Acute Pandysautonomia Research Articles

Varianty akutní polyradikuloneuritidy

Akutni polyradikuloneuritida (Guillainův a Barreho syndrom – GBS) je multifokalni autoimunitni forma neuropatie. Ma jednak sve klasicke formy – myelinovou pochvu postihuje akutni zanětliva demyelinizacni polyradikuloneuritida (AIDP) a primarně axonalni postiženi charakterizuje cistě motoricka forma AMAN a těžka smisena motoricko-senzitivni forma AMSAN. Mezi varianty ci fokalni formy se řadi Miller-Fisherův syndrom s klinickou triadou oftalmoplegie, ataxie a areflexie a faryngo-cerviko-brachialni forma s poruchami polykani, slabosti sijoveho a brachialniho svalstva. Kromě těchto dvou nejcastějsich variant se vyskytuji dalsi varianty – Bickerstaffova kmenova encefalitida, parapareticka forma GBS (Guillainův a Barreho syndrom), kranialni neuropatie, akutni pandysautonomie, cistě senzitivni forma GBS. Jednotlive varianty se vyznacuji charakteristickou klinickou symptomatikou, průběhem, abnormitami neurofyziologickeho vysetřeni, terapii.

Guillain Barre Syndrome and polyuria: A rare variant

A 33 year- old gentleman came with a progressive flaccid paraparesis, patchy hyperhidrosis and polyuria following an acute gastro enteritis, ultimately diagnosed as axonal variants-GBS associated with acute pandysautonomia. He was managed successfully with parenteral fluids and desmopressin nasal spray in addition to immunoglobulin.

Autoimmune autonomic ganglionopathy: Bench to Bedside

Autoimmune autonomic ganglionopathy (AAG) presents as severe generalized sympathetic and parasympathetic autonomic failure. This disorder is also known as acute pandysautonomia or idiopathic subacute autonomic neuropathy. Themost disablingmanifestations are orthostatic hypotension (OH) and gastrointestinal dysmotility. An autoimmune pathogenesis was suggested by the subacute onset, frequent associationwith antecedent viral illness, occasional association with cancer or other autoimmune disorders, and spontaneous recovery in some patients. Many cases have a rapid onset, but others have a chronic progressive course. Clinical and experimental observations demonstrate that AAG is an antibody-mediated disorder. (1) AAG patients have high levels of serum antibodies specific for the neuronal AChR in autonomic ganglia. This ganglionic AChR mediates fast synaptic transmission in all autonomic ganglia. Ganglionic AChR antibodies found in AAG patients are distinct from muscle AChR antibodies found in patients with MG. (2) Ganglionic AChR antibody levels correlate with severity of autonomic deficits. Patients with high antibody levels have a combination of OH, dry eyes and dry mouth, Adies pupil, gastroparesis, and neurogenic bladder. (3) Induction of ganglionic AChR antibodies in rabbits by immunization leads to experimental AAG which reproduces many of the cardinal features of the human disease. (4) Passive transfer of ganglionic AChR antibodies tomice causes transient autonomic failure. In electrophysiological studies, ganglionic AChR antibodies produce a reduction in ganglionic AChR current in cultured neuroblastoma cells and inhibit synaptic transmission in isolated autonomic ganglia. AAG is an antibody-mediated channelopathy that produces a severe, but treatable, form of autonomic failure.

Sudden Death in a Patient With Spinal Cystic Space-occupying Lesion Presenting as Elevated Cerebrospinal Fluid Protein

Elevated cerebrospinal fluid (CSF) protein concentration usually occurs in CSF of patients with meningitis and leptomeningeal carcinomatosis. We identify patients at risk of prolonged illness and those with a benign course. However, sudden death has been rarely reported. We report a case of sudden death presented as elevated CSF protein. Magnetic resonance imaging of the 61-year-old man spine revealed that L4, L5, and S1 of the spinal cord were compressed by the cystic space-occupying lesions. On admission day 2, the examination of CSF showed severe elevated CSF protein. Known causes of elevated CSF protein were carefully excluded before the planned operation. During the diagnostic period for elevated CSF protein, multiple organ dysfunction syndrome suddenly occurred and caused death on admission day 8. Acute pandysautonomia, which is not familiar to neurosurgeons, might explain the pathogenesis of this unusual evolution.

