Horner''s syndrome associated with Guillain-Barré syndrome

Autonomic dysfunctions are classically described during the acute phase of Guillain-Barré syndrome (GBS). This involvement concerns visceral afferent, parasympathetic and/or sympathetic efferent fibers and is closely related to sudden deaths in the acute phase of the disease. We report a case of a patient suffering from a GBS associated with a transient Horner syndrome without extraocular muscle involvement. Pharmacological tests of the pupils pointed to an orthosympathetic pre-ganglionic disturbance. Neither clinical nor electrophysiological parameter suggested a broader involvement of the autonomic nervous system.     

Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is an acute-onset, monophasic, immune-mediated polyneuropathy that often follows an antecedent infection. The diagnosis relies heavily on the clinical impression obtained from the history and examination, although cerebrospinal fluid analysis and electrodiagnostic testing usually provide evidence supportive of the diagnosis. The clinician must also be familiar with mimics and variants to promptly and efficiently reach an accurate diagnosis. Intravenous immunoglobulin and plasma exchange are efficacious treatments. Supportive care during and following hospitalization is also crucial.     

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.     

Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is currently divided into the two major subtypes: acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). This review highlights relevant recent publications, particularly on the pathophysiology of AMAN. Molecular mimicry of the bacterial lipo-oligosaccharide by the human gangliosides is now considered an important cause of AMAN. Gangliosides GM1, GM1b, GD1a, and GalNAc-GD1a expressed on the motor axolemma are likely to be the epitopes for antibodies in AMAN. At the nodes or paranodes, deposition of antiganglioside antibodies initially cause reversible conduction block followed by axonal degeneration. Electrodiagnostic findings support this process. Disruption of glycolipids, which are important to maintain ion channel clustering at the nodes and paranode, may impair nerve conduction. Genetic polymorphisms of Campylobacter jejuni determine the expression of the gangliosides on the bacterial wall. In contrast, target molecules in AIDP have not yet been identified. Meta-analyses show efficacy of plasmapheresis and immunoglobulin therapy, but not corticosteroids, in hastening recovery.     

Guillain-Barré syndrome as the first manifestation of POEMS syndrome

POEMS syndrome is a rare multisystem disorder, characterized by the presence of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. The variety of clinical pictures and asynchronous manifestation of dominant features make diagnosis difficult. We report a case of a 42-year-old man with polyneuropathy who was initially negative for monoclonal protein and so Guillain-Barré syndrome was diagnosed. Other signs and symptoms, including monoclonal gammopathy, developed later in the course of the disease and finally POEMS syndrome was diagnosed.     

Guillain-Barré syndrome in a patient with primary sicca syndrome

At the age of 23 the patient showed the first signs of dryness syndrome. Those symptoms developed progressively and during a few years primary Sj?gren syndrome was noted. In the 37th year of life suddenly the patient developed very severe Gullian-Barré syndrome with involvement of the peripheral and central nervous system and with a considerable autonomic component. After treatment the patient improved, however mild symptoms of central and peripheral nervous system destruction remained. Those symptoms are still present and the patient is under the care of the Neurology and Rheumatology Clinic.     

Dopa responsive burning mouth syndrome: Restless mouth syndrome or oral variant of restless legs syndrome?

Burning mouth syndrome (BMS) is an oral dysesthesia presenting as a burning sensation of the tongue and other oral and perioral mucosae. A painful symptomatology in different bodily regions (extraoral) may also be a common feature in patient with BMS. The management of BMS is challenging and there is no clear guideline for the management of idiopathic BMS. Herein, we describe a group of patients (5 patients) in whom symptoms of BMS responded to levodopa. In parallel, four patients fulfilled the criteria for restless legs syndrome (RLS). Family history of RLS was positive in two patients. We reviewed the literature and noted a marked overlap between BMS and RLS. Overlaps were noted in epidemiological profiles, pattern of clinical features and even in neurophysiological observations (alterations in the striatal dopaminergic system). We suggest that a subset of patients with BMS may be a phenotypic variant of RLS and a trial of dopaminergic drugs should be given in patients with BMS who has a history suggestive of RLS or in a patient who do not show a response to usual therapies for BMS.     

