Acute sensorimotor polyneuropathy that resembles Guillain-Barré syndrome (GBS) is rarely accompanied with nephrotic syndrome, and its underlying immunological mechanisms are unclear. A 56-year-old man presented with simultaneous acute progressive symmetric sensorimotor polyneuropathy and proteinuria. A kidney biopsy revealed focal segmental glomerulosclerosis. Serial electrophysiologic studies showed only a transient proximal conduction ...

Patients with chronic itch are diagnosed and treated by dermatologists. However, itch is a neural sensation and some forms of chronic itch are the presenting symptoms of neurological diseases. Dermatologists need some familiarity with the most common neuropathic itch syndromes to initiate diagnostic testing and to know when to refer ...

Sjögren's syndrome is a systemic autoimmune disease that, apart from exocrine glands, may affect every organ or system. Involvement of different sections of the peripheral nervous system results in a wide spectrum of neuropathic manifestations. Based on distinct clinical, electrophysiological and histological criteria, the types of neuropathies seen in Sjögren's ...

Anti-SRP (signal recognition particle) positive necrotizing myopathy is commonly not associated with neoplasms. We demonstrate two histologically confirmed cases with unusual manifestations of anti-SRP positive necrotizing myopathy. A 65-year-old man presented with rapidly progressing weakness and mild difficulties in swallowing and speaking. Screening for underlying disorders revealed a moderately differentiated ...

In this case report, we describe a case of adult-onset bulbar ptosis in a patient with Joubert syndrome. Joubert syndrome is a rare neurodevelopmental disorder with malformations in cerebellum and brainstem. Many ocular abnormalities have been noted in Joubert syndrome, but the association of this syndrome with adult-onset ptosis has ...

Sweet's syndrome is a rare cutaneous disease of unknown etiology. About 20% of the cases are associated with hematological neoplasms, and cases related with Hodgkin's disease are rare. We present the case of a 57-year old male patient who developed the syndrome concomitantly with the neoplasm. The diseases were controlled ...

To highlight an unexpected clinical presentation and to review the associated polyneuropathy phenotypes of SCA3. Clinical follow-up. Neurological referral center. Patient  Middle-aged man with no family history for SCA3. Presentation with an isolated axonal, distal, symmetric, sensorimotor polyneuropathy for 6 years before developing a cerebellar syndrome prompting genetic testing for ...

Patients with Bertolotti's syndrome have characteristic lumbosacral anomalies and often have severe sciatica. We describe a patient with this syndrome in whom standard decompression of the affected nerve root failed, but endoscopic lumbosacral extraforaminal decompression relieved the symptoms. We suggest that the intractable sciatica in this syndrome could arise from ...

Cubital tunnel syndrome (CuTS) is the second most common peripheral nerve compression syndrome. In German-speaking countries, cubital tunnel syndrome is often referred to as sulcus ulnaris syndrome (retrocondylar groove syndrome). This term is anatomically incorrect, since the site of compression comprises not only the retrocondylar groove but the cubital tunnel, ...

A 64-year-old woman, with asthma and sinusal polyposis in her history, suddenly developed a painful polyneuropathy with diplopia. Nerve conduction studies, performed at the very onset of the neuropathy, could not definitely rule out a Guillain-Barré syndrome (GBS) and high-dose i.v. immunoglobulins were administered. Clinical and laboratory findings subsequently supported ...

Septo-optic dysplasia (SOD), also called De Morsier's syndrome, is a highly heterogeneous condition comprising a spectrum of central nervous system malformations that involves in various degrees the optic nerves, the hypothalamic-pituitary axis, and other midline structures such as the septum pellucidum and the corpus callosum. In a discrete number of ...

A 63-year-old male with Wolff-Parkinson-White syndrome was admitted for ablation of accessory pathway. Intracardiac electrogram revealed a left-side accessory pathway during tachycardia, which was successfully ablated from the right posterior tricuspid annulus because of a long slanted transseptal accessory pathway (2.2 cm).

