Bilateral glaucomatocyclitic crisis in a patient with Holmes Adie syndrome. (1/15)

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)

Flaccid paraplegia: a feature of spinal cord lesions in Holmes-Adie syndrome and tabes dorsalis. (2/15)

In a patient with Holmes-Adie syndrome, and in another with tabes dorsalis, a transverse cord lesion resulted in a severe, but flaccid paraplegia with absent tendon reflexes. Flexor spasms were severe in both patients, but spasticity was absent. The significance of these observations is discussed in relation to the functional and anatomical disorder in these two syndromes.  (+info)

Ross' syndrome (tonic pupil plus). (3/15)

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)

Holmes-Adie syndrome, autoimmune hepatitis and celiac disease: a case report. (4/15)

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)

Ross syndrome: a rare or a misknown disorder of thermoregulation? A skin innervation study on 12 subjects. (5/15)

Ross syndrome is described as a rare disorder of sweating associated with areflexia and tonic pupil. Since Ross's first description in 1958, approximately 40 cases have been described. We assessed the involvement of cutaneous innervation in 12 subjects with Ross syndrome using quantitative sensory testing, sweating assessment and immunohistochemical study of anhidrotic and hyperhidrotic skin. This evaluation was repeated over time in 4 out of 12 subjects. In addition, we enrolled four subjects with Holmes-Adie syndrome (areflexia and tonic pupil) to investigate similarities between the two conditions. We found in Ross patients a complex and progressive involvement of cutaneous sensory and autonomic innervation underlying the impairment of heat production and heat dissipation through both loss of sweating and loss of cutaneous blood flow regulation. In Holmes-Adie subjects we found a mild impairment of sweating without thermoregulatory problems. The persistence of a sudomotor vasoactive intestinal peptide-immunoreactive (VIP-ir) innervation, although deranged and poor, definitely differentiated Holmes-Adie from Ross patients. Ross syndrome is a progressive and complex disorder of thermoregulation difficult to differentiate from the probably pathogenetically related Holmes-Adie syndrome. Sweating assessment and skin biopsy are suitable tools to define a boundary between them. Owing to the large number of Ross patients observed in only 5 years, and to the long and complex medical history of most of them, doubts arise on the effective rarity of this condition, and we warn family doctors and other specialists, besides neurologists, to become aware of this complex disorder.  (+info)

Bilateral tonic pupils: Holmes Adie syndrome or generalised neuropathy? (6/15)

AIM: To compare the pupil signs in patients with bilateral pupillotonia caused by Holmes-Adie syndrome or generalised peripheral neuropathy. METHODS: Infrared video pupillographic techniques were used to measure a number of pupil variables in patients with Holmes-Adie syndrome, generalised neuropathy (various aetiologies) and healthy age-matched control subjects. RESULTS: Regardless of aetiology, the patients generally had pupil signs typical of pupillotonia (small dark diameters, large light diameters, tonic near responses, attenuated light responses with light-near dissociation, and sector palsy). However, significant differences were found in the prevalence and magnitude of several pupil variables in the two patient groups. In particular, sector palsy and anisocoria exceeding 1 mm (in the light) were seen much more commonly in Holmes-Adie patients than patients with generalised neuropathy. The presence of both these pupil signs can be used to distinguish between these diagnoses with a sensitivity of 58% and a specificity of 90%. CONCLUSIONS: The tonic pupils of patients with Holmes-Adie syndrome are significantly different to those found in patients with generalised neuropathy; recognition of these differences may allow distinction between these diagnoses.  (+info)

Anisocoria in liver recipients during the perioperative period: Two case reports. (7/15)

Two cases of anisocoria that occurred just after the induction of general anesthesia for living donor liver transplantation are reported. Space-occupying lesions were not observed in brain computed tomography. Mydriasis was temporary in both cases, suggesting that the anisocoria was most likely related to Adie syndrome.  (+info)

Pure sensory neuropathy in patