Biomedical subjects
A R Berger
Publications and source records attributed to A R Berger.
2',3'-dideoxycytidine (ddC) toxic neuropathy: a study of 52 patients.
We administered the antiviral agent 2',3'-dideoxycytidine (ddC) to HIV-infected patients with either ARC or AIDS as part of the AIDS Clinical Treatment Group protocol 012 and serially evaluated them with neuropathic symptom questionnaires, neurologic examinations, nerve conduction studies, and quantitative sensory testing (QST). All patients treated with high-dose ddC (0.06 and 0.03 mg/kg every 4 hours) developed a painful, predominantly sensory peripheral neuropathy, with a mean onset of 7.7 weeks, which reached severe intensity over several days. Abnormalities of vibration QST threshold preceded clinical symptoms. Treatment with 0.01 mg/kg every 4 hours produced a similar neuropathy, although of milder severity, later onset (mean, 9.3 weeks), and slower progression. In these patients, the onset of clinical symptoms and QST abnormalities were coincident. Only two of six patients treated with 0.005 mg/kg every 4 hours developed clinical or laboratory evidence of neuropathy; in both cases it was very mild and delayed in onset (26 weeks). All patients treated with high-dose ddC reported progression of symptoms (coasting) for 2 to 3 weeks following discontinuation of therapy. This study documents a painful sensory neuropathy resulting from treatment with ddC. With high-dose treatment, only the rapidity of onset and progression differentiated it from the distal, predominantly sensory neuropathy of AIDS.
Erythromelalgia: association with hereditary sensory neuropathy and response to amitriptyline.
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Brachial plexopathy associated with interleukin-2 therapy.
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Dose response, coasting, and differential fiber vulnerability in human toxic neuropathy: a prospective study of pyridoxine neurotoxicity.
We administered either 1 or 3 g/d of pyridoxine (vitamin B6) to five healthy volunteers and repeatedly followed serum pyridoxal phosphate levels, clinical symptoms and signs, quantitative sensory thresholds (QSTs), and sural nerve electrophysiology. Pyridoxine was discontinued at the first sign of either clinical or laboratory abnormality. In all subjects, sensory symptoms and QST abnormalities occurred concurrently. Subjects receiving higher doses became symptomatic earlier than low-dose subjects. Elevation of thermal QSTs preceded or exceeded that for vibration in the three low-dose subjects; vibration and thermal QST became abnormal simultaneously in the higher-dose subjects. A reduction in the amplitude of the sural sensory potential lagged behind QST changes in two of three subjects. Symptoms continued to progress ("coasting") for 2 to 3 weeks despite stopping pyridoxine administration and the return of serum pyridoxal phosphate levels to normal. This study suggests that (1) there is a clear dose-percent relationship for pyridoxine-induced neuropathy, (2) QST is a sensitive measurement for detecting early peripheral neuropathy; QST abnormalities may precede changes in nerve conduction studies, (3) coasting appears unrelated to persistently elevated blood levels of the toxin, and (4) a dose-dependent vulnerability may exist among nerve fibers of different caliber when exposed to an axonal toxin, such as pyridoxine.
Endogenous Pseudallescheria boydii endophthalmitis. Clinicopathologic findings in two cases.
Two cases of endogenous Pseudallescheria boydii endophthalmitis are presented. One patient had severe pulmonary fibrosis but no history of ocular trauma and no clinical or laboratory evidence of immunocompromise. Despite therapy with repeated intravitreal miconazole nitrate injections and systemic fluconazole, enucleation of the globe was required, and the patient eventually died with disseminated pseudallescheriasis. The other patient was an immunosuppressed cardiac transplant recipient who also received systemic fluconazole therapy. The infected eye eventually required evisceration, but there was no evidence of disseminated pseudallescheriasis before his death of unrelated causes.
Central serous choroidopathy in patients over 50 years of age.
