This report describes a 5-year-old girl with Langerhans' cell histiocytosis (LCH) of the clivus. To date only five patients, including our patient, have been described with LCH at this site. Our patient differs from those previously reported by her atypical clinical presentation with torticollis, but without a sixth nerve palsy. ...

OBJECTIVE: To assess the frequency and type of vascular changes and neurologic abnormalities in patients with erythromelalgia. DESIGN: Prospective study of patients with no spontaneous symptoms at the time of their visit and with provoked symptoms. SETTING: Tertiary referral center. PATIENTS: Sixty-seven patients presenting with erythromelalgia at Mayo Clinic, Rochester, ...

The authors investigated whether T cells have a role in the pathogenesis of axonal polyneuropathy and monoclonal gammopathy by comparing the presence of T cells in sural nerves of 23 patients with axonal polyneuropathy and monoclonal gammopathy (12 IgM, 11 IgG), of 15 patients with chronic idiopathic axonal polyneuropathy, and ...

Poliomyelitis has recently been identified as a cause of muscle weakness in patients with West Nile virus (WNV) infection. However, the clinical spectrum of WNV-associated weakness has not been described. We reviewed data on 13 patients with WNV infection. Patients with muscle weakness were classified into one of three distinct ...

Trigeminal neuralgia is considered as a paroxysmal single nerve phenomenon. Abnormal sensory perception has been previously described in 15-25% of patients with clinical examination. Quantitative sensory testing (QST) was used to evaluate sensory perception in patients with idiopathic trigeminal neuralgia (ITN). Nine patients and 10 normal control subjects were evaluated ...

Because patients with scleroderma report neuropathic symptoms including numbness, paresthesias, and dysesthesias, we assessed peripheral nerve function in such patients. Fourteen scleroderma patients underwent complete neurologic examination, nerve conduction studies (NCS) and quantitative sensory testing (QST). Neurologic examination revealed reduced vibration (7) or pinprick (4) sensation in the upper or ...

OBJECTIVE: To assess the value of clinical (House-Brackmann grading) and neurophysiological (conventional electroneurography) monitoring of the facial nerve before and after (at day 10 and day 80) microsurgical parotidectomy in a group of patients with parotid tumors. STUDY DESIGN AND SETTING: From January 7, 1999, to February 27, 2001, 33 ...

Progression periods for Guillain-Barré syndrome (GBS) differ from those of chronic inflammatory demyelinating polyneuropathy (CIDP), but physicians could classify patients with CIDP within 4 weeks of onset as GBS. We studied and report the frequency of GBS patients who were later diagnosed as CIDP (11/663, 2%). Plasmapheresis or intravenous immunoglobulin ...

The purpose of this study is to define the prevalence of peripheral nerve injury associated with full-thickness tears of the rotator cuff presenting with shoulder muscle atrophy. Twenty-five patients with the diagnosis of full-thickness rotator cuff tear were included. Electrodiagnostic testing, including nerve conduction studies and needle examination, was performed ...

This paper reports three females and two males with a distinctive congenital syndrome characterized by severe congenital hypotonia, facial diplegia, jaw ankylosis, velo-pharyngeal incoordination, pyramidal tract signs, and ocular motor apraxia. Patients were followed up at ages ranging from 20 months to 16 years. All cases of this syndrome are ...

BACKGROUND: Brachioradial pruritus (BRP) is a localized pruritus of the dorsolateral aspect of the arm. BRP is an enigmatic condition with a controversial cause; some authors consider BRP to be a photodermatosis whereas other authors attribute BRP to compression of cervical nerve roots. OBJECTIVE: We sought to investigate the presence ...

Over a 33-year period, 260 patients (< 17 years of age; 119 males, 141 females) from New South Wales, Australia who had peripheral neuropathies confirmed by nerve biopsy, were studied. Of these, 50 infants presented with symptoms or signs of neuropathy under 1 year of age: including 24 patients with ...

