Guillain-Barrè syndrome and inadequate antidiuretic hormone secretion syndrome

Several neurologic disorders including Guillain-Barré syndrome (GBS) are associated with hyponatremia. Hyponatremia and its overly fast correction have major implications to the course of the underlying neurologic disease. We report a case of GBS complicated by hyponatremia secondary to the development of inadequate antidiuretic hormone secretion syndrome. Differential diagnosis, pathophysiology, and therapeutic approach of hyponatremia in association with GBS are discussed.     

Sarcoidosis and Gullain-Barré syndrome

A 35-year-old female presented with three days' history of aching discomfort in her back, chest, and ankles. She had also noticed increasing weakness of her legs and a week before admission had shown flu-like symptoms. Chest X-ray showed bilateral hilar and right paratracheal lymphadenopathy. Bronchoscopic biopsies revealed non-caseating granuloma. She was diagnosed with sarcoidosis and was given prednisolone. The patient developed facial palsy and rapidly progressive ascending paralysis beginning from the lower extremities on the third and fourth days after initial presentation, respectively. Analysis of lumbar puncture showed acellular fluid with a high protein content. EMG was consistent with diffuse sensorimotor demyelinating polyneuropathy. Thus, the diagnosis was Guillain-Barré syndrome (GBS) presenting with sarcoidosis. Intravenous immune globulin was given and prednisolone stopped. One month after initial presentation the patient was completely recovered and discharged on prednisolone therapy. If neurologic symptoms such as aching discomfort and weakness are the main complaints in patients with suspected or biopsy proven sarcoidosis, GBS should be suspected.     

Guillain-Barré syndrome and its treatment

Guillain-Barré syndrome typically presents with an acute ascending areflexic weakness, progressing over 4 weeks or less. The most common form of the disease is an acute inflammatory demyelinating polyneuropathy, but other forms with primarily axonal pathologies are well documented. The association of Guillain-Barré syndrome with a range of antecedent infections, particularly Campylobacter jejuni enteritis, is also established. A range of serological and neurophysiological investigations can assist in making an accurate diagnosis. Background information about the syndrome and the evidence base for such treatments are discussed herein.     

Parry-Romberg syndrome and Rasmussen syndrome: only an incidental similarity?

The Parry-Romberg syndrome is a rare and poorly understood disease characterized by slowly progressive, localized atrophy of the skin, subcutaneous tissue, muscles, and bones. The atrophy is typically localized in the face and begins in youth. In some patients, imaging can show the lesions and atrophy of the ipsilateral hemisphere of the brain. We report on a patient in whom the disease has lasted 36 years and discuss the possibility that the Parry-Romberg syndrome is related to known autoimmune disorders of the soft tissue (e.g., linear scleroderma) and Rasmussen's syndrome. There are some remarkable clinical similarities between these two syndromes, including age of onset, unilateral manifestation, and occurrence of focal seizures. It is most probable that both diseases have an autoimmunological background.     

Carpal tunnel syndrome—course and prognosis

Summary In a retrospective study 157 patients with electrophysiologically proven carpal tunnel syndrome (CTS) were followed up. Of the 85 operatively treated patients, 86% showed clear improvement or cure, as did 32% of the patients not operated upon. Multivariate analysis of variance (MANOVA) and linear discriminant analyses indicated atrophy and length of history as effective predictors of clear improvement. The optimized discriminant point was ascertained in the receiver operator characteristics (ROC) curve. A predictive value of positive test of 0.89 was found for the course form not cured and a predictive value of negative test of 0.75 for the course form cured with a prevalence of one-third for the good course form. The prognosis was bad if atrophy occurred or the history exceeded 7 months. The determination of distal motor latency is indispensable for diagnosing CTS. For prognosis, however, length of history and clinical findings have been more helpful.

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Zusammenfassung In einer retrospektiven Studie konnten 157 Patienten mit einem elektrophysiologisch gesicherten CTS nachuntersucht werden. Bei 86% der 85 operativ behandleten Patienten war eine deutliche Besserung oder Heilung eingetreten. Aber auch bei 32% der nicht operierten Patienten war eine deutliche Besserung bzw. Heilung zu verzeichnen. Die multivariate Varianz- und lineare Diskriminanzanalyse bei 68 nicht operierten Patienten zeigten Atrophie und Anamnesedauer als geeignete Prädiktoren für eine deutliche Besserung. Der optimierte Diskriminanzpunkt wurde in der ROC-Kurve ermittelt. Dabei ergab sich ein predictive value of positive test von 0,89 für die Verlaufsform nicht geheilt und ein predictive value of negative test von 0,75 für die Verlaufsform geheilt bei einer Praevalenz von einem Drittel für die gute Verlaufsform. Die Prognose ist schlecht bei auftretenden Atrophien oder einer Anamnesedauer von über 7 Monaten. Die Bestimmung der distalen motorischen Latenz ist für die Diagnose eines CTS unerläßlich. Für prognostische Aussagen sind jedoch die Dauer der Anamnese und die klinischen Befunde bedeutungsvoller.

