyelinating neuropathy and MGUS who were seen during the same period. METHODS: Retrospective review of a consecutive series of patients with neuropathy and MGUS evaluated during a five year period. RESULTS: The axonal group had mild, symmetric, slowly progressive, predominantly sensory neuropathy, usually limited to the legs. There were no differences in the age of onset or duration of symptoms at the time of presentation, initial symptoms, or the severity of weakness between the axonal and demyelinating cases. However, the axonal process was associated with less vibration and proprioceptive loss, did not include leg ataxia (present in 55% of patients with demyelinating type), less often had generalised areflexia (19% v 70%), IgM gammopathy (19% v 80%), and anti-MAG antibodies (0% v 40%), and had lower CSF protein concentrations (mean, 49 v 100 mg/dl). The illness was also generally milder with less disability (mean Rankin score 2.1 v 2.8). Fewer patients with axonal neuropathy improved with immunomodulating therapy (27% v 75%). CONCLUSION: There is an axonal neuropathy associated with MGUS that is clinically and electrophysiologically distinct from the more typical demyelinating pattern.

Adrenal Cortex Hormones↗
PubMed1997

Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy.

We report the clinical and EMG details of 67 consecutive patients with strictly defined chronic inflammatory demyelinating polyneuropathy (CIDP) during a 4-year period and compare responses to treatment in patients with idiopathic CIDP (CIDP-I) and CIDP with monoclonal gammopathy of uncertain significance (CIDP-MGUS). Patients were examined an average of 28 months after first symptoms. There were several variant presentations that still conformed to the clinical and electrophysiologic definitions of CIDP, including a pure motor syndrome (10%), sensory ataxic variant (12%), mononeuritis multiplex pattern (9%), paraparetic pattern (4%), and relapsing acute Guillain-Barré syndrome (16%). Pain was more frequent than in previous studies (42%). Conduction block was the commonest EMG abnormality (detected in at least one nerve in 73% of patients), but only 31% had a pure demyelinating neuropathy and the majority had some degree of axonal change. Patients with CIDP-MGUS had less severe weakness, greater imbalance, leg ataxia, vibration loss in the hands, and absent median and ulnar sensory potentials, but were as likely as CIDP-I patients to respond to plasma exchange. Seventeen of 44 patients (39%) with idiopathic CIDP improved for at least 2 months with an initial therapy. Although the response rates among plasma exchange, IVIG, and steroids were similar, functional improvement (Rankin score) was greatest with plasma exchange. Of 26 patients who failed to respond to an initial therapy, 9 (35%) benefited from an alternative treatment, and of the 11 who required a third modality 3 (27%) improved. Overall, 66% responded to one of the three main therapies for CIDP.

Adolescent↗
PubMed1997

Improvement following interferon-alpha 2A in chronic inflammatory demyelinating polyneuropathy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a progressive or relapsing immune-mediated neuropathy usually responsive to plasma exchange, intravenous gammaglobulin or steroids, with some patients being refractory to these conventional therapies. We report a patient with CIDP who had spontaneous improvement after an episode of sepsis, but subsequently relapsed with severe generalized weakness; he was unresponsive to the conventional treatments for CIDP but had dramatic improvement following treatment with interferon-alpha 2A. Nerve conduction studies following treatment showed improved distal compound muscle action potential amplitudes without change in the degree of conduction block. The mechanism of action of interferon-alpha is unknown, but it may modulate proinflammatory cytokines that have a role in immune-mediated demyelination. Interferon-alpha may be an effective alternative therapy in patients with CIDP who relapse or are refractory to conventional treatments.

Chronic Disease↗
PubMed1996

Prospective evaluation of MRI lumbosacral nerve root enhancement in acute Guillain-Barré syndrome.

Nerve root enhancement of the cauda equina occurs in Guillain-Barré syndrome (GBS), but the frequency, diagnostic value, and meaning of this finding is unknown. We prospectively obtained gadolinium-enhanced lumbosacral spine MRIs in 24 consecutive patients with acute GBS and blindly rated nerve root enhancement as absent, mild, or prominent. The MRIs were obtained 13 days, mean, after onset of symptoms (range 2 to 42 days). Twenty of 24 patients had cauda equina nerve root enhancement, which was mild in 6 and prominent in 14. Eighteen of 19 with "typical" GBS had enhancement, compared with 2 of 5 with a variant presentation. Sixty percent of patients with prominent enhancement had severe back or leg pain in contrast to 10% of patients with mild or no enhancement. The GBS disability grade (0 to 5 scale) was higher in patients with prominent enhancement, and significantly fewer patients with prominent nerve root enhancement could walk independently by 2 months. There was no relationship between nerve root enhancement and the timing of the MRI, CSF protein, any of several EMG abnormalities, duration of hospitalization, mean disability grade at 2 months, or the time required for patients to improve to grade 2. In two patients, the EMGs at 11 and 20 days, respectively, were normal except for slightly prolonged F-responses and neurogenic recruitment, but there were prominent nerve root enhancement and an elevated CSF protein. Enhancement of the cauda equina nerve roots with gadolinium on lumbosacral MRI is 83% sensitive of acute GBS and was present in 95% of typical cases. This finding may be useful when electrophysiologic abnormalities are equivocal. In addition, conspicuous nerve root enhancement correlates with pain, GBS disability grade, and duration of recovery.