Abstract We retrospectively screened a large cohort of 554 ALS patients with regard to documented nerve compression syndromes and identified 23 patients, mostly with carpal tunnel syndrome. Patients could be subdivided into three groups. Group A comprised 13 patients in whom nerve compression was apparently confused with early ALS signs. ...

Antiganglioside GQ1b antibodies mediate a continuum of disorders with overlapping features, fostering the concept of anti-GQ1b antibody syndrome. We present a patient whose palatal insufficiency was the only clinical sign of postinfectious GQ1b antibody syndrome. Cerebral magnetic resonance imaging confirmed involvement of the glossopharyngeal nerve and vagus nerve bilaterally, revealing ...

We describe two familial and three nonfamilial cases from Argentina, examined between February 1, 1990-July 31, 2008, who met the diagnostic criteria of progressive encephalopathy, peripheral edema, hypsarrhythmia, and optic atrophy syndrome. All five children were products of normal gestation, although one was premature. Birth was uneventful in all patients. ...

PURPOSE: Frey syndrome (profuse sweating and cutaneous flushing in the area innervated by the auriculotemporal nerve) is a frequent and unpleasant complication of parotidectomy. Fat injections may be useful in preventing the abnormal nerve neo-anastomoses sprouting to the sweat glands that are responsible for gustatory sweating, but have never been ...

Dourado ME, de Miranda Félix RH, da Silva WKA, Queiroz JW, Jeronimo SMB. Clinical characteristics of Guillain-Barré syndrome in a tropical country: a Brazilian experience. Acta Neurol Scand: DOI: 10.1111/j.1600-0404.2011.01503.x. © 2011 John Wiley & Sons A/S. Objective -  To analyze the clinical variants, outcomes, and prognosis of Guillain-Barré syndrome (GBS) ...

We report the case of a 23-year-old male presenting with carpal tunnel syndrome and a swelling over the flexor surface of the wrist. MRI findings were initially suggestive of a median nerve schwannoma but sonography (US) showed a heterogenous mass infiltrating the flexor tendons of the fingers and displacing the ...

Mitochondrial disorders (MIDs) occasionally manifest as polyneuropathy either as the dominant feature or as one of many other manifestations (inherited mitochondrial neuropathy). MIDs in which polyneuropathy is the dominant feature, include NARP syndrome due to the transition m.8993T>, CMT2A due to MFN2 mutations, CMT2K and CMT4A due to GDAP1 mutations, ...

A 67-year-old woman developed hypertension, drowsiness, hemianopia, ascending flaccid tetraparesis, and areflexia. Nerve conduction studies revealed a demyelinating polyneuropathy. Brain magnetic resonance imaging demonstrated hyperintense white matter lesions. IgM antibodies against West Nile virus were positive. She was treated for hypertension and with intravenous immunoglobulins and recovered completely within 2 ...

Ramsay Hunt syndrome, first described by J. Ramsay Hunt in 1907, encompassed the symptoms of otalgia, erythematous vesicular rash on the auricle, and facial paralysis. Although rare, in some cases, the varicella zoster virus responsible for the illness can also be associated with involvement of cranial nerves III-XII, cervical nerves, ...

The creation of a vascular access for hemodialysis is a frequently performed procedure. Ischemic monomelic neuropathy (IMN) is a rare, but important complication of hemodialysis access (HA) procedures, which can lead to severe and nonreversible limb dysfunctions. Therefore, in any case of postoperative neurological malfunction, immediate neurological investigations should be ...

We report 2 patients diagnosed simultaneously with an overlap of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS), who had anti-GT1a, anti-GQ1b, anti-GD1a and anti-GD1b antibodies. There was no identifiable specific preceding infection. Both patients presented with upper and lower limb paresthesias and severe weakness, bulbar and facial weakness, ophthalmoparesis ...

Klippel-Trenaunay syndrome (KTS) consists of a vascular nevus involving an extremity, varicosities of that extremity, and hypertrophy of bone and soft tissue. When arteriovenous malformation is also present, it is called Klippel-Trenaunay-Weber syndrome (KTWS). Ophthalmic features of these syndromes include vascular anomalies of the orbit, iris, retina, choroid, and optic ...

