Diagnostic value of ultrasonography in peroneal neuropathy

Background/aim: Peroneal neuropathy at the fibular head (PNFH) is one of the most common entrapment neuropathies. Our aim in this study was to analyze the efficiency of ultrasonography in the diagnosis of PNFH. Materials and methods: The study included 15 peroneal nerves of 12 patients with PNFH and 24 peroneal nerves of 12 healthy controls. PNFH confirmation was based on clinical and electrophysiological findings. All patients and controls underwent ultrasonographic evaluations for peroneal nerves. The cross-sectional area (CSA) was measured. Echogenicity of the nerve was evaluated by comparing it with the adjacent connective tissue deep under the subcutaneous fat. Results: CSA measurement of the peroneal nerve is a valuable diagnostic tool in predicting PNFH (AUC: 0.87, 95% CI: 0.73­1.00, P < 0.01). The CSA cutoff value for diagnosing PNFH was found to be 0.115 cm 2 with 80% sensitivity and 99% specificity. Hypoechoic peroneal nerve in patients with PNFH was very frequent. Conclusion: Ultrasonography is a useful technique in diagnosing PNFH. In addition to clinic and electrophysiological findings, it may improve diagnostic performance.

Familial Restless Legs Syndrome: A Family with all Female Patients.

BACKGROUND: Restless legs syndrome (RLS) is a chronic condition characterized by odd sensations in the body, most commonly in the legs and an irresistible urge to move them. More than half of the patients with RLS have a family history. Most of the RLS cases are women and most of the families show characteristics of an autosomal dominant pedigree. Here, we shall present a family consisting only of women; to our knowledge, such a family has not been reported yet. METHODS: The family presented here met the diagnosis criteria specified by International Restless Legs Syndrome Study Group (IRLSS). Clinical characteristic are described along with demographic properties. RESULTS: The patients were between 12 and 59 years of age with a mean age of 35.3 ± 14.4 years. All 7 cases were women. The pedigree of the patients exhibited an autosomal dominant inheritance pattern. CONCLUSIONS: The present family tree indicates that familial RLS can exhibit a heredity pattern which shows autosomal dominant inheritance. The present family is still under follow-up. Future research is required to support the present data.

Diagnosing carpal tunnel syndrome with Doppler ultrasonography: a comparison of ultrasonographic measurements and electrophysiological severity.

INTRODUCTION: Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy. Diagnosis is based on clinical history, physical examination, and electrophysiological studies. Imaging techniques are performed for difficult-to-diagnose cases because they provide information about the morphology of the median nerve. More recently, it has been shown that Doppler ultrasonography can detect increased intraneural blood flow in CTS. The aim of our study is to evaluate the relationship between the severity of CTS, hypervascularization, and cross-sectional area (CSA) to determine the diagnostic value of Doppler ultrasonography. PATIENTS AND METHODS: The study group comprised 125 wrists of 75 patients who had been diagnosed with CTS, both clinically and electrophysiologically. The control group comprised 100 wrists of 50 healthy volunteers. Wrists were classified into five stages of CTS severity based on electrophysiologic studies. A radiologist examined the wrists blindly with grayscale images and Doppler ultrasonography to assess CSA and hypervascularization. RESULTS: A total of 121 wrists were included. There were 28 wrists with minimal CTS severity stage, 36 with mild, 36 with moderate, and 21 with severe. The sensitivity and specificity of CSA and hypervascularization in detecting CTS was 90.9, 94.0, 93.4, and 90.0%, respectively. There was a significant correlation between CTS severity and hypervascularization (p < 0.005) for all stages. CONCLUSION: Our study shows that Doppler ultrasonography results strongly correlate with CTS severity. Hence, this is a useful method for diagnosing CTS and estimating its severity.

Electroneuromyographic Features in Fabry Disease: A Retrospective Review.