Reviewing all records of patients with the diagnosis of central serous choroidopathy (CSC) at Retina Consultants, Ltd since 1975, we identified 47 patients with CSC who were older than 50 years at initial presentation. These patients were divided into three groups, based on type of management: observation; light "CSC-type" photocoagulation; and heavy "subretinal-neovascular-membrane (SRNVM)-type" photocoagulation. Although most of these patients retained excellent central visual acuity, overall final visual acuities were poorer than those in other reported series. Heavier photocoagulation, as compared with lighter treatment, was associated with a decreased incidence of posttreatment SRNVM formation and appeared to have no adverse effect on final visual outcome. Patients with multiple irregular leaks on fluorescein angiography had a greater tendency to develop SRNVMs, with or without treatment. Ophthalmologists diagnosing CSC in patients over age 50 should be aware that there is a greater risk of SRNVM developing in these patients.
Bilateral subretinal neovascularization after focal argon laser photocoagulation for diabetic macular edema.
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Electrophysiologic evaluation of spinal cord motor conduction.
Spinal cord motor conduction was determined by stimulating with a monopolar needle at the C5 cord level and recording evoked motor potentials from the ipsilateral tibialis anterior muscle. Minimal F wave and direct motor latencies from the peroneal nerve were used to calculate peripheral conduction time. Mean velocity index (defined as the distance from C5 to L4 divided by central conduction time) of 15 normal subjects was 64.9 m/sec (SD 7.5). In 5 patients with multiple sclerosis the mean velocity index was 40.6 m/sec (SD 6.5), whereas in 3 patients with cervical myelopathies, due to extradural compression, the mean velocity index was 32.8 m/sec. A repeat study in one of the latter patients, 4 days after removal of a C5 disc, documented a marked improvement in cord conduction. In a patient with a thoracic and cervical syrinx, cervical cord stimulation on the clinically affected side failed to evoke a motor potential, whereas the unaffected side was normal. This method provides a simple and effective way to evaluate spinal cord motor conduction using routine electrodiagnostic equipment.
Injury to the tibial and peroneal nerves due to hemorrhage in the popliteal fossa. Two case reports.
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Stretch-induced spinal accessory nerve palsy.
Left spinal accessory nerve palsy occurred in a young man when he quickly turned his head to the right while his shoulders were pulled down by heavy hand-held objects. Electrophysiologic studies demonstrated partial axonotmesis of the spinal accessory nerve branches innervating the sternocleidomastoid and upper and middle trapezius and complete axonotmesis of spinal accessory branches to the lower trapezius. There was a separate, although functionally minor, cervical plexus innervation of the lower trapezius.
Reversible proximal conduction block underlies rapid recovery in Guillain-Barré syndrome.
Three patients with Guillain-Barré syndrome underwent electrophysiological examination prior to and during a period of rapid clinical recovery. In each case, improved strength of the abductor digiti minimi coincided with electrophysiologic evidence of a marked reversal of proximal conduction block. In contrast, the degree of distal conduction block remained relatively unchanged after stimulation at the wrist, elbow, axilla, and Erb's point. These findings indicate that rapid motor recovery, early in the course of Guillain-Barré syndrome, can result from reversal of proximal conduction block and explains the often noted dissociation between clinical improvement and conventional distal nerve conduction studies.
Early seizures following intracerebral hemorrhage: implications for therapy.
Seizures occurred in 19 of 112 patients (17%) with nontraumatic, supratentorial intracerebral hemorrhage (ICH). All seizures occurred at ICH onset; patients without seizures at hemorrhage onset remained seizure-free until the last recorded follow-up. Seizures were significantly associated with extension of blood into the cerebral cortex. We found no association between seizures and hemorrhage size or the presence of subarachnoid or intraventricular blood. These data suggest that (1) seizures, in ICH, occur at hemorrhage onset, (2) patients without seizures at hemorrhage onset are at very low risk for subsequent seizures during their hospitalization, (3) hemorrhage involving the cerebral cortex, regardless of site of origin, predisposes to seizures, and (4) the prophylactic use of anticonvulsants in the acute management of these patients appears unwarranted, especially in patients without cortical extension.
Lobar vs thalamic and basal ganglion hemorrhage: clinical and radiographic features.