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.     

Fatal Guillain-Barré syndrome

Fourteen of 320 patients (4%) admitted with Guillain-Barré syndrome (GBS) died as a direct result of the illness. Deaths most commonly resulted from ventilator-associated pneumonia. In comparison with 101 other patients with severe GBS admitted to the intensive care unit, the patients who died were older (p = 0.006) and more likely to have underlying pulmonary disease (p = 0.004). In a specialized center, the primary event leading to death in GBS was ventilator-associated pulmonary infection, predominantly in elderly patients with significant comorbidity.     

Meningitis–retention syndrome

BACKGROUND: A combination of acute urinary retention and aseptic meningitis has not been well known. This combination can be referred to as meningitis-retention syndrome (MRS), when accompanied by no other abnormalities. OBJECTIVE: To describe the results of a uro-neurological assessment in our patients with MRS. METHODS: In three patients (two men, one woman; age, 34-68 years), we performed urodynamic studies and relevant imaging and neurophysiological tests, in addition to cerebrospinal fluid (CSF) examination. RESULTS: All three patients developed acute urinary retention along with headache, fever and stiff neck.None had obvious neurological abnormalities, other than a slightly brisk reflex in the lower extremities. One had previously experienced generalized erythematous eruptions, but none had pain, hypalgesia or skin eruptions in the sacral dermatomes suggestive of Elsberg syndrome (infectious sacral polyradiculitis; mostly genital herpes). Brain/spinal/lumbar plexus MRI scans and nerve conduction studies were normal. CSF examination showed mild mononuclear pleocytosis, increased protein content, and normal to mildly decreased glucose content in all patients; increased myelin basic protein suggestive of central nervous system demyelination in one; and increased viral titers in none.Urodynamic study revealed, during the voiding phase, an underactive detrusor in all patients and an unrelaxing sphincter in one. These clinical manifestations were ameliorated within 3 weeks. CONCLUSIONS: We reported three cases of MRS, a peculiar syndrome that could be regarded as a mild variant of acute disseminated encephalomyelitis (ADEM). Urinary retention might reflect acute shock phase of this disorder. Although MRS has a benign and self-remitting course, management of the acute urinary retention is necessary.     

Is the slit ventricle syndrome always a slit ventricle syndrome?

The term slit ventricle syndrome (SVS) refers to the occurrence of headache, vomiting, and possibly some degree of consciousness impairment in shunted hydrocephalic children in whom slit-like ventricles are seen on computerized tomography (CT) scan examination. The syndrome has been typically observed in older hydrocephalic children operated on in early infancy, even though it has also been observed occasionally in young children and adults. The clinical characteristics of the SVS, the results of the laboratory investigations and the therapeutic measures utilized in the various cases described in the literature differ so widely as to suggest that different clinical conditions are actually being described. In some cases, the diagnosis of SVS should be avoided and substituted by a correct diagnosis of CSF shunt malfunction or of disturbances unrelated to the CSF shunting therapy, e.g., childhood migraine.Presented at the Consensus Conference: Hydrocephalus '92, Assisi, Italy, 26–30 April 1992     