Horner syndrome, in which ptosis, miosis, and anhidrosis occur concomitantly, can arise from injury to the sympathetic nerve pathways anywhere from the brain to the end organs. Incomplete Horner syndrome lacks the sign of anhidrosis. We present a case of incomplete Horner syndrome caused by internal carotid artery dissection and ...

Hemicrania continua (HC) is a rare primary headache syndrome, characterized by unilateral pain and an absolute response to indometacin. Since the term was first coined in 1984, more than 100 cases have been described worldwide. Most recently, detailed case series that provide more detailed information concerning the sometimes complex clinical ...

Background Ataxic Guillain-Barré syndrome is characterised by profound ataxia with negative Romberg sign and no ophthalmoplegia. Its nosological relationship to acute sensory ataxic neuropathy has yet to be discussed. Methods Medical records were reviewed of patients suffering acute ataxia and reduced muscle stretch reflexes but without external ophthalmoplegia. Clinical features ...

A 7-year-old male miniature schnauzer dog with unilateral cryptorchidism was presented for elective orchiectomy. Surgery to remove the cryptorchid testis revealed a fully formed uterus with horns attached to both testis and the body and cervix terminating at the prostate gland. The gross and microscopic diagnosis for the genital tract ...

Numb chin syndrome (NCS), also known as mental nerve neuropathy, is characterized by facial and oral numbness restricted to the distribution of the mental nerve. Although not a common neuropathy, the clinical importance of this syndrome is its frequent association with malignancies, particularly breast cancer and lymphoma. In this paper, ...

We report the case of an 11-month-old girl with congenital axonal neuropathy and West syndrome. She had generalized hypotonia and an abnormal posture since birth, and apparently, her development was stalled. Deep tendon reflexes were absent, and at 5months of age, she developed West syndrome followed by refractory seizures. Magnetic ...

The cubital tunnel syndrome is widely considered as the second most frequent compression neuropathy in the upper extremities although the existence of a compressive cause has not been determined conclusively. As far as we know, operational photography of compression of the ulnar nerve at the elbow is almost never found ...

Objective: The purpose of this study was to investigate the clinical, electrophysiological and pathological features of Churg Strauss syndrome (CSS) neuropathy. Methods: Biopsies were selected from over 700 sural nerve biopsies. The diagnosis of vasculitis was based on established clinicopathological criteria. Complete laboratory, clinical, electrophysiological and pathological studies were performed ...

Möbius syndrome (OMIM#157900) is an extremely rare congenital entity involving bilateral or unilateral palsy of the facial nerve, usually with dysfunction of other cranial nerves (second, third, fifth, sixth, ninth, tenth and twelfth). It was estimated that Möbius syndrome occurs in 1 of 50 000 live births. The aetiology and ...

Subcutaneous occipital nerve region stimulation is becoming an important part of the overall treatment regimen for a number of chronic headache syndromes refractory to nonsurgical, medical management. A combination of improved device technology and methodology, further understanding about appropriate indications and achievement of on-label FDA status should support continued use ...

The glomerular filtration barrier is affected in a large number of acquired and inherited diseases resulting in extensive leakage of plasma albumin and larger proteins, leading to nephrotic syndrome and end-stage renal disease. Unfortunately, the molecular mechanisms governing the development of the nephrotic syndrome remain poorly understood. Here, I give ...

We report on a previously healthy 11-year-old boy with unilateral periorbital mild headache and facial nerve palsy, followed during the next 5 months by recurrent unilateral headaches and subsequent extrinsic paresis of the third cranial nerve and paresis of the sixth cranial nerve, each of which improved with steroids. Cranial ...

The aim of study was to assess the usefulness of 3D ultrasonography (3DUS) in the diagnosis of carpal tunnel syndrome. Fifty patients with carpal tunnel syndrome confirmed by electromyography and 37 healthy control participants underwent 3DUS of the wrists. The mean times per participant for the 3DUS examination and review ...

there are several variants of Wallenberg's syndrome. Wallenberg's syndrome with proximal quadriparesis has not been reported before. we report a 25-year-old woman presenting with sudden onset of vertigo, hoarseness, dysphagia, right facial numbness, and proximal quadriparesis. Brain magnetic resonance imaging revealed right posterolateral medullary infarction with medial extension and sparing ...

