1/30. Multisystem neuronal involvement and sicca complex: broadening the spectrum of complications. We report two patients with multisystem neuronal involvement associated with sicca complex. One had a lower motor neuron syndrome combined with a flaccid bladder and rectum. The second patient had unilateral hearing loss, sensory neuronopathy, Adie's pupils, upper motor neuron signs, and autopsy-proven anterior horn cell degeneration. Our cases lead us to propose that the spectrum of neuronal involvement occurring with sicca syndrome may be wider than is currently appreciated. ( info) |
2/30. adie syndrome: evidence for refractive error and accomodative asymmetry as the cause of amblyopia. PURPOSE: To report objective changes in accommodation in a child with adie syndrome. methods: A child aged 2 years 10 months when initially examined was found to have good visual acuity in both eyes, a low degree of hypermetropia (isometropic), and Adie pupil presumed to be caused by chicken pox that had occurred 2 months earlier. amblyopia developed but responded well to treatment, which involved correction of the refractive error and occlusion therapy. Objective changes in the refraction of the eye were measured on the Canon R1 autorefractor at 3.8 m and 33 cm. RESULT: The degree of accommodation in the affected eye when both eyes were open was markedly reduced. CONCLUSION: The presence of isometropic hypermetropia, which remains uncorrected when adie syndrome is present, can lead to the development of amblyopia in a child. ( info) |
3/30. Bilateral glaucomatocyclitic crisis in a patient with Holmes adie syndrome. A patient with pre-existing bilateral tonic pupils presented with simultaneous bilateral glaucomatocyclitic crisis. Deep tendon reflexes were absent although they were documented to be present 6 years ago. A possibility of a progressive autonomic dysfunction in both these conditions is discussed. ( info) |
4/30. The Holmes-Adie plus syndrome. A clinical syndrome of tonic pupil associated with tendon areflexia was first described by Holmes and Adie; autonomic neuropathy and peripheral neuropathy can be associated. The postulated mechanism of areflexia in Holmes-adie syndrome is a synaptic disorder of the spinal reflex pathways. We report a case of a Holmes-adie syndrome variant with hitherto unreported cranial neuropathy. A 41 year old woman developed insidious onset of sensory symptoms related to her left trigeminal and chorda tympani nerves over a few months. physical examination showed generalised tendon areflexia and a left sided Adie's pupil. Imaging did not reveal any structural abnormality. Electrophysiological studies demonstrated an absent blink reflex on stimulating the left supraorbital and infraorbital nerves. These findings were suggestive of a dysfunction affecting the brain stem reflex arc. The pathophysiological process of Holmes-adie syndrome may be more widespread than previously thought. ( info) |
5/30. Ross' syndrome (tonic pupil plus). Two cases of tonic pupil, hyporeflexia, and segmental hypohidrosis (Ross' syndrome) are reported. The relationship of this syndrome to other autonomic dysfunction is discussed. Those symptoms (emotional instability, loss of sweating, orthostatic hypotensive symptoms, and signs of bilaterality of the tonic pupil) which should alert the clinician to more extensive disease states are noted. It is suggested that these conditions may represent a continuum or spectrum of disorders with a widespread degree of severity and progression. ( info) |
6/30. Idiopathic unilateral hyperhidrosis with Holmes-adie syndrome: case report. hyperhidrosis is a disease that is characterized by excessive sweating due to hyperactivation of eccrine sweat glands. It may be localized or generalized form. Holmes-adie syndrome is an idiopathic disease with unilateral pupil dilatation and loss of deep tendon reflexes. We present a 37-year-old female patient diagnosed with unilateral hyperhidrosis coincident with Holmes-adie syndrome because of this unusual presentation. ( info) |
7/30. Segmental facial anhidrosis and tonic pupils with preserved deep tendon reflexes: a novel autonomic neuropathy. A 31-year-old woman had exertional right-sided hemifacial flushing and sweating. Examination demonstrated slightly dilated pupils with absent constriction to light and a tonic near response and redilatation, features consistent with adie syndrome. Neurological examination was otherwise normal, including preservation of deep tendon reflexes. magnetic resonance imaging of brain and spine were normal. The combination of unilateral loss of sudomotor and vasomotor activity without loss of ocular sympathetic innervation fulfills the diagnosis of Harlequin syndrome. The combination of Harlequin and Adie syndromes has been called Ross syndrome, but the preservation of deep tendon reflexes precludes a diagnosis of Ross syndrome in our patient. This previously undescribed variant adds further complexity to the spectrum of autonomic neuropathies. ( info) |
| A 35-year-old female patient presented with the following symptoms of Holmes-adie syndrome: photophobia,enlargement of the left pupil unresponsive to light, Achilles areflexia. The pilocarpine test was positive. No tumor or other neurological abnormality was found. She had a 19-year history of autoimmune hepatitis.Flares up were observed following each 3 deliveries. At age of 31 she presented with diarrhea and weight loss.Abdominal tumor was detected by ultrasound. The surgically removed tumor was histologically a benign mesenteric multicystic lymphangioma.Simultaneously, celiac disease was diagnosed.Gluten-free diet resulted in a significant improvement of celiac disease,but not of autoimmune hepatitis. Autonomic neuropathy was proven by standard cardiovascular tests. The patient was a homozygous carrier for HLA DQ2 antigen characteristic for celiac disease and heterozygous for HLA DR3 B8 frequent in autoimmune liver diseases. Our novel observation on association of Holmes-adie syndrome with autoimmune hepatitis and celiac disease is suggestive for a common immunological background for all three entities present in a patient with mesenteric multicystic lymphangioma. ( info) |
9/30. Adie's pupil following Le Fort I maxillary osteotomy. A complication or coincidence? A case is reported of right-sided tonic pupil in isolation (Adie's pupil) following Le Fort I maxillary osteotomy. It is difficult to identify whether this rare finding immediately postoperatively is either a pure coincidence or a genuine complication of surgery. ( info) |
| We investigated pupillary responses to parasympathetic (pilocarpine) and sympathetic agents (tyramine, cocaine, and phenylephrine) in a 51-year-old woman with tonic pupils, loss of muscle stretch reflexes in the limbs, and hemifacial loss of sweating and flushing (Ross' syndrome). A smaller pupillary response to tyramine and cocaine eyedrops on the symptomatic side indicated that outflow was disrupted in the postganglionic section of the ocular sympathetic pathway. A greater response to phenylephrine eyedrops on this side was consistent with denervation supersensitivity to adrenergic agents. Loss of thermoregulatory sweating and flushing and emotional blushing in the forehead, cheek, and chin indicated that sympathetic disruption was proximal to the bifurcation of the common carotid artery, probably in the superior cervical ganglion. A similar degenerative process may be responsible for loss of muscle stretch reflexes, tonic pupils, and other autonomic disturbances in Ross' syndrome. ( info) |