     

Treatment of Guillain-Barré syndrome and CIDP

Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating poly-(radiculo)neuropathy (CIDP) are immune-mediated disorders with a variable duration of progression and a range in severity of weakness. Infections can trigger GBS and exacerbate CIDP. Anti-ganglioside antibodies are important, but there is debate on the role of genetic factors in the pathogenesis of these disorders. Randomized controlled trials (RCT) have shown that intravenous immunoglobulin (IVIg) and plasma exchange (PE) are effective in both GBS and CIDP. Most CIDP patients also improve after steroid therapy. Despite current treatment options, many patients have residual deficits or need to be treated for a long period of time. Therefore, new treatment trials are highly indicated. This review focuses on the current and possible new treatment options that could be guided by recent results from laboratory experiments.     

Entrapment neuropathies and polyneuropathies in joint hypermobility syndrome/Ehlers–Danlos syndrome

ObjectiveThis study aims to investigate the involvement of the peripheral nervous system in Ehlers–Danlos syndromes/hypermobility type patients with particular attention to entrapment syndromes.MethodsWe consecutively enrolled Ehlers–Danlos syndromes/hypermobility type patients. Patients underwent clinical, neurophysiological and ultrasound evaluations. Dynamic ultrasound evaluation was also performed in healthy subjects as control group.ResultsFifteen Ehlers–Danlos syndromes/hypermobility type patients and fifteen healthy subjects were enrolled. Most of patients presented tingling, numbness, cramps in their hands or feet. Clinical evaluation was normal in all patients. One patient was affected with carpal tunnel syndrome and one with ulnar nerve entrapment at elbow. One patient had an increased and hypoechoic ulnar nerve at elbow at ultrasound evaluation. Dynamic ultrasound evaluation of ulnar nerve at elbow showed, in patients, twelve subluxations and three luxations. In the control group dynamic evaluation showed one case of ulnar nerve luxation.ConclusionStatistical analysis showed a significant difference in the occurrence of ulnar nerve subluxation and luxation between patients and control subjects.SignificanceThe study shows an inconsistency between symptoms and neurophysiological and ultrasound evidences of focal or diffuse nerve involvement. The high prevalence of ulnar nerve subluxation/luxation at elbow in Ehlers–Danlos syndromes/hypermobility type patients could be explained by the presence of Osborne ligament laxity.     

Multiple pterygium syndrome, bilateral periventricular nodular heterotopia and epileptic seizures--a syndrome?

We present the clinical, neurophysiological and radiographic findings in a boy with coexisting multiple pterygium syndrome, bilateral periventricular nodular heterotopia (BPNH), mental retardation and epileptic seizures. This constellation has not been previously reported. We discuss the possibility of a new BPNH syndrome associated with multiple pterygium syndrome in our patient.     

Horner’s syndrome,Pseudo-Horner’s syndrome,and simple anisocoria

This discussion reviews the common causes of Horner’s syndrome, with emphasis on case reports from the past several years. Much of the recent literature concerns the use of apraclonidine as a diagnostic test for Horner’s syndrome, possibly as an alternative for the current gold standard of cocaine eye drops. This new literature is discussed in the context of the current standards for clinical diagnosis.     

Guillain-Barré syndrome and serum activities of gamma-Glutamyltransferase and glutamic-pyruvic transaminase

Summary Objective laboratory indicators of alcohol consumption (mean corpuscular volume and serum glutamic-pyruvic transaminase (GPT), glutamyltransferase (-GT), and glutamic-oxaloacetic transaminase (GOT)) were measured in 18 patients with Guillain-Barré syndrome (GBS) and 710 control patients. All of the indicators examined were more frequently found to be pathological in GBS patients, reaching significance for -GT and GPT. Some explanations for this result are discussed. It is concluded that alcohol consumption may be a risk factor for GBS.     

Acute axonal polyneuropathy and Gougerot-Sjögren syndrome

IntroductionGougerot-Sjögren syndrome (GSS) is an autoimmune disease characterized by a lymphocytic infiltration and destruction of saliva and lachrymal glands. About 20% of patients develop a neurological involvement.Case reportA 29-year-old woman was admitted with a dysautonomic and sensorial neuropathy. Clinical manifestations (dry syndrome and positive Schirmer's test), blood (anti-SSA and anti-SSB antibodies) and histological (chronic sialadenitis) tests linked this neuropathy to a primary GSS. The characteristics of this case were acute onset (within a few days), severe clinical presentation (continued confinement to bed and malnutrition) and non-responsiveness to all treatments (intravenous immunoglobulins, corticosteroids, plasmapheresis, rituximab).ConclusionThis case illustrates various clinical signs and inconstant responsiveness to treatment of neuropathy associated with primary GSS.     

Cranial pachymeningitis and primary Sjögren's syndrome

Introduction

Central neurological manifestations of primary Sjogren's syndrome are very polymorphic, including pachymeningitis, one of the rarest manifestations, which is exceptionally inaugural.

Clinical case

A 50-year-old patient was admitted for an intracranial hypertension syndrome with paralysis of the III and VII cranial nerves. Brain MRI revealed pachymeningitis of the right cerebral hemisphere. History taking revealed the existence of xerostomy and xerophthalmos. The accessory salivary gland biopsy demonstrated Chisholm stage III sialadenitis. Search for anti-SSA antibodies and anti-SSB antibodies was positive. The diagnosis of primary Sjogren's syndrome was retained. The patient improved with corticosteroid therapy and cyclophosphamide.

Conclusion

This case illustrates the unusual observation of pachymeningitis in primary Sjogren's syndrome in the rare setting as an inaugural manifestation.