Richard J. Lederman

West Nile virus infection A new acute paralytic illness

Objective: To determine the clinical, laboratory, electrodiagnostic, radiologic, and pathologic characteristics that define the spectrum of CNS disease caused by West Nile virus (WNV) infection. Methods: The records of all patients hospitalized at the Cleveland Clinic from August 2002 to September 2002 with WNV infection were reviewed. Results: Of 23 cases, the median age was 74 years old, and 74% were men. Symptoms included fever (100%), altered mental status (74%), gastrointestinal complaints (43%), back pain (35%), and rash (26%). In half, meningitis or encephalitis overlapped with flaccid weakness that progressed over 3 to 8 days, with a tendency to be proximal and asymmetric. Laboratory abnormalities included hyponatremia (30%) and initial CSF neutrophilic pleocytosis. Electrodiagnostic studies in two patients showed reduced motor amplitudes with normal conduction velocities and active denervation. In two other patients, reduced sensory amplitudes were also seen. MRI changes included cauda equina enhancement and parenchymal spinal cord signal abnormalities and parenchymal or leptomeningeal signal changes in the brain. Autopsy in three cases showed chronic perivascular inflammation in the brain and inflammatory changes with anterior horn cell loss in the spinal cord. Conclusion: An overlapping spectrum of meningitis, encephalitis, and myeloradiculitis occurs in CNS WNV infection. Fever, rash, abdominal and back pain, preceding a proximal, asymmetric flaccid weakness, with CSF pleocytosis help distinguish the motor syndrome from Guillain–Barré syndrome. Pathologic changes in the CNS resembled poliomyelitis.

Neuromuscular and musculoskeletal problems in instrumental musicians

Over the past 20 years, there has been increasing interest in the medical problems of performing artists. In this review, the major playing‐related disorders seen in instrumental musicians are discussed. Among the 1353 instrumentalists personally evaluated, the major diagnoses included musculoskeletal disorders in 64%, peripheral nerve problems in 20%, and focal dystonia in 8%. Of these instrumentalists, 60% were women, although men were the majority in the group with focal dystonia. The average age at the time of evaluation was 37 years for men and 30 years for women. Among musculoskeletal disorders, regional muscle pain syndromes, particularly of the upper limb, upper trunk, and neck, were most common. Specific entities such as tendinitis and ligament sprain were less common. Frequent peripheral nerve disorders included thoracic outlet syndrome, ulnar neuropathy at the elbow, and carpal tunnel syndrome. Each instrument group showed a characteristic distribution of symptoms and signs that appeared to be directly related to the static and dynamic stresses inherent in the playing of the instrument. Electrodiagnostic studies are an important part of the evaluation of these disorders, particularly nerve entrapment syndromes. With carefully designed treatment, the majority of instrumental musicians can return to full and pain‐free playing. Nerve entrapment syndromes have the highest treatment success rate, followed by musculoskeletal pain syndromes. Despite some recent innovative approaches, focal dystonia remains largely resistant to therapy. Muscle Nerve 27: 549–561, 2003

Neurologic complications of liver transplantation.

Nineteen adult patients underwent 21 orthotopic liver transplants at the Cleveland Clinic between November 1984, and August 1986. Eight of 19 (42%) patients developed seizures. One patient suffered a single seizure, and seven patients had multiple, generalized seizures. Two of these seven patients became comatose after several days of seizure activity. Over several weeks, both of these patients regained consciousness—however, they exhibited a cerebellar-type syndrome, manifested as severe ataxia, weakness, and dysarthria. Both patients have improved, but remain neurologically impaired. Laboratory evaluation included serum electrolytes, magnesium, osmolality, and cyclosporine levels. Neurologic testing consisted of cerebrospinal fluid (CSF) analysis, computed tomographic (CT) scanning, and electroencephalography (EEG). Although the CSF protein was mildly elevated in two patients, all cultures remained sterile. None of the CT scans demonstrated any abnormalities. In five patients, the EEG showed generalized slowing consistent with diffuse encephalopathy. Other factors associated with seizures in transplant patients were analyzed, including fluid retention, hypertension, high-dose steroids, hypomagnesemia, graft dysfunction, and demyelinization. Many of our patients had the first three of these factors, since all but one developed their seizures within the first ten postoperative days. Only one patient had mild hypomagnesemia. Trough cyclosporine levels (whole blood, HPLC) were not in the toxic range (>500 ng/mL). The serum osmolality was elevated in all four patients in whom it was measured, ranging from 309 to 341 mOsm/kg. Only three patients exhibited graft dysfunction—two moderate and one severe. The cause of neurologic toxicity following transplantation is unclear. Although many factors have been implicated, no common denominator has emerged. Several reports have linked cyclosporine with seizures and other neurologic problems, such as the cerebellar-type syndrome exhibited in two of our patients. Future studies should include magnetic resonance (MR) imaging of the head and measuring osmolality and cyclosporine levels in the blood and CSF.