INTRODUCTION: Fabry disease (FD) is an X-linked recessive inherited disorder characterized by lysosomal alpha-galactosidase deficiency. The purpose of our study was to assess and compare the electroneuromyographic (ENMG) findings of 15 patients with Fabry disease and the electroneurographic (ENG) findings of 15 healthy controls. We have not encountered any similar study in the medical literature of our country. Therefore, we believe that our study will contribute to national literature. METHODS: Fifteen patients with Fabry disease, 13 females and 2 males and 15 healthy controls, 13 females and 2 males, were included in the study. The definite diagnosis of patients with Fabry disease was made based on the enzyme level and genetic mutation. The patients with Fabry disease were examined with ENMG, while the healthy control group was examined with ENG. In the patients with a normal ENMG examination, neuropathic pain was attributed to the small fiber involvement. RESULTS: Patients with Fabry disease had neuropathic pain (LANSS score≥12). While neurological examination was normal in eight patients, glove- and stocking-type hypoesthesia and decreased deep tendon reflexes were observed in five and two patients, respectively. Axonal polyneuropathy was detected in one patient. The ENMG examinations of the other patients were normal. Enzyme replacement therapy could not be initiated in one patient because of pregnancy. The neurological examination of the healthy control group was normal. There was no statistically significant difference between the ENMG features of both groups (p>.05). As in other studies, a routine ENMG examination was normal in our patients with early-stage Fabry disease. Neuropathic pain, seen in patients with Fabry disease in literature, is thought to be due to small fiber involvement. CONCLUSION: Fabry disease should be considered in the differential diagnosis of patients with neuropathic pain at young ages. It should be kept in mind that ENMG examination can be normal at the early stages. Quantitative sensory test, autonomic tests (R-R interval and sympathetic skin response) and skin biopsy should be performed in such cases. In our country, pediatric physicians work on Fabry disease more than physicians dealing with Fabry disease in adults. Therefore, in this retrospective study, we aimed to draw adult and pediatric neurologists' attention to Fabry disease.

Neurological Complications after Renal Transplantation: A Retrospective Clinical Study.

INTRODUCTION: The aim of this study was to evaluate the incidence and types of neurological complications (NCs) and associated factors in renal transplantation (RT) patients. METHODS: Three hundred and forty-four patients who had RT performed at our institution between January 2005 and July 2014 were retrospectively evaluated. RESULTS: File records of the patients revealed 19 who experienced a total of 22 episodes of NCs, of whom three had more than one episode. The mean age of 19 patients included in the study, of whom eight were female, was 37.52±13.08 (range, 18-65) years. NCs were classified into central or peripheral depending on the location of involvement of the central nervous system (CNS). CNS involvement was found in 16 (84.2%) of the 19 patients. Tremor (36.8%) was the most common CNS complication in these patients. Encephalopathy, generalized tonic-clonic seizures, and status epilepticus were observed in two patients (10.5%). Delirium and dementia were observed in one patient (5.2%). Headache was experienced by one patient, and agitated depression was observed in one patient. Six patients (26.3%) had the peripheral nervous system involvement. One patient had the numbness of hands with normal electromyography findings, and four patients had polyneuropathy. In one patient, lumbar plexopathy was observed. Seventeen of the 22 NCs were considered to be caused by immunosuppressive agents. Each incidence of amyloidosis, infection, septic emboli, and hypoglycemia caused a neurological episode. The etiology of one episode was unknown. CONCLUSION: Different neurological disorders can be seen after RT, and most of them are caused by immunosuppressive drugs. NCs seen after RT can be treated by decreasing the dose or changing the immunosuppressive drug.

Validation of self report version of the Leeds Assessment of Neuropathic Symptoms and Signs score for identification of neuropathic pain in patients from northern Turkey.

BACKGROUND: Diagnosis and treatment of neuropathic pain is an important clinical problem. OBJECTIVES: A self report version of the Leeds Assessment of Neuropathic Symptoms and Signs (S-LANSS) score provides identification of neuropathic pain without the help and need of a clinician. We targeted validation of the S-LANSS score in the northern Turkish population in this study. MATERIAL AND METHODS: For the linguistic validation of S-LANSS, translation and back-translation method was used to adapt S-LANSS into Turkish and a cognitive-debriefing test was performed. A total of 148 patients were enrolled in the present study. S-LANSS, The Leeds Assessment of Neuropathic Symptoms and Signs (LANSS), Visual Annalogue Score (VAS) and Neuropathic Pain Questionnaire (NPQ) were performed twice for every patient. The patients were examined and diagnosed as having nociceptive or neuropathic pain by neurologists, who were blind for S-LANSS, LANSS and NPQ scores of the patients. RESULTS: Results of the McNemar test indicated that S-LANSS scores were reliable when the first and the second scores were compared. The sensitivity and specificity of the scale were found to be 98% and 97% respectively. CONCLUSIONS: We believe that using S-LANSS scores for the diagnosis of neuropathic pain may help our colleagues as a tool for a quicker differential diagnosis of pain in daily practice.

Propriospinal myoclonus in a child.