PP13.5 – 2902: Acute pandysautonomia case treated with intravenous immunoglobulin

Objective Acute pandysautonomia is a rarely seen acquired disorder with acute or subacute development of systemic sympathetic and parasympatheticautonomic dysfunction. Patients may suffer from orthostatic hypotension, bradycardia, anhidrosis, unreactive pupils, ptosis, decreased lacrimation and salivation, gastrointestinal paresis, and impaired genitourinary function. Methods Herein, we present a 15 year-old male patient who underwent laparotomy due to acute abdomen, and suffered from autonomic dysfunction postoperatively. Results The patient had no significant medical history except the laparotomy he underwent a month ago, due to acute appendicitis and ileus. Patient was consulted to neurology because of repetitive syncopes, occurring when he was trying to stand up, and ptosis. A urinary catheter was placed in order to treat globe vesical. Bradycardia (ongoing at sleep) was detected in physical examination, in addition to bilateral ptosis, and absence of deep tendon reflexes. His pupillary reflexes were normal. Blood count, chemical tests, and cardiac sonography didn't reveal any pathologic findings. Electroencephalography and cranial magnetic resonance were also normal. Acetylcholine receptor antibody result was negative, and cerebrospinal fluid protein level was 31.9 mg/dl. 400 mg/kg/day of intravenous immunoglobulin was administered for five days with the diagnosis of acute pandysautonomia. Deep tendon reflexes were reactive, and ptosis, bradycardia and syncopes vanished after treatment. Conclusion Acute pandysautonomia is a rare form of Guillain Barre Syndrome and it is usually seen after an infectious disease or a surgical trauma. Some of these cases may recover spontaneously in childhood. In more severe cases, corticosteroids and intravenous immunoglobulin are effective agents in treatment.

抗利尿ホルモン不適合分泌症候群 (SIADH) を伴った急性特発性汎自律神経失調症の1例

抗利尿ホルモン不適合分泌症候群 (SIADH) を伴った急性特発性汎自律神経失調症の1例

Acute pandysautonomia – restitutio ad integrum by high prednisolone therapy

Acute pandysautonomia is a rare disease defined as acute widespread and severe sympathetic and parasympathetic failure and sparing of somatic nerve fibers. The causes of this syndrome are often an autoimmune disease leading to autonomic ganglionopathy. The majority of cases have a poor prognosis with a chronic debilitating course. We present a previously healthy 24-year-old female patient, who developed a loss of accommodation, pupillotonia, lacrimation, swallowing disturbances, gastrointestinal symptoms and an atonic bladder with 750 ml residual volume. The Ewing battery showed signs of parasympathetic and sympathetic dysfunction leading to the diagnosis of acute pandysautonomia. Further tests failed to find a cause of acute neuropathy especially where there was no evidence for paraneoplastic or infectious etiology. The patient was treated with high dose intravenous prednisolone and completely recovered.

Profound bradycardia and hypotension in a normal heart

Bradycardia is a common arrhythmia that rarely can be caused by autonomic dysfunction. There are case reports of bradycardia attributed to diabetic autonomic neuropathy, 1 Horng H.C. Chen F.C. Kuo C.P. Wu C.T. Wong C.S. Bradycardia and hypotension refractory to ephedrine and atropine treatment: severe autonomic dysfunction with abnormal heart rate variability. Acta Anaesthesiologica Taiwanica. 2006; 44: 109-112 PubMed Google Scholar and Guillain-Barré syndrome. 2 Pfeiffer G. Schiller B. Kruse J. Netzer J. Indicators of dysautonomia in severe Guillain-Barre syndrome. J Neurol. 1999; 246: 1015-1022 Crossref PubMed Scopus (47) Google Scholar Autoimmune autonomic neuropathy (AAN), also known as idiopathic autonomic neuropathy or pure pandysautonomia, 3 Schondorf R. Low P.A. Idiopathic orthostatic tachycardia syndrome: an attenuated form of acute pandysautonomia?. Neurology. 1994; 43: 132-137 Crossref Google Scholar has been reported to rarely manifest with bradycardia as one of a constellation of signs. 4 Suarez G.A. Fealey R.D. Cailleri M. Low P.A. Idiopathic autonomic neuropathy: Clinical, neurophysiologic, and follow-up on 27 patients. Neurology. 1994; 44: 1675-1682 Crossref PubMed Google Scholar , 5 Klein C.M. Vernino S. Lennon V.A. et al. The spectrum of autoimmune autonomic neuropathies. Ann Neurol. 2003; 53: 752-758 Crossref PubMed Scopus (177) Google Scholar We present an unusual case of severe bradycardia in a young patient with probable autoimmune autonomic dysfunction.