OBJECTIVE: To measure the motor performance of the flexor pollicis brevis, first dorsal interosseus, and flexor pollicis longus muscles using the parameters electromyographic muscle reaction time and movement time in patients with carpal tunnel syndrome during key turning function. METHOD: A total of 37 female patients with right hand pain ...

BACKGROUND: Celiac disease (CD) is a chronic inflammatory enteropathy resulting from sensitivity to ingested gluten. Neurologic complications are estimated to occur in 10% of affected patients, with ataxia and peripheral neuropathy being the most common problems. The incidence and clinical presentation of patients with CD-associated peripheral neuropathy have not previously ...

OBJECTIVE: The aim of this study was to report a series of Japanese patients with neuropathic symptoms following HCV infection. PATIENTS AND METHODS: Fifteen patients with neuropathic symptoms and HCV infection were studied retrospectively (neuropathy group). We evaluated clinical and electrophysiologic findings. As a control group, we investigated prospectively 11 ...

Partial motor conduction block, an electrophysiological hallmark of demyelination, helps to identify acquired demyelinating neuropathies but its electrophysiological detection can be difficult. We report a technique that may be helpful in this regard. Twenty-five patients with partial motor conduction block secondary to acquired demyelinating polyneuropathy (ADP), 7 with amyotrophic lateral ...

BACKGROUND: Although the symptoms of unilateral "medically unexplained" or "functional" weakness and sensory disturbance present commonly to neurologists, little is known about their long term prognosis. OBJECTIVE: To determine the long term outcome of functional weakness and sensory disturbance. PATIENTS: A previously assembled cohort of 60 patients seen as inpatients ...

OBJECTIVES: A body of evidence shows that coeliac disease is associated with protean manifestations outside the intestine, and neurological disorders are well recognised. However, it remains obscure whether there are signs of clinical or subclinical nervous system involvement even in patients adopting an adequate gluten free diet. The aim of ...

This retrospective analysis was undertaken to determine whether a subset of diabetic patients with demyelinating polyneuropathy were similar to patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Ten patients meeting the clinical criteria for idiopathic CIDP were compared to nine patients with diabetes and demyelinating polyneuropathy. The diabetic patients with demyelinating ...

Previous reports have suggested that outcome is worse in the axonal compared with the demyelinating form of Guillain-Barré syndrome (GBS). We performed a retrospective study of 23 children with electrophysiologically confirmed cases of predominant subtypes of GBS to investigate this issue. The patients were classified based on the electrodiagnostic features: ...

OBJECTIVE: To report a new technique for unilateral brow suspension for facial paralysis that is minimally invasive, limits supraciliary scar formation, does not require specialized endoscopic equipment or expertise, and has proved to be equal to direct brow suspension in durability and symmetry. DESIGN: Retrospective survey of a case series ...

PURPOSE: The purpose of this study was to describe the clinical characteristics of a series of patients presenting with benign trigeminal sensory neuropathy. PATIENTS AND METHODS: We conducted a retrospective analysis of the clinical and pathologic characteristics of 23 patients presenting with facial numbness of unknown etiology. RESULTS: Patients presented ...

Ataxic sensory neuronopathy with Sjögren's syndrome is a devastating neurologic complication for which there is no established treatment. IV immunoglobulin (IVIG) was given to five patients with severe disabilities for an average of 12 years. Four patients showed remarkable improvement, two of whom responded after the first course. The authors ...

Guillain-Barré syndrome (GBS) is traditionally considered to be a large-fibre neuropathy. However, the presence of hypo-aesthesia, dysaesthesia and dysautonomia in GBS patients raises the possibility that small-diameter sensory and autonomic nerves may also be affected. To investigate small-fibre neuropathy in GBS, we performed a skin biopsy from the distal leg ...

Besides the common distal symmetrical sensory-motor polyneuropathy (DSP) that is often associated with autonomic dysfunction, diabetic patients may develop multifocal sensory-motor deficits (MDN) secondary to roots, plexus and nerve trunk involvement. Nerve ischaemia has been suggested as a common mechanism for the different patterns of diabetic neuropathies, yet the important ...