A 6-year-old girl was experiencing repetitive involuntary and massive jerks immediately involving limbs and trunk. The first motor events appeared approximately at 1 year old and only 5 months after a back trauma. Myoclonus became progressively more frequent and more violent, causing episodes of falls. Neurological examination showed jerks characterized by upper limb abduction, lower limb abduction, and head-body hyperextension. Apart from these motor events, the neurological examination was normal. The results of vitamin B(12) and folate, antinuclear antibody, anti-DNA, anti-Tiroglobulin, anti-thyroid peroxidase antibody, lupus anticoagulant, anti-cardiolipin antibody, rheumatoid factor, and C3 and C4 were unexceptional. Electroencephalography and brain and spinal magnetic resonance imaging were unremarkable. Electromyographic records with surface electrodes showed that duration of myoclonic jerks was ranging from 100 to 300 ms. We thought she had propriospinal myoclonus because of presence of the spreading through the shoulder, upper limbs, and lower limbs in addition to thoracolumbar paraspinal muscles.

Ultrasonography in patients with ulnar neuropathy at the elbow: comparison of cross-sectional area and swelling ratio with electrophysiological severity.

The aim of this study was to determine the diagnostic value of ultrasonographic measurements in ulnar neuropathy at the elbow (UNE) and to assess the relationship between the measurements and the electrophysiological severity. The largest anteroposterior diameter (LAPD) and cross-sectional area (CSA) measurements of the ulnar nerve were noted at multiple levels along the arm, and the distal-to-proximal ratios were calculated. Almost all of the measurements and swelling ratios between patients and controls showed statistically significant differences. The largest CSA, distal/largest CSA ratio, CSA at the epicondyle, and proximal LAPD had larger areas under the curve than other measurements. The sensitivity and specificity in diagnosing UNE were 95% and 71% for the largest CSA, 83% and 85% for the distal/largest CSA ratio, 83% and 81% for the CSA at the epicondyle, and 93% and 43% for the proximal LAPD, respectively. There was a statistically significant correlation between the electrophysiological severity scale score (ESSS) and the largest CSA, the CSA at the epicondyle and 2 cm proximal to the epicondyle, and the LAPD at the level of the epicondyle (P < 0.05). None of the swelling ratios showed a significant correlation with the ESSS. The largest CSA measurement is the most valuable ultrasonographic measurement both for diagnosis and determining the severity of UNE.

Hereditary neuropathy with liability to pressure palsy (HNPP) in childhood: a case study emphasizing the relevance of detailed electrophysiological examination for suspected HNPP in the first decade.

Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant disorder characterized by recurrent mono-neuropathies secondary to minor trauma or compression. Whilst typical episodes of palsy generally become apparent during the second and the third decades, HNPP is rarely diagnosed in the first decade. We present the case of a 6-year-old patient to draw attention to the possibility of HNPP attacks in the first decade and the importance of detailed electrophysiological examination.

Visual evoked potentials in differential diagnosis of multiple sclerosis and neurobehcet's disease.

Behcet's disease, a multisystemic vascular inflammatory disorder of unknown origin, is relatively rare and central nervous system involvement is seen in 5% of affected individuals. This form of the disease, called as neurobehcet's disease (NB), can be misdiagnosed as multiple sclerosis (MS), a demyelinating disorder of central nervous system, so their differential diagnosis is important. In this study, to identify the parameters of electrophysiological testing that might be useful in their differential diagnosis, we performed evoked potentials (EPs) and electroneuromyography (ENMG) on patients with MS and NB, and on normal volunteers. A total of 95 persons, 55 MS patients, 20 NB patients and 20 normal volunteers between ages 31 and 55, were studied electrophysiologically. Visual evoked potential (VEP), brainstem auditory evoked potential (BAEP), posterior tibial somatosensory evoked potential (SEP) and nerve conduction and needle electromyography studies were performed on all patients and volunteers. All parameters of EPs were compared among the groups. The results of the BAEP and SEP studies did not show statistically significant difference between NB and MS. However, the VEP study indicated that the amplitude values of cortical VEP potentials (P100) in the NB and MS groups were lower than those of the normal group (p < 0.01), and that the amplitudes in the NB group were lower than for the MS group (p < 0.05). Therefore, P100 amplitude measured from peak to peak seems to be more reliable and thus should be used in the differential diagnosis of MS and NB.

Bifid median nerve in patients with carpal tunnel syndrome.

