PubMed Health⌕ Search

Biomedical subjects

A H Ropper

Publications and source records attributed to A H Ropper.

At least 19 recordsLinked to original sources
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↗
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↗
PubMed1993

Successful management of paraprotein-associated peripheral polyneuropathies by immunoadsorption of plasma with staphylococcal protein A.

Two patients with paraprotein-associated peripheral polyneuropathy were treated successfully using immunoadsorption of patient's plasma with staphylococcal protein A. Both had previously been treated with immunosuppressive agents or plasma exchange, and were rapidly relapsing at the time of their protein A immunoadsorption therapy. One patient was treated "on-line" with a blood cell separator, and one was treated "off-line." Both responded well to therapy with minimal toxicity. Serum levels of circulating immune complexes were elevated in one patient and remained so during and after therapy. Immunoadsorption with protein A should be investigated as a therapeutic option for patients with paraprotein-associated peripheral polyneuropathy. The therapy is relatively easy to administer, particularly "off-line," and was well tolerated by our patients. More experience, including formal clinical trials, will be required to properly define the indications for, and mechanism of response to, this therapy.

Aged↗
PubMed1993

Acute respiratory failure neuropathy: a variant of critical illness polyneuropathy.

OBJECTIVE: To describe a severe axonal polyneuropathy that follows acute respiratory failure and, in turn, causes continued ventilator dependence and paralysis after resolution of the primary illness. DESIGN: Retrospective chart review of three patients and prospective analysis of two patients. SETTING: Respiratory and neurologic ICUs of a general hospital. PATIENTS: Five critically ill patients after an episode of acute respiratory failure. Neuromuscular blocking drugs were used in four patients, intermittently in two patients, high doses of corticosteroids were briefly administered in four, four patients had multiple organ failure, three patients had sepsis, but weakness preceded these complications in two patients. INTERVENTIONS: None. MAIN RESULTS: All patients had moderate-to-severe limb weakness with reduced or absent reflexes. Sensation was relatively preserved and the spinal fluid protein concentrations were normal. Electrophysiologic studies showed a severe, acute axonal motor neuropathy in four patients and normal studies in the fifth that later demonstrated denervation. Sensory potentials were mildly or not affected. Two quadriparetic patients died at 2.5 months, one remained weak and ventilator dependent several months after onset, and two patients recovered to walk in 4 to 6 months. CONCLUSIONS: Severe axonal motor neuropathy after acute respiratory failure probably represents a variant of "critical illness polyneuropathy" that can be recognized from the temporal course of a conversion from primarily pulmonary to a pattern of neuromuscular ventilatory failure.

Action Potentials↗
PubMed1993

Syndrome of transtentorial herniation: is vertical displacement necessary?

MRI from a comatose patient with a massive acute subdural haematoma showed most of the features of transtentorial herniation described in the classic pathology literature. In addition to encroachment on the perimesencephalic cisterns, infarction in the anterior and posterior cerebral artery territories, ischaemic change in the lower diencephalon, and ventricular enlargement were visualised. Despite the clinical syndrome and these secondary changes due to compression, there was only approximately 2 mm of downward displacement of the upper brainstem compared with 13 mm horizontal displacement. Although tissue shifts adjacent to the tentorial aperture cause brainstem and vascular compression, these changes may occur with minimal downward herniation.

Aged↗
PubMed1993

Human immune globulin infusion in Guillain-Barré syndrome: worsening during and after treatment.

Because of an unexpectedly high rate of worsening, we report the results of treatment of Guillain-Barré syndrome with a standard regimen of human immune globulin (HIG) in 15 consecutive patients. Patients were treated with 1.5 g/kg HIG for 4 days beginning a mean of 7 days after the first symptoms. Eight stabilized or improved, but seven deteriorated during or within the first 5 days after treatment, and four remained ventilator-dependent for at least 2.5 months. One had a severe relapse 5 weeks after treatment. Five patients subsequently received plasma exchange and two improved. Our experience varies from previous reports that have endorsed HIG treatment for Guillain-Barré syndrome.

Action Potentials↗
PubMed1993

Seronegative, non-neoplastic acute cerebellar degeneration.

Five women are presented with a subacute pure pancerebellar syndrome of ataxia and dysarthria to emphasize that this illness may occur without anti-Purkinje-cell or anti-Yo antibodies, or associated systemic cancer. The syndrome evolved over weeks or less and stabilized in four of the patients. Three had had a preceding infection and one, a rash, but there was no inflammatory response in the spinal fluid. Over the 5-year period during which these patients appeared in an individual general neurology practice, there was only one patient with paraneoplastic cerebellar degeneration. Despite the association of this syndrome with cancer, particularly in women, clinicians will still commonly confront the idiopathic variety.

Adult↗
PubMed1992

Early Guillain-Barré syndrome without inflammation.

A patient with typical acute Guillain-Barré syndrome died 72 hours after his first symptoms occurred, and an autopsy was performed 8 hours after his death. Extensive sampling of cranial and peripheral nerves, sensory ganglia, and autonomic nerves showed only minimal inflammatory lymphocytic and macrophage infiltrates. This case, one of the earliest studied extensively, represents an extreme example of a noninflammatory mechanism that has been proposed in some cases of Gullain-Barré syndrome.

Cranial Nerve Diseases↗