Botulinum toxin injections in the treatment of musician's dystonia

The authors present the results of 84 musicians with focal task-specific dystonia treated with EMG-guided botulinum toxin injections. Treatment outcome was assessed by subjective estimation of playing before and after treatment and self-rating of treatment response. Fifty-eight (69%) of the musicians experienced improvement from the injections and 30 of 84 musicians (36%) reported long-term benefit in their performance ability.

Ischemic optic neuropathy A complication of cardiopulmonary bypass surgery

Ischemic optic neuropathy followed cardiopulmonary bypass surgery in the postoperative period in 7 of 7685 consecutive procedures. The visual loss was unilateral in four patients and bilateral in three and there was little improvement. This ischemic infarction of the optic nerve disk was attributed to hypotension, hypothermia, and activation of certain complement factors by the bypass procedure.

Brachial plexopathy: recurrent cancer or radiation

We reviewed clinical and electrodiagnostic features of 16 patients with neoplastic brachial plexopathy (NBP) and 17 patients with radiation-induced plexopathy (RBP). The groups were similar in symptom-free interval after cancer diagnosis and location of the plexus lesions. NBP patients had pain and Horners syndrome; RBP patients had paresthesias, but rarely Horners. NBP patients presented earlier after symptom onset and had a shorter course. RBP patients more frequently had abnormal sensory and normal motor nerve conduction studies and characteristically had fasciculations or myokymia on EMG.

Ocular and orbital toxicity following intracarotid injection of BCNU (carmustine) and cisplatinum for malignant gliomas

Eleven patients treated with intracarotid BCNU, cisplatinum, or BCNU and cisplatinum in combination for recurrent malignant gliomas were followed with serial ophthalmologic examinations for 2 to 11 months. Eight patients developed significant visual loss ipsilateral to the side of infusion. Secondary glaucoma and internal ophthalmoplegia were new complications observed after BCNU treatment. An unusual pigmentary retinopathy, previously unreported, was seen in patients treated with cisplatinum. One patient also developed a cavernous sinus syndrome after the intracarotid administration of cisplatinum.

Long‐term outcome of focal dystonia in string instrumentalists

This study describes the clinical characteristics and long‐term outcome in string instrumentalists with focal task‐specific dystonia. We present the results of a follow‐up telephone survey of 21 violin and viola players with focal dystonia. Eighteen musicians responded to the questionnaire. Information on long‐term outcome was available on average 13.8 years after onset of symptoms. Main complaints were playing‐related loss of control and involuntary movements affecting the fingering hand in 16 and the bow arm in 5 patients. In 18 patients (86%), signs of abnormal posture could be detected by watching them play their instrument. Treatment attempts included nerve decompression, physical therapy, retraining, and anticholinergic medication. In selected patients, botulinum toxin injections or splint devices were offered. Only 38% of the performing artists were able to maintain their professional careers, among them none with bow arm dystonia. Focal dystonia may affect the fingering hand or bow arm in violin and viola instrumentalists. Treatment benefit is limited and in more than half of the patients, dystonia leads to the end of their musical career.

Mechanism and Frequency of Brachial Plexus Injury in Open-Heart Surgery: A Prospective Analysis

A computer-assisted prospective analysis of 531 patients undergoing open-heart operations revealed that 26 patients (5%) sustained brachial plexus injury. In 22 of the 26 patients (85%), the lesion involved the lower trunk or C8-T1 nerve roots. Electromyograms confirmed the clinical impression in 13 patients. In 19 of the 26 patients (73%), the side on which the plexus lesion was found correlated with the side of internal jugular vein cannulation. Because of the anatomical proximity of the lower trunk to the internal jugular vein and the preponderance of lower trunk lesions, we postulate that traumatic cannulation may be a major mechanism of plexus injury. Thus, the resulting syndrome of pain, dysesthesias, and hand weakness may sometimes be preventable.

Aphasic status epilepticus A case report

Complex partial status epilepticus with aphasia as the sole manifestation has been reported only twice before. We studied a 60-year-old diabetic who was aphasic for 12 days. The diagnosis of aphasic status was supported by electroencephalographic seizure patterns in the left hemisphere.