Plasmapheresis Treatment in Guillain-Barré Syndrome: Potential Benefit over Intravenous Immunoglobulin

Guillain-Barré syndrome includes acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, Miller Fisher syndrome and acute pandysautonomia. Plasma exchange was the first treatment in Guillain-Barrd syndrome proven to be superior to supportive treatment alone and intravenous immunoglobulin was subsequently shown to be equally effective and is now commonly used as first-line treatment. We describe a 78-year-old woman who presented with a two-day history of progressive generalised weakness and left facial nerve palsy, preceded by a flu-like illness lasting for one week. A five-day course of daily immunoglobulin (0.4 g/kg/day) was commenced without benefit and progressive clinical deterioration. Seven days after completion of immunoglobulin treatment, plasma exchange was started with an exchange of about three litres of plasma every day for three days and every second day on two further occasions. A gradual improvement of respiratory function and peripheral muscle strength was observed after the first plasma exchange and on the eighth day the patient was weaned off mechanical ventilation. This case suggests that patients with severe Guillain-Barrd syndrome may benefit from plasma exchange after immunoglobulin treatment in refractory cases. Plasma exchange should be considered early in Guillain-Barrć syndrome cases with axonal involvement, and in the recurrent or familial Guillain-Barré syndrome forms.

Anti-neuronal Antibodies in Acute Pandysautonomia

We encountered two patients with acute pandysautonomia who subacutely exhibited extensive autonomic dysfunction after antecedent infections. Although these patients had been suffering from autonomic disturbance for several months, they both had a good clinical course after plasma exchange and intravenous immunoglobulin therapy. Thin-layer chromatography (TLC)-immunostaining did not demonstrate any antibodies against gangliosides, but immunoblot analysis showed antibodies against a neuroblastoma cell line, SH-SY5Y, in serum samples. Furthermore, ganglionic acetylcholine receptor autoantibodies were detected in one patient. These findings suggest that neuronal antibodies against the autonomic nervous system play an important role in the pathogenesis of acute pandysautonomia.

S3. Autonomic neuropathies — Recent advances: S3.1 Pathogenesis of autoimmune autonomic ganglionopathy

Antibodies against nicotinic acetylcholine receptors (AChR) are implicated in myasthenia gravis as well as in autoimmune autonomic ganglionopathy (AAG). AAG typically presents as severe generalized sympathetic and parasympathetic autonomic failure. This disorder is also known as acute pandysautonomia or idiopathic subacute autonomic neuropathy. The most disabling manifestations are orthostatic hypotension (OH) and gastrointestinal dysmotility. An autoimmune pathogenesis was suggested by the subacute onset, frequent association with antecedent viral illness, occasional association with cancer or other autoimmune disorders, and spontaneous recovery in some patients.

Acute pan-dysautonomia as well as central nervous system involvement and peripheral neuropathies in a patient with systemic lupus erythematosus

A 32-year-old woman was diagnosed with leucopenia in 2002, being antinuclear antibody, anti-DNA antibody, and antiphospholipid antibody positive, and she was administered low-dose aspirin. In July 2006, she was admitted to our hospital because of pyrexia and abdominal pain. Examination revealed paralytic ileus, absence of the pupillary light reflex, dyshidrosis and anuresis. In addition, with high-level interleukin-6 in cerebrospinal fluid, the sensory nerve conduction velocity was derivation impotence. She was subsequently diagnosed with systemic lupus erythematosus (SLE) with central nervous system involvement, peripheral neuropathy as well as acute pan-dysautonomia. After pulse corticosteroid therapy, paralytic ileus was improved, however, the urination disorder persisted, and syncope due to orthostatic hypotension became marked. Plasma exchange and a second course of pulse corticosteroid therapy were performed, and were ineffective, whereas intravenous cyclophosphamide was effective. This patient is a rare case of central nervous system, peripheral neuropathy as well as acute pan-dysautonomia with SLE.

Pandysautonomie aiguë lentement régressive associée à une achalasie de l’œsophage : à propos d’un cas

Acute pandysautonomia is a rare acute autonomic neuropathy that mainly affects young women. We report a case of idiopathic acute pandysautonomia associated with an esophageal achalasia in a 30-year-old woman. The clinical features were inaugural dysphagia followed by signs of parasympathetic failure of the entire digestive tract, bladder and pupils. Twenty-four hours of electrocardiographic recording showed involvement of sympathetic adrenergic nerves. Esophageal achalasia was patent on esophageal manometry. Upper digestive tract motility was first involved and then extended to the entire digestive tract with intestinal obstruction, which required emergency ileostomy. Recovery of autonomic functions was slow. After 16 months, dysphagia and gut paresis improved and digestive continuity was restored. In case of subacute intestinal pseudo-obstruction associated with autonomic dysfunction, acute pandysautonomia should be suspected. In our report, the association with esophageal achalasia is uncommon.

