Ultrasonographic findings in hereditary neuropathy with liability to pressure palsies.

OBJECTIVES: The aims of this study were to evaluate the sonographic findings of patients with hereditary neuropathy with liability to pressure palsies (HNPP) and to examine the correlation between sonographic and electrophysiological findings. METHODS: Nine patients whose electrophysiological findings indicated HNPP and whose diagnosis was confirmed by genetic analysis were enrolled in the study. The median, ulnar, peroneal, and tibial nerves were evaluated by ultrasonography. RESULTS: We ultrasonographically evaluated 18 median, ulnar, peroneal, and tibial nerves. Nerve enlargement was identified in the median, ulnar, and peroneal nerves at the typical sites of compression. None of the patients had nerve enlargement at a site of noncompression. None of the tibial nerves had increased cross-sectional area (CSA) values. There were no significant differences in median, ulnar, and peroneal nerve distal motor latencies (DMLs) between the patients with an increased CSA and those with a normal CSA. In most cases, there was no correlation between electrophysiological abnormalities and clinical or sonographic findings. DISCUSSION: Although multiple nerve enlargements at typical entrapment sites on sonographic evaluation can suggest HNPP, ultrasonography cannot be used as a diagnostic tool for HNPP. Ultrasonography may contribute to the differential diagnosis of HNPP and other demyelinating polyneuropathies or compression neuropathies; however, further studies are required.

Clinical REM sleep behavior disorder and motor subtypes in Parkinson's disease: a questionnaire-based study.

OBJECTIVES: Studies documenting the association between rapid eye movement sleep behavior disorder (RBD) and motor subtypes in Parkinson's disease (PD) are rare. Our hypothesis is that RBD may be more severe in non-tremor dominant (NTD) patients with RBD than those tremor dominant (TD) with RBD. In this study, we investigated the association between motor subtypes and clinical RBD in PD. PATIENTS AND METHODS: We evaluated 104 consecutive patients older than 18 years presenting with PD to the Neurology Clinic of the University Hospital for one year in this study. The clinical diagnosis of RBD was based on the minimal diagnostic criteria of International Classification of Sleep Disorders, revised. The Stavanger Sleepiness Questionnaire was used to rate the severity of clinical RBD. The patients were divided into two subgroups as TD and NTD. The patient and control groups were compared with each other for severity and frequency of clinical RBD, and the Unified Parkinson's Disease Rating Scale (UPDRS) and Hoehn-Yahr stage scores. The correlation between severity of clinical RBD and clinical severity of PD was analyzed in the patient groups. RESULTS: Of the patients, 45.2% (n=47) had the NTD subtype of PD and 54.8% (n=57) had the TD subtype of PD. There was no significant difference among the groups in terms of frequency and severity of clinical RBD. For the NTD patients, there was a weak positive correlation between severity of clinical RBD and clinical severity of PD. However, there was no correlation in the TD subgroup. CONCLUSION: In our study, frequency of clinical RBD was unrelated to motor subtypes of PD. However, in the present study, we found a weak correlation between clinical severity (UPDRS and the Hoehn-Yahr) of PD and severity of clinical RBD in the NTD subtype but not in the TD subtype.

Phenobarbital-induced pellagra resulted in death.

Pellagra is caused by deficiency of niacin or its precursor tryptophan. While cutaneous lesions are the most prominent feature of the disease, gastrointestinal, neurological and psychiatric signs and symptoms are the other characteristics of the disease. In this case report, we present a 29-year-old female patient with discoloration of hands and feet diagnosed with pellagra.

Syncope associated with subthalamic nucleus deep brain stimulation in a patient with Parkinson's disease.

In advanced Parkinson's disease (PD), deep brain stimulation (DBS) may be an alternative option for the treatment of motor symptoms. Side effects associated with subthalamic nucleus (STN) DBS in patients with PD are emerging as the most frequent sensory and motor symptoms. DBS-related syncope is reported as extremely rare. We wanted to discuss the mechanisms of syncope associated with STN DBS in a patient with Parkinson's disease. Case report. Sixty-three-year-old female patient is followed up with diagnosis of idiopathic Parkinson's disease for 6 years in our clinic. The patient has undergone STN DBS due to painful dystonia and drug resistant tremor. During the operation, when the left STN was stimulated at 5 milliampere (mAmp), the patient developed presyncopal symptoms. However, when the stimulation was stopped symptoms improved. During the early period after the operation, when the right STN was stimulated at 1.3 millivolts (mV), she developed the pre-yncopal symptoms and then syncope. Our case shows that STN DBS may lead to directly autonomic symptoms resulting in syncope during stimulation-on (stim-on).