One hundred and twelve patients with spontaneous supratentorial intracerebral hemorrhages were reviewed to identify features which distinguish lobar intracerebral hemorrhage (LH; n = 42) from thalamic or basal ganglionic hemorrhage (TGH; n = 70). Chronic hypertension occurred more commonly in TGH (TGH 67%; LH 48%) while bleeding diathesis was more common in LH (LH 19%; TGH 6%). Clinical presentations were extremely variable and not associated with the type of hemorrhage. Bleeding into the ventricles and hydrocephalus occurred more often with TGH. At last follow-up, there were minimal differences between LH and TGH in overall mortality and functional outcome of the survivors. Alertness on admission was associated with a good outcome regardless of the type of hemorrhage, while a low Glasgow Coma Scale score, coma, ataxic respiration, abnormal pupil reactions, acute hypertension, large hemorrhage size and intraventricular blood were associated with a poor outcome. These data confirm etiological distinctions between LH and TGH, but fail to confirm previously reported differences in clinical presentation and outcome.
Intracerebral haemorrhage: a model for the prediction of outcome.
The hospital charts and CT scans of 112 patients with spontaneous supratentorial haemorrhage were retrospectively reviewed to evaluate factors important to prognosis. A low Glasgow Coma Scale score, coma, ataxic respiration, abnormal pupils, acute hypertension, large haemorrhage size and intraventricular extension of blood were associated with a poor outcome. Multivariate analysis using the technique of logistic regression identified three variables, the Glasgow Coma Scale score, haemorrhage size and intraventricular extension of blood, which were most predictive of outcome. A model was developed from this analysis which accurately estimates the probability of good outcome and the risk of poor outcome based on data available on presentation. With additional validation, this model may be useful in choosing patients for surgical evacuation of haematomas.
Cervical root stimulation in the diagnosis of radiculopathy.
Cervical root stimulation (CRS) was compared with conventional EMG, nerve conduction, and late response studies in 34 patients with possible cervical radiculopathy. Cervical roots were stimulated by monopolar needles inserted into paraspinal muscles, recording compound muscle action potentials in biceps, triceps, and abductor digiti minimi muscles. In 18 patients with clinical evidence of radiculopathy, EMG was abnormal in 11 (61%), but CRS was abnormal in all 18. Of 16 patients with symptoms but no signs of radiculopathy, EMG was abnormal in 5 (31%) and CRS was abnormal in 9 (56%).
Lumbrical sparing in carpal tunnel syndrome: anatomic, physiologic, and diagnostic implications.
Motor axons supplying lumbrical muscles are less severely affected than axons supplying thenar muscles in the carpal tunnel syndrome; sometimes lumbrical motor fibers are less affected than digit 2 sensory fibers. This pattern is consistent with compression of both the anterior and posterior aspects of the median nerve in the carpal tunnel because nerve fibers responsible for thenar, lumbrical, and digit 2 functions lie in an anterior-posterior gradient within the distal median nerve. Recognition of lumbrical sparing supports the electrodiagnosis of carpal tunnel syndrome when the distal latency to thenar muscles or the palm-to-wrist mixed median nerve conduction velocity is normal.
Rapidly progressive glomerulonephritis in a patient with rheumatoid arthritis during treatment with high-dosage D-penicillamine.
A patient with advanced rheumatoid arthritis and severe clinical manifestations of rheumatoid vasculitis died of acute renal failure after 30 months of treatment with high-dosage D-penicillamine. She had had no signs of adverse drug reactions until the terminal illness. Although streptococcal pharyngitis was diagnosed late in her disease, penicillamine-induced immune complex glomerular damage is considered more likely than poststreptococcal glomerulonephritis, because her microscopic hematuria preceded diagnosis of pharyngitis. Postmortem examination disclosed findings suggestive of rapidly progressive glomerulonephritis of immune complex pathogenesis. The short period of microscopic hematuria and the rapidity of development of renal failure before death emphasize the need for frequent monitoring of renal function and prompt discontinuation of D-penicillamine treatment upon detection of otherwise unexplained hematuria. There is urgent need for early immunological evaluation, renal biopsy, and vigorous therapeutic measures.