Acute Disease↗
PubMed1995

Idiopathic distal small fiber neuropathy.

We describe the clinical details of 20 elderly patients with idiopathic small fiber neuropathy. This neuropathy is ubiquitous in practice but has not been well characterized. The clinical syndrome is relatively stereotyped and appears to be a frequent cause of burning feet in the elderly. The main features were burning, painful paresthesias and dysesthesias in the feet, lancinating pains, moderate to severe distal small fiber sensory loss, absent ankle reflexes, and minimal or no distal foot weakness. All but 2 had mild loss of vibration sense but none had significant proprioceptive loss or sensory ataxia. EMG was normal in 9 while the others had a mild sensorimotor axonal neuropathy. Sural nerve biopsy was normal in 3 and showed axonal loss in 6. Progression was slow, and although pain was a troublesome symptom, no patient became disabled. Symptoms were refractory to most symptomatic therapies but several patients improved with gammaglobulin infusions.

Adult↗
PubMed1994

Further regional variants of acute immune polyneuropathy. Bifacial weakness or sixth nerve paresis with paresthesias, lumbar polyradiculopathy, and ataxia with pharyngeal-cervical-brachial weakness.

OBJECTIVE: To describe four syndromes of acute regional weakness with clinical, spinal fluid, and electrophysiologic similarities to the acute immune polyneuropathy of Guillain-Barré syndrome. DESIGN: Case series of personally examined patients. RESULTS: Seven patients are described: four with facial diplegia and distal limb paresthesias, one with sixth nerve palsy and distal paresthesias, one with bilateral lumbar polyradiculopathy, and one with combined Fisher's syndrome and pharyngeal-cervical-brachial weakness. These self-limited illnesses, which evolved over days or weeks, involved acellular cerebrospinal fluid with raised protein concentration and electrophysiologic findings that were consistent with a demyelinating polyneuropathy. CONCLUSIONS: The first three regional variants of Guillain-Barré syndrome may cause diagnostic difficulty, particularly at the onset of illness, and the fourth links Fisher's syndrome with the typical syndrome. The consistently bilateral weakness of Guillain-Barré syndrome and its regional variants and the absence of a monoparetic or hemiparetic pattern suggest that the pathologic process occurs in the same single or contiguous groups of nerves on both sides of the sagittal plane but is not randomly distributed in the peripheral nervous system.

Abducens Nerve↗
PubMed1994

Homolateral hemiparesis as an early sign of cerebellar mass effect.

A patient with Wallenberg's syndrome and an inferior cerebellar infarction developed progressive hemiplegia ipsilateral to the infarction as cerebellar edema emerged. An MRI showed diagonal displacement of the medulla with impaction of the pyramids against the clivus; the hemiplegia resolved after posterior fossa decompression. In the pathologic specimen, the pyramids were flattened and showed small subpial ischemic lesions. Progressive ipsilateral hemiparesis in the setting of cerebellar infarction is an early sign of posterior fossa mass effect similar to the Kernohan's notch phenomenon.

Cerebellum↗
PubMed1994

Intensive care of acute Guillain-Barré syndrome.

Guillain-Barré syndrome causes, in addition to paralysis and respiratory failure, many general medical problems that have great bearing on outcome. The main features of daily care that require attention are: respiratory and urinary tract infections, gastrointestinal dysfunction, hyponatremia, pain control, and the potential for pulmonary embolism. Dysautonomic problems that arise specifically in the intensive care unit include: hyper- and hypotension, cardiac arrhythmias, and ileus. Throughout the illness, certain psychological aberrations and communication problems must be addressed. The experience reviewed herein provides a practical approach to these problems.

Acute Disease↗
PubMed1993

Accelerated neuropathy of renal failure.

OBJECTIVE: To describe a syndrome of rapidly evolving polyneuropathy in patients with severe renal failure. DESIGN: Retrospective case series of four patients. SETTING: In-hospital evaluations and personal examinations of patients. PATIENTS: Four patients with severe or end-stage renal failure who were receiving peritoneal dialysis. RESULTS: These patients had an acute or subacute syndrome characterized by generalized limb weakness over days or weeks, severe imbalance, diminished reflexes, and numbness. Spinal fluid protein levels were elevated and some demyelinating features were noted on electrophysiological testing. Improvement occurred with more frequent peritoneal dialysis in one patient and transplantation in another, but the neuropathy progressed in the other two in whom diabetes may have played a role. CONCLUSION: This partly reversible acute uremic neuropathy, which is probably caused by the metabolic disturbances of end-stage renal failure, simulates Guillain-Barré syndrome or chronic inflammatory demyelinating polyneuropathy and may be complicated by diabetic neuropathy.

Adult↗