Is symptomatic atherosclerotic cerebrovascular disease a risk factor for normal-tension glaucoma?

OBJECTIVE: To compare the incidence of glaucomatous optic disk appearance between patients with symptomatic atherosclerotic stroke and healthy individuals with normal intraocular pressures (IOP). SUBJECTS AND METHODS: 46 patients with ischemic stroke with evident lacunar infarction or large vessel atherosclerosis, and 93 age- and sex-matched healthy individuals, all with normal IOP, were included. Patients and controls were examined for the presence of high cup-to-disk ratios (> 0.5). RESULTS: Seven patients (15.22%) in the ischemic cerebrovascular disease (CVD) group and 3 controls (3.23%) had glaucomatous optic disk appearance. All subjects with glaucomatous optic disk appearance in the control group and 3 patients in the study group had visual field defects in concordance with normal-tension glaucoma (NTG). The incidence of glaucomatous optic disk appearance was significantly higher in the group with symptomatic atherosclerotic CVD. CONCLUSION: Atherosclerotic CVD is a risk factor for having glaucomatous optic disk appearance. Symptomatic atherosclerosis involving the brain vasculature may also affect the eye and lead to NTG. Patients with ischemic strokes due to large artery atherosclerosis or small artery occlusion must be examined and followed for NTG.

Visual evoked potentials in guillain-barré syndrome.

BACKGROUND AND PURPOSE: Guillain-Barré syndrome (GBS) is an acute demyelinating polyneuropathy with various clinical features. Optic neuritis occurs in rare cases. In this study we determined the incidence and patterns of visual evoked potential (VEP) abnormality in GBS in association with ophthalmologic findings. METHODS: Thirty-two patients with a diagnosis of GBS were included in the study. The correlation between pathologic VEPs and categories of neurologic deficit and electrophysiological findings were examined statistically. RESULTS: The patients ranged in age from 19 to 77 years. Five cases (16%) had abnormal VEPs. All five of these patients exhibited increased P100 latency differences between the two eyes. Other abnormalities were prolonged p100 latency, increased interocular amplitude difference, and distorted p100 configuration. Pathologic signs on ophthalmologic examination were observed in 80% of patients with abnormal VEPs. VEP abnormality was never present in pure axonal forms. There was no significant correlation between pathologic VEP and cerebrospinal fluid protein level or categories of neurologic deficits. CONCLUSIONS: Involvement of the optic pathways is not a frequent finding in GBS. When present it is always asymmetric and generally accompanied with pathologic findings on ophthalmologic examination. VEPs may be abnormal in different clinical variants of GBS, and especially in demyelinating forms.

Does long term use of piracetam improve speech disturbances due to ischemic cerebrovascular diseases?

Aphasia causes significant disability and handicap among stroke survivors. Language therapy is recommended for aphasic patients, but not always available. Piracetam, an old drug with novel properties, has been shown to have mild beneficial effects on post-stroke aphasia. In the current study, we investigated the effects of 6 months treatment with piracetam on aphasia following stroke. Thirty patients with first-ever ischemic strokes and related aphasia were enrolled in the study. The scores for the National Institutes of Health Stroke Scale (NIHSS), Barthel Index (BI), modified Rankin Scale (mRS), and Gülhane Aphasia Test were recorded. The patients were scheduled randomly to receive either 4.8 g piracetam daily or placebo treatment for 6 months. At the end of 24 weeks, clinical assessments and aphasia tests were repeated. The level of improvement in the clinical parameters and aphasia scores was compared between the two groups. All patients had large lesions and severe aphasia. No significant difference was observed between the piracetam and placebo groups regarding the improvements in the NIHSS, BI and mRS scores at the end of the treatment. The improvements observed in spontaneous speech, reading fluency, auditory comprehension, reading comprehension, repetition, and naming were not significantly different in the piracetam and placebo groups, the difference reached significance only for auditory comprehension in favor of piracetam at the end of the treatment. Piracetam is well-tolerated in patients with post-stroke aphasia. Piracetam taken orally in a daily dose of 4.8 g for 6 months has no clear beneficial effect on post-stroke language disorders.