First described in the past decade, schwannomatosis is a syndrome distinct from neurofibromatosis 2 (NF2). It is characterized by the development of multiple schwannomas, sparing the vestibular division of cranial nerve VIII, and may also predispose to develop meningiomas. We report two female patients, a 27 and a 44 years old ...

Background The prevalence of peripheral neuropathy in patients with Sjögren syndrome remains unclear owing to conflicting results in the published series, with numbers ranging from 2% to over 60% of Sjögren syndrome patients. Whether peripheral neuropathy is a feature of the systemic or glandular disease or whether it is related ...

Hemichorea-hemiballismus is a rare complication of nonketotic hyperglycaemia in type 2 diabetes. It is associated with contralateral striatal radiological abnormality, most typically T1 hyperintensity on MRI. We report a case of a 91 year old woman who presented in a nonketotic hyperglycaemic state with right sided hemichorea. Brain CT revealed ...

BACKGROUND: Auriculotemporal nerve syndrome is characterised by recurrent episodes of facial gustatory flushing and/or sweating along the cutaneous distribution of the auriculotemporal nerve. The condition is rare in children and is normally a sequel of perinatal birth trauma. We report a case of a sixteen-month-old boy referred by paediatric oncology ...

Summary: Foster-Kennedy syndrome was described in 1911 as an ophthalmologic manifestation of compression by a solid tumor in the frontal area with intracranial hypertension (ICHT). We describe a peculiar case of Foster-Kennedy syndrome associated with an arteriovenous malformation in which neither optic nerve compression nor ICHT was obvious. We discuss ...

Ghosal hematodiaphyseal dysplasia syndrome (GHDD) is a rare clinical syndrome characterized by increased bone density and a severe, myelophthisic anemia. Few cases have been reported worldwide and there are no detailed descriptions of the associated hematologic abnormalities and long-term clinical outcomes after treatment. Here, we report two siblings with GHDD ...

Neuromyelitis optica (NMO, Devic's syndrome) is a demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. It is often confused with multiple sclerosis. Early discrimination between NMO and multiple sclerosis is important because the two diseases have different natural histories and treatment regimens. ...

There are many reports of the use of free fibular flap for mandible reconstruction, but donor site morbidity is not uncommon. The authors present the case of a 52-year-old man who underwent mandible reconstruction with free fibular flap. After surgery, he developed severe compartment syndrome involving all four-calf compartments. Debridement ...

A 52-year-old woman has been under observation for a complete locked-in syndrome of vascular origin, since 1984. Her cognitive functions today are still normal. When first diagnosed, a CT-scan was made and 23 years later performed, a cerebral MRI was performed. A focal, bilateral and symmetric atrophy of the dorsomedial prefrontal ...

The McCune-Albright syndrome was described as a syndrome of polyostotic fibrous dysplasia, café au lait skin pigmentation, and autonomous endocrine hyperfunction in 1937. We report a 17-year girl with early menarche and accelerated growth for the past three years. The endocrinological examination showed slight rise of growth hormone with other ...

Guillain-Barré syndrome (GBS) is an acquired acute autoimmune polyradiculoneuropathy. The 2 features considered essential for the diagnosis of GBS are progressive motor weakness and areflexia. There have been several descriptions of reflex preservation and hyperreflexia in axonal variant of GBS in Chinese, Japanese, and European populations but it is not ...

Ankyloblepharon-ectodermal dysplasia-clefting (AEC) syndrome is a rare disorder characterized by ankyloblepharon (congenital adhesions of the eyelids), ectodermal dysplasia, and orofacial clefts. Here, we report the case of an infant born with severe ectodermal dysplasia including generalized neonatal erosions with scalp involvement, facial clefting but notably without ankyloblepharon. Mutational analysis of ...