BACKGROUND: Graves' orbitopathy can produce proptosis, compressive optic neuropathy, and extraocular motility abnormalities; symptoms result from an increase in orbital volume due to expansion of intraorbital fat, with or without extraocular muscle involvement. STUDY DESIGN: We conducted a chart review of patients who underwent orbital decompression to treat Graves' orbitopathy. ...

Peripheral neuropathy is one of the clinical manifestations of the MELAS (mitochondrial encephalopathy with lactic acidosis and stroke-like episodes) syndrome, but its frequency and phenotypic variability have not been properly characterised. We therefore studied the clinical and electrophysiological features of peripheral neuropathy in 32 patients with the 3243A > G ...

OBJECTIVE: The study goal was to evaluate the association among laryngopharyngeal sensory deficits, pharyngeal motor function, and the prevalence of aspiration with thin liquids. STUDY DESIGN AND SETTING: We conducted a prospective study of 204 consecutive patients undergoing flexible endoscopic evaluation of swallowing with sensory testing and an assessment of ...

A 57-year-old female patient with widespread chronic plaque psoriasis and a 32-year-old male patient with severe oral lichen planus are reported, who developed sensory symptoms in the extremities 3 and 4 months after the onset of oral acitretin therapy, respectively. Both patients showed clinical and electrophysiological evidence of a sensory ...

We examined 29 patients with chronic progressive ganglionopathy of different etiology. Neurophysiological abnormalities were dominated by a widespread decrease in sensory nerve action potential amplitudes, which involved both upper and lower limb nerves, even in patients with asymmetrical or patchy clinical presentation. This impairment of sensory nerve conduction, reflecting a ...

OBJECTIVE: The pathophysiology and treatment of facial nerve paralysis associated with acute otitis media are still under debate. The objective of this study was to review treatment strategies and extent of recovery in adult patients with the aim of defining a standard treatment protocol for this rare pathologic condition. STUDY ...

To clarify the factors contributing to patient satisfaction with facial movement after the classic hypoglossal-facial nerve anastomosis, we examined 46 such patients who consented to an interview and video-recording. No correlation was seen between the physician's evaluation of returned function (according to Yanagihara's 40-point scale and the grading system of ...

Large number of people from 9 out of 18 districts of West Bengal, India are endemically exposed to arsenic contaminated groundwater due to drinking of tubewell water containing arsenic level above World Health Organization's maximum permissible limit of 50 microg/L. From our ongoing studies on neurological involvement in patients of ...

Sometimes before signs of lung tumor, peripheral nerve involvement with sensory neuropathy tell us about lung cancer. This sensory neuropathy often accompany with oat cell carcinoma of lung. The initial symptoms are numbness or paresthesic in a limb and over a period of days or weeks symptoms became bilateral and ...

These studies were intended to explore the relationship between autonomic neuropathy and erectile dysfunction (ED). Sensory thresholds reflecting the integrity of both large diameter, myelinated neurons (ie pressure, touch, vibration) and small diameter axons (ie hot and cold thermal sensation) were determined on the penis and finger. Data were compared ...

OBJECTIVE: To determine the value of subcutaneous fat aspiration in patients with sensorimotor peripheral neuropathy. PATIENTS AND METHODS: We retrospectively reviewed the medical records of all patients undergoing subcutaneous fat aspiration for suspected amyloidosis from January 1, 1994, through December 31,1999. We classified patients undergoing subcutaneous fat aspiration due to ...

The clinical and neurophysiological features of multifocal motor neuropathy (MMN) indicate selective involvement of motor axons, but pathological abnormalities in sensory axons suggest a more widespread disease process. The present study was undertaken to determine whether the focal abnormalities are associated with widespread subclinical abnormalities in motor axons. Threshold tracking ...

BACKGROUND: Anticipating the prospect of specific treatment, we studied a large group of Australians with Fabry's disease. AIMS: We aimed to: (i) document the clinical features of Fabry's disease in Australian patients, (ii) test the hypothesis that clinical features vary with specific mutation and blood group and (iii) assess small-fibre ...

PURPOSE: To ascertain the location of the facial nerve in patients with microtia by using thin-section computed tomography (CT). MATERIALS AND METHODS: Thin-section CT was performed in 66 ears of patients with microtia (unilateral, n = 12; bilateral, n = 34) and mandibulofacial dysostosis (MFD, n = 20). Findings were ...

OBJECTIVE: To analyze voice function before and after thyroidectomy for patients with normal preoperative voice using a standardized multidimensional voice assessment protocol. SUMMARY BACKGROUND DATA: The natural history of post-thyroidectomy voice disturbances for patients with preserved laryngeal nerve function has not been systematically studied and characterized with the intent of ...

Dejerine-Sottas disease (DSD) was originally described as a hypertrophic polyneuropathy characterized by onset in infancy or early childhood in patients born to unaffected parents. The clinical features included distal sensory changes with ataxia; pes cavus, at times with kyphoscoliosis; motor deficit and atrophy predominating in the distal lower limbs and ...

The strength-duration function is a classic measure of neural excitability. When studied on peripheral motor axons it reflects the intrinsic nodal membrane properties, and its time-constant (tau(SD) or chronaxie) predominantly depends on non-voltage-gated, rest Na(+) inward conductances. We assessed the strength-duration curve of ulnar motor axons in 22 nerves of ...

Quantitative cardiovascular autonomic function tests (AFTs) and sympathetic skin response (SSR) were performed in 12 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Parasympathetic autonomic function was examined by Valsalva ratio and R-R interval variation during rest and deep breathing. Sympathetic autonomic function was evaluated by blood pressure responses to sustained ...

OBJECTIVES: Several serum antibodies against gangliosides are diagnostically important, particularly in Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS), and multifocal motor neuropathy (MMN). Although hyperreflexia is an atypical symptom in these disorders, it has been found in some patients with GBS, MFS, and MMN. The aim of the study was ...

BACKGROUND: Small brainstem infarctions are increasingly recognized as a cause of isolated ocular motor and vestibular nerve palsies in diabetic and/or hypertensive patients. This raises the question whether there are also isolated 7(th) nerve palsies due to pontine infarctions in patients with such risk factors for the development of cerebrovascular ...

The authors evaluated epidermal nerve density (END) and thermal thresholds in 18 patients with chronic inflammatory demyelinating polyneuropathy (CIDP). END of patients with CIDP were lower than those of controls (4.5 +/- 2.9 vs 10.5 +/- 3.9 fibers/mm, p < 0.001). Reduced END were associated with autonomic symptoms. Thermal thresholds ...

OBJECTIVE: To review the causes and prognosis of sixth nerve palsies in patients who are 20 to 50 years of age. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: All patients aged 20 to 50 years with a nontraumatic sixth nerve palsy seen in a neuro-ophthalmic practice from 1994 to 2000. INTERVENTION: ...

X-linked Charcot-Marie-Tooth disease (CMTX) is a hereditary demyelinating neuropathy caused by mutations in the connexin 32 (Cx32) gene. Cx32 is widely expressed in brain and peripheral nerve, yet clinical manifestations of CMTX mainly arise from peripheral neuropathy. We have evaluated two male patients with CMTX who on separate occasions developed ...

To improve sensitivity of the analysis of axon reflex flare reaction, the authors used a laser Doppler scanner and analyzed flare intensity and size induced by histamine iontophoresis simultaneously at the foot and thigh in patients with small-fiber neuropathy (n = 10) and controls (n = 9). Flare size, but ...

A crossed-design experimental study has been made involving simple blind paired data and random assignment to treatment, with the aim of evaluating the action of an occlusal splint with transcutaneous electric nerve stimulation (TENS) upon the manifestations of temporomandibular disorders (TMD) in patients with bruxism. The prevalence of TMD in ...