Manifestation of Lambert-Eaton myasthenic syndrome during last trimester of pregnancy.

Lambert-Eaton myasthenic syndrome (LEMS) is a rare immune-mediated disorder of neuromuscular junctions. The knowledge of the effects of pregnancy on the course of patients with LEMS is limited. Here we describe a patient without a history of previous known illness who has complained of weakness during the last trimester of her pregnancy, delivered a healthy baby and was diagnosed with non-paraneoplastic LEMS during the postpartum period. With this case we wanted to emphasize the effects of pregnancy on the course of patients with LEMS.

Electrophysiological assessment of polyneuropathic involvement in rheumatoid arthritis: relationships among demographic, clinical and laboratory findings.

OBJECTIVES: The aims of this study were to electrophysiologically evaluate polyneuropathy in rheumatoid arthritis (RA) patients and to examine the relationships among polyneuropathy and demographic, clinical and laboratory findings. PATIENTS AND METHODS: Sixty consecutive patients (51 women and nine men) with a clinical diagnosis of RA were examined electrophysiologically for the evidence of polyneuropathy. Parameters including age, gender, subcutaneous nodules, erosions, joint deformities, laboratory parameters, duration of RA, as well as dose, duration and type of disease modifying anti-rheumatic drug (DMARD) and steroid usage were recorded. RA activity was assessed using a 28-joint disease activity score (DAS28). The functional status of patients was measured using the health assessment questionnaire (HAQ). The symptoms and signs of polyneuropathy were quantified using the neuropathy symptoms score (NSS) and the neuropathy disability score (NDS), respectively. RESULTS: Ten patients (17%, eight women and two men) had polyneuropathic involvement as defined by nerve conduction studies (NCS). Two patients had mild symmetric sensory neuropathy and eight patients had mild symmetric sensorimotor axonal polyneuropathy. There was no significant difference in age, gender, subcutaneous nodules, erosions, joint deformities, rheumatoid factor, as well as dose, duration and type of DMARD and steroid therapy administered. We found a significant relationship among polyneuropathy and duration of RA, DAS28, HAQ, as well as abnormal NSS and NDS values. The durations of RA and DAS28 were also associated with a four- and three-fold increase in the risk of polyneuropathy, respectively. CONCLUSION: Mild symmetric sensory or sensorimotor axonal polyneuropathies are common in RA patients and it is difficult to distinguish the symptoms of polyneuropathy from those of arthritis. An electrophysiological examination should be routinely carried out especially when patients have had a long disease duration and high scores for DAS28, HAQ, NSS and NDS.

Effects of haemodialysis and continuous ambulatory peritoneal dialysis on P300 cognitive potentials in uraemic patients.

The aim of this study was to determine the effects of haemodialysis (HD) and continuous ambulatory peritoneal dialysis (CAPD) on P300 cognitive potentials in patients with chronic renal failure (CRF) and to find out if P300 potential is a valuable marker for following subclinical cognitive disorder. This study was performed in 42 patients with chronic uraemia, of whom 25 were on HD and 17 on CAPD, and in 25 healthy subjects. All the subjects were investigated in terms of P300 cognitive potential obtained from auditory stimuli with the oddball paradigm and the Mini-Mental State (MMS) examination. Patients undergoing HD were evaluated before (pre-dialysis) and after (post-dialysis) standard HD treatment. P300 latency was longer in HD patients than in the control group and CAPD patients. The MMS score was greater and P300 latency was shorter after a standard HD session when compared to pre-dialysis values in HD patients (p < 0.001 for both values). There was a negative correlation between P300 latency and MMS scores, and a positive correlation between P300 amplitude and MMS scores. In conclusion, P300 is useful for evaluating cognitive function in uraemia, even in asymptomatic patients, and CAPD is superior to HD in the management of cognitive impairment.