Autoimmune autonomic neuropathy: Clinical and experimental

Autoimmune autonomic neuropathy (AAN) results in severe generalized sympathetic and parasympathetic autonomic failure. This disorder is also known as acute pandysautonomia or idiopathic subacute autonomic neuropathy. The most disabling manifestations are orthostatic hypotension (OH) and gastrointestinal dysmotility. An autoimmune pathogenesis was suggested by the subacute onset, frequent association with antecedent viral illness, occasional association with cancer or other autoimmune disorders, and spontaneous recovery in some patients.

Autonomic Disorders and the Eye

Autonomic Disorders and the Eye

OPL132 The autonomic study in a case of acute pandysautonomia associated with Campylobacter jejuni infection

OPL132 The autonomic study in a case of acute pandysautonomia associated with Campylobacter jejuni infection

Pandysautonomie isolée subaiguë : intérêt du traitement par immunoglobulines polyvalentes

Acute or sub-acute pure dysautonomia is uncommon. We report a case of sub-acute pure pandysautonomia with favorable outcome after intravenous immunoglobulin therapy. A 29-year-old right-handed student, with an uneventful medical history presented, for one month, bilateral loss of visual acuity and digestive disorders, associating diarrhea, vomiting and anorexia. Physical examination revealed bilateral intrinsec oculomotor nerve palsy, a dryness syndrome and severe orthostatic hypotension. Ophthalmologic examination showed bilateral diffuse parasympathic impairment associating an Argyll Robertson pupil and full pupil light reflex abolition. Elevated protein level (0.93g/l) was the only cerebrospinal fluid anomaly. Serum tests were negative for anti-gangliosides antibodies. The patient improved slowly after two series of intravenous immunoglobulin infusions. Clinical course and laboratory findings suggest that acute or sub-acute pure pandysautonomia events are likely to be related to acute polyradiculoneuritis. Therefore intravenous polyvalent immunoglobulin infusions should be attempted, even if their efficacy needs to be confirmed.

Syndrome of Inappropriate Secretion of Antidiuretic Hormone and Thrombocytopenia Caused by Cytomegalovirus Infection In a Young Immunocompetent Woman

We report the first case of the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) associated with cytomegalovirus (CMV) infection. A 32-year-old woman was admitted to our hospital because of pandysautonomic signs and symptoms. Thrombocytopenia and hyponatremia were present. Serum anti-CMV IgM and IgG antibodies were positive. Despite hyponatremia, urinary osmolality exceeded plasma osmolality and plasma vasopressin levels related to plasma osmolality were high. Restriction of water intake and administration of dimethylchlorotetracycline improved hyponatremia, suggesting this patient had SIADH. In this patient, SIADH may have been caused by acute pandysautonomia that developed following CMV infection.

Life-threatening orthostatic hypotension in a case with bulbo-myelo-radiculo-neuropathy

A 59-year-old female developed acute autonomic failure accompanied by life-threatening orthostatic hypotension. Reduced plasma noradrenaline levels and enhanced pressure response to noradrenaline infusion were compatible with a diagnosis of acute pan-dysautonomia. However, nerve conduction tests clearly revealed motor and sensory nerve involvement and abnormal F-responses. A sural nerve biopsy and catecholamine fluorescence study of the rectal mucosa revealed relatively preserved postganglionic unmyelinated nerve fibers. Six weeks later, the patient developed another episode of bulbar palsy and right hemiparesis; the MRI showed lesions in the medulla oblongata and right cervical spinal cord. The prognosis of acute pan-dysautonomia is usually unsatisfactory, but the present patient showed good steroid-responsiveness probably because impaired preganglionic sympathetic myelinated fibers and medulla oblongata recovered quickly.

Acquired Inflammatory Demyelinating Neuropathies

The acquired demyelinating neuropathies can be divided into those with an acute onset and course and those with a more chronic course. The acute neuropathies present as Guillain-Barré syndrome and include acute inflammatory demyelinating polyradiculoneuropathy (AIDP), Miller Fisher syndrome, acute motor axonal neuropathy (AMAN), acute motor and sensory axonal neuropathy (AMSAN), and acute pandysautonomia. The chronic neuropathies are collectively known as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and include MADSAM (multifocal acquired demyelinating sensory and motor neuropathy, also know as Lewis-Sumner syndrome) and DADS (distal acquired demyelinating symmetric neuropathy) as variants. The clinical features, pathology, pathogenesis, diagnosis, treatment, rehabilitation, and prognosis of these neuropathies are discussed.