The impact of familial structure on Parkinson's disease in Istanbul Medical School, Turkey.

The prevalence and family structure of idiopathic Parkinson disease (iPD) in Turkey is not known. Patients with iPD were recruited consecutively at the Medical School of Istanbul University over an 18-month period. Clinical details were assessed with standardized forms. Of the 219 iPD patients, 136 had sporadic iPD [26 with parental consanguinity (cs)], 20 autosomal recessive PD (9 with cs) and 63 autosomal dominant or pseudo-dominant inheritances (20 with cs). Age at onset was 49.1 ± 17.1 years (range 3-83) and age at examination 56.4 ± 16.5 years (range 4-93). Ages at examination and at clinical onset of PD were significantly greater in sporadic iPD than in familial iPD patients, but disease duration was similar. Patients with familial PD had significantly lower basal UPDRS III and Hoehn and Yahr scores than sporadic PD patients and brisk reflexes in the lower limbs were significantly more frequent, but they suffered less from mictional problems. The frequency of familial PD and consanguinity in Turkey is higher and age at onset of iPD earlier than in Western countries. Molecular diagnoses and genetic counseling will therefore have a very important impact on the medical, psychological, and familial handling of PD in Turkey.

Short segment incremental study in ulnar neuropathy at the wrist: report of three cases and review of the literature.

Ulnar nerve lesions may occur at different sublocations at wrist and may involve various branches of the nerve. Standard neurophysiological studies are generally insufficient in revealing these lesions. Demonstration of conduction block and/or focal slowing of nerve conduction is the most definitive electrodiagnostic evidence for the localization of segmental demyelination. Short-segment incremental study (SSIS) is a sensitive technique for detecting the ulnar neuropathy at the wrist (UNW). We report 3 cases of UNW caused by ganglion cysts in Guyon's canal which were studied by using SSIS across the wrist. Even though SSIS is a time-consuming and technically demanding method, it increases the electrodiagnostic potential of detecting segmental demyelination in this location.

Electromyography in Pediatric Population.

INTRODUCTION: Electrodiagnostic evaluation provides an important extension to the neurological examination for the evaluation of pediatric neuromuscular disease. Many pediatric neuromuscular diseases are analogous to those seen in the adult. However, the relative frequency of these illnesses varies greatly when different age populations are compared. The purpose of the present study is to provide a retrospective analysis of children referred to our electromyography (EMG) laboratory for electrophysiological examinations. METHODS: We retrospectively reviewed electrodiagnostic records of patients aged between 0-15 years, from January 2004 to June 2013. Patients were classified as having plexopathy, nerve root lesions, polyneuropathy, myopathy, mononeuropathy, anterior horn cell disease, neuromuscular transmission disorder, facial nerve palsy, and other rare disorders. RESULTS: We reviewed totally 5563 pediatric records, which was on the average 578 studies per year. It was about 14% of the all EMG examinations performed in our laboratory. When we looked at all the procedures, 3271 of the records included needle EMGs, 170 of them were single fiber EMGs, 100 of them were repetitive nerve stimulations, and 52 of them were evoked potentials. The results were normal in 55% of the cases. As a result of electrophysiological examinations, the common diagnoses were: plexopathy (28.6%), polyneuropathy (7.4%), and myopathy (6.6%) in patients aged 0-5 years (41.2% of all records); myopathy (9.4%), PNP (8.5%), mononeuropathy (6.4%), and plexopathy (5.9%) in 6-10 years (28.2% of all records); PNP (11.3%), myopathy (6.6%), and mononeuropathy (5.6%) in 11-15 years (30.6% of all records). CONCLUSION: Infants and toddlers mostly suffered from brachial plexopathy which can be prevented by proper obstetrical management. Nerve conduction studies and EMG yielded diagnostic importance for demyelinating neuropathy and myopathy in patients older than 6 years of age.

Pain is common in Parkinson's disease.

OBJECTIVE AND BACKGROUND: Patients with Parkinson's disease may present with severe or intractable pain, which can be more distressing than the motor disability. The aim of this prospective study was to assess the prevalence of pain and underlying causes in patients with idiopathic Parkinson's disease. PATIENTS AND METHODS: Ninety-six patients (42 female, 54 men) were interviewed and pain was assessed using patient descriptions, Visual Analog Scale (VAS) and Leeds Assessment of Neuropathic Symptoms and Signs (LANSS). Stait Trait Anxiety Inventory (STAI-TX 1 and 2) and Beck Depression Scale were also administered. RESULTS: Pain as the first symptom of PD was seen in 3 patients (2.8%), 63 (64.9%) out of 96 patients reported pain. Pain types included musculoskeletal type of different etiologies (28 patients, 44.4%), radicular or neuropathic pain (7 patients, 11.1%), pain secondary to dystonia (12 patients, 19.1%) and central pain (8 patients, 12.7%). Eight patients (12.7%) described more than one type of pain. Pain did not correlate with sex, duration of disease, disease stage, use of dopamine agonists and levodopa, years of levodopa treatment and current levodopa dosage, depression, anxiety, sleep disturbances, age at onset of PD or history of disease in first-degree relatives. Akathisia seemed to be correlated with presence of pain (p<0.02). CONCLUSIONS: Our results suggest that pain is one of the most common non-motor symptoms in patients with PD. In order to identify the appropriate treatment strategy, it is essential to identify the underlying etiology.