OBJECTIVE: The aim of this study was to investigate the frequency of the anatomic variation of a bifid median nerve in patients with carpal tunnel syndrome (CTS) and to determine the size criteria for sonography. METHODS: On axial sonograms of 320 hands of 170 patients with CTS and 240 hands of 120 unaffected individuals, the median nerve was evaluated morphologically for a bifid median nerve, and the cross-sectional area was measured at 3 levels (radial-ulnar junction, pisiform, and hook of the hamate). Electrophysiologic studies were performed in addition to clinical and sonographic evaluations in all patients, controls with a bifid median nerve, and controls with a cross-sectional area of greater than 0.09 cm(2). RESULTS: A bifid median nerve was seen in 32 (19%) of 170 patients and 11 (9%) of 120 controls. It occurred relatively frequently in patients with CTS (P < .01). The cross-sectional area of the bifid median nerve was relatively higher than that of the nonbifid median nerve in controls at 2 of the 3 levels (P < .001; P = .226; P < .01). The cutoff values for the cross-sectional area at the level of the pisiform were 0.11 cm(2) (sensitivity, 90%; specificity, 99%; P < .001) for patients with a bifid median nerve and 0.10 cm(2) (sensitivity, 98%; specificity, 81%; P < .001) for patients with a nonbifid median nerve. CONCLUSIONS: A bifid median nerve occurs relatively frequently in patients with CTS. It may facilitate compression of the median nerve in the carpal tunnel because of its relatively higher cross-sectional area compared with a nonbifid median nerve. The size criterion for CTS in patients with a bifid median nerve is slightly higher than in those with a nonbifid median nerve.

Hypothyroid myopathy with manifestations of Hoffman's syndrome and myasthenia gravis.

Although hypothyroid myopathy is seen frequently and the relationship with autoimmune hypothyroidism and myasthenia gravis is well known, specific forms of hypothyroid myopathy such as Hoffman's syndrome (HS) are rarely described. Here we describe a 40-year-old patient with Hashimoto thyroiditis showing symptoms and signs of two discrete forms of hypothyroid myopathy (HS and myasthenic syndrome) together. To our knowledge this is the first reported case with features of both of these syndromes. We discuss the diagnosis, speculate whether this patient may represent a unique form of hypothyroid myopathy, and report the 6-month follow-up of the patient both clinically and electrophysiologically.

Axillary neuropathy mimicking quadrilateral space syndrome and its follow up for one year.

Axillary neuropathy due to entrapment of the nerve in the quadrilateral space is seen rarely. Here, we describe a 24-year-old patient diagnosed with isolated axillary neuropathy that mimicked quadrilateral space syndrome. Quadrilateral or quadrangular space syndrome (QSS), first described by Cahill and Palmer in 1983, was defined as the entrapment of the distal branch of the axillary nerve and the posterior humeral circumflex artery (PHCA) in the quadrangular shaped anatomic space. We tracked the follow up of the patient for one year both clinically and electrophysiologically. Our aim in reporting this case is to stress the point that cases of such a nature usually represent situations of diagnostic and treatment challenges, where multidisciplinary approaches are required.

Pyridoxine and pyridostigmine treatment in vincristine-induced neuropathy.

Vincristine is a commonly used antineoplastic drug and frequently causes neurotoxicity. Here the authors report a 4-year-old boy with acute lymphoblastic leukemia in whom vincristine-induced peripheral and cranial neuropathy developed during remission induction therapy. The patient seemed to benefit from pyridoxine and pyridostigmine therapy greatly and this therapy is recommended in patients with severe vincristine-induced neuropathy.

Isolated lesion of the cervical spine in a case of neuro-Behcet.

Behcet`s disease is characterized by small vessel vasculitis and involvement of multiple systems. In neuro-Behcet cases, spinal cord involvement is approximately 10-18%. Spinal cord lesions are often associated with cerebral or brainstem lesions, and isolated spinal cord involvement in Behcet`s disease is very rare. Here, we report a case with an isolated lesion of the cervical spine.

Effects of high stimulus frequencies on SEPs of patients with neuro-Behcet's Disease.

BACKGROUND: Behcet's disease is a multisystemic vascular inflammatory disorder of unknownorigin. It is relatively rare and central nervous system involvement is seen in 5% of affected individuals. Somatosensory evoked potentials (SEPs) can provide information that shows the presence of clinically unsuspected lesions in the central nervous system of these patients. However, the effects of changing the stimulus frequencies on latencies of SEP potentials and central conduction time (CCT) in patients with neuro-Behcet's disease (NB) have not been studied yet. In this study, our aim was to reveal these effects to investigate whether the change of stimulus frequencies could be of convenient use in obtaining more accurate CCT estimations in SEP studies of these patients. METHODS: We performed median nerve SEPs of 14 patients with NB and 15 healthy volunteers. We changed the stimulus frequency: 2 Hz, 4Hz, 6Hz and 9Hz in successive recordings and statistically compared the changes on SEP potentials and peak and onset CCT in the neuro-Behcet (NB) group and the normal group. RESULTS: Our results indicated that the onset CCT values of the NB group were higher than the normal group at 4Hz and 9Hz stimulations. However, the comparison of peak CCT in the NB group and the normal group did not show any statistically meaningful differences at all stimulation frequencies. CONCLUSION: Onset CCT has not been measured before in former SEP studies of patients with NB. We highly recommend measuring onset CCT at higher stimulation frequencies in order to reveal central conduction time pathologies in these patients.