Frederick Delius: controversies regarding his neurological disorder and its impact on his compositional output.

Frederick Delius was born in Yorkshire, England, on June 29, 1862, the son of German immigrants. He showed early musical talent but his father, a wealthy wool merchant, insisted he pursue a business career. After several failures, including an assignment managing an orange grove near Jacksonville, Florida, his father agreed to support his musical studies in Leipzig, assuming he would then become self-sufficient. Delius spent most of his adult life in France, living with and ultimately marrying Jelka Rosen, a painter of independent means, composing prolifically, and being sexually promiscuous both before and after starting life with Jelka. He contracted syphilis in 1895 and manifestations of neurosyphilis appeared in 1910. Despite periods of relative good health over the ensuing 10 years, he became progressively disabled from 1920 on, ultimately quadriparetic and blind but with preserved cognition and musical inspiration. In his final years, he completed several compositions aided by a young British musician, Eric Fenby, who served as his amanuensis and caregiver, along with Jelka, to his death on June 10, 1934.

Alice G. Brandfonbrener, MD-A personal remembrance.

Alice Brandfonbrener died peacefully at home on May 31, 2014, after a protracted illness. For all of us involved in performing arts medicine, she was the inspiration and guiding spirit. She will be missed. [Alice Brandfonbrener was one of the founding members of the field of performing arts medicine, perhaps the most critical founding member. In 1983, she organized the first "Conference on the Medical Problems of Musicians" in Aspen, Colorado, and co-directed these for the next 20 years. These symposia led to the formation of the Performing Arts Medicine Association, of which Dr. Brandfonbrener was the first president, and in 1986, establishment of the first journal in the field--this journal, Medical Problems of Performing Artists--for which she served as Editor for 20 years. At her performing arts clinic in Chicago, she saw and treated thousands of musicians, from young students to world-renowned professionals, and mentored many students and professionals who would extend research and practice in performing arts medicine.].

Presence of voltage-gated potassium channel complex antibody in a case of genetic prion disease.

Voltage-gated potassium channel (VGKC) complex antibody-mediated encephalitis is a recently recognised entity which has been reported to mimic the clinical presentation of Creutzfeldt-Jakob disease (CJD). Testing for the presence of this neuronal surface autoantibody in patients presenting with subacute encephalopathy is therefore crucial as it may both revoke the bleak diagnosis of prion disease and allow institution of potentially life-saving immunotherapy. Tempering this optimistic view is the rare instance when a positive VGKC complex antibody titre occurs in a definite case of prion disease. We present a pathologically and genetically confirmed case of CJD with elevated serum VGKC complex antibody titres. This case highlights the importance of interpreting the result of a positive VGKC complex antibody with caution and in the context of the overall clinical manifestation.

Sural mononeuropathy: a report of 36 cases.

INTRODUCTION: Isolated sural mononeuropathy is rare and frequently constitutes a diagnostic challenge. METHODS: This investigation was a retrospective study of sural neuropathy at a single electrodiagnostic center. RESULTS: Our study included 36 patients with sural neuropathy, the largest sample so far reported. Non-surgical, non-traumatic etiologies account for 50% of the cases, including 7 patients with inflammatory or vasculitic conditions. Routine sural conduction study was positive in 34 of 36 patients, whereas a distal recording method was used to verify the diagnosis of sural mononeuropathy in 2 patients. Most (58%) patients did not require specific treatment, but persistent sensory symptoms were seen in a minority of cases. Sural nerve biopsy in 1 patient helped diagnostic and treatment planning. CONCLUSIONS: Sural mononeuropathy has distinct etiologic, clinical, and electrophysiological features. Recognition can be beneficial in treating patients with sensory symptoms involving the distal lower extremity.

Primary bowing tremor: a task-specific movement disorder of string instrumentalists.

Fear of a tremulous or unsteady bow is widespread among string instrumentalists. Faulty technique and performance anxiety have generally been blamed. The cases of 4 high-level violinists and 1 violist, 3 women and 2 men, with uncontrollable bow tremor are presented. Age at onset was from 16 to 75 years, and symptom duration 8 months to 20 years at the time of neurological evaluation. The degree of tremor varied with type of bow stroke and even the portion of the bow contacting the string. Only 1 patient had a slight postural tremor of the opposite limb. In 3 of 5 the tremor was task-specific; the other 2 had mild and nontroubling tremor with other activities. The tremor appeared to worsen over time but then seemed to stabilize. The characteristics of this tremor appear to be distinguishable from the features of both essential tremor and focal dystonia; comparison is made with representative string players afflicted by these other disorders. Analogy of this tremor is made with primary writing tremor, a well-defined task-specific movement disorder also sharing at least some features with both essential tremor and writers' cramp, a focal dystonia. Hence, it was decided to call this primary bowing tremor. Clinical features, family history, diagnostic studies, and responsiveness to treatment of primary writing tremor are discussed to emphasize the similarity to primary bowing tremor. This appears to represent a previously unreported form of task-specific movement disorder of string instrumentalists.

Focal peripheral neuropathies in instrumental musicians.

Instrumental musicians often seek medical consultation for symptoms suggestive of nerve entrapment. About 20% of those seen in the author's performing artists' clinic were diagnosed with a focal neuropathy. In general, neuropathies that are most common in the overall population tend also to be most common among musicians, although some expectations exist, including, for example, localized peri-oral sensory syndromes associated with playing a brass instrument, and, possibly, ulnar neuropathies related to the playing position of bowed string players. The diagnosis is made, as always, by careful clinical assessment, including observation of the instrumentalist playing, with ancillary procedures such as nerve conduction studies and needle electromyography adding to the accuracy of the diagnosis. Treatment is similar to that used in nonmusicians, but certain factors, including the musician's requirement for extraordinary neuromuscular dexterity, may influence the therapeutic decisions. Very limited long-term outcome results are available, and additional studies in musicians would be helpful in determining the most appropriate therapeutic approaches. Virtually no longitudinal studies have been performed to look at methods for preventing these disorders.

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.

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.

Multifocal varicella-zoster virus vasculopathy without rash.

A 51-year-old woman with CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) developed stepwise progressive focal neurological deficits without zoster rash. Multifocal ischemic infarcts were seen on magnetic resonance imaging, and cerebral angiography revealed focal stenosis of arteries affecting the intracranial circulation. A brain biopsy was nondiagnostic. Virological etiology of the disease was verified by the detection of varicella-zoster virus antibody in cerebrospinal fluid and by reduced serum-cerebrospinal fluid varicella-zoster virus IgG ratios (compared with normally high ratios of total IgG and albumin). Treatment with intravenous acyclovir stabilized but did not significantly improve her neurological deficits.

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.

Neuromuscular problems in musicians.

BACKGROUND: Musicians are an occupational (or avocational) group that may on occasion have highly specific health care problems apparently caused by or adversely affecting instrumental performance. Neurologists have been intimately involved in the development of a burgeoning interest in these disorders and, because of the nature of the most common symptoms, neurologists can expect to be called upon to evaluate such patients. REVIEW SUMMARY: In this review, the most common playing-related disorders are discussed. These include the regional pain syndromes, primarily involving the neck and upper extremity, the focal neuropathies, again predominantly involving the upper extremity, and the focal dystonias or occupational cramps, which typically affect the hand or the cranial-innervated muscles involved in the embouchure (the relationship of the facial musculature to the mouthpiece of the instrument). Risk factors contributing to the development of these disorders are reviewed, the diagnostic approach is described, and the management of these playing-related problems is summarized. Aspects in which the instrumental musician may differ from other patients commonly seen by the neurologist, particularly with respect to the types of problems seen, methods of evaluation, and therapeutic strategies, are emphasized. CONCLUSIONS: Many unanswered questions remain in each of these areas. Neurologists and neuroscientists are in an excellent position to help fill the voids in our knowledge base. It is hoped that the reader will be stimulated to participate in this effort.