HIV-associated Guillain-Barré syndrome

Human immunodeficiency virus-associated Guillain-Barré syndrome (HIV-GBS) has been reported since 1985. Based on previous reports, this neuropathy typically occurs early in HIV infection, even at seroconversion, prior to developing acquired immunodeficiency syndrome (AIDS). Patients with GBS and CD4 counts of <50 have been proposed to have cytomegalovirus (CMV) infection and empiric gancyclovir is recommended. We reviewed medical records of 10 patients with HIV-GBS at five hospitals from 1986 to 1999. The mean CD4 count was 367/mm(3) (range 55-800). GBS was the first symptom of HIV infection in three patients. Four patients had AIDS with CD4 counts ranging from 55 to 190. CSF white blood cell (WBC) was 0 wbc/mm(3) in four patients, 2-10 wbc/mm(3) in three and 11-17 wbc/mm(3) in two. Three had recurrent weakness from 9 weeks to 4 years after the onset of symptoms, which persisted. HIV-GBS occurs in early and late stages of HIV infection, and may follow the onset of AIDS. No patients were seen with severe immunosuppression (CD4<50). A mild cerebrospinal fluid (CSF) pleocytosis in GBS suggests HIV infection, but is frequently absent. Compared to HIV-negative people, HIV-GBS may be associated with more frequent recurrent episodes or the development of CIDP.     

Posterior reversible encephalopathy syndrome as a complication of Guillain-Barré syndrome

Posterior reversible encephalopathy syndrome (PRES) is characterised by sudden hypertension that is associated with headache, seizure, visual disturbance and altered mental function. We report a 62-year-old woman with a sudden onset of complete bilateral visual loss, an admission blood pressure (BP) of 204/113 mmHg, and flaccid weakness in all four limbs. The patient’s cerebrospinal fluid and nerve conduction studies were consistent with a diagnosis of Guillain-Barré syndrome (GBS) and her brain MRI showed classic features of PRES. Her blood pressure was managed appropriately and her GBS was treated with plasmapheresis. Two months following presentation, the patient’s vision recovered completely and she regained full power of her four limbs. She did not need any continuing antihypertensive medication. This report is the 8th of accumulating evidence that links PRES with GBS, and it shows that PRES can be the presenting feature following GBS treatment with intravenous (IV) immunoglobulins or later in recovery. Dysautonomia resulting from GBS is the most likely explanation of this strong association. GBS is mostly reversible when managed correctly; however, the literature suggests that failure to recognize or delay treatment can lead to cerebral infarction or even death.     

Sensory Guillain-Barré syndrome

OBJECTIVE: To report eight cases of sensory Guillain-Barré syndrome (GBS). BACKGROUND: The concept of sensory equivalent to ascending paralysis of GBS was raised in 1958, and the diagnostic criteria for a sensory loss and areflexia variant of GBS were proposed in 1981. However, clinical cases meeting these criteria have been relatively scarce. METHODS: During a 13-year period between 1986 and 1999, the authors collected eight cases of an acute sensory demyelinating neuropathy that met most of the proposed diagnostic criteria of a sensory variant of GBS. RESULTS: In all patients, sensory neuropathy was sudden at onset and peaked to maximal deficit within 4 weeks. In five (63%) cases, there was an antecedent viral illness. All patients had objective sensory loss and diminished or absent reflexes. None showed any muscle weakness. In all four patients in whom the spinal fluid was examined during the first 4 weeks, there was albuminocytologic dissociation. All of the patients had electrophysiologic evidence of demyelination in at least two nerves. Demyelination was demonstrated in motor nerve conduction in seven patients and in sensory nerve conduction in one, indicating that motor nerve conduction studies were the key for the diagnosis of demyelinating neuropathy. All patients had sensory nerve conduction abnormalities in at least one nerve. Three patients responded to immunotherapies. All had a favorable outcome, with a monophasic course of disease and no sign of relapse. CONCLUSION: The current study confirms the existence of sensory GBS.     

Aicardi-Goutières syndrome

Aicardi-Goutieres syndrome is a familial progressive early onset encephalopathy with basal ganglia calcifications, chronic CSF lymphocytosis and high level of interferon-alpha in CSF. Cutaneous necrotic lesions and the neuropathological aspect of microangiopathy and microinfarctions suggest a vascular process in relation to elevated interferon-alpha. A genetic defect in the regulation of its synthesis may be the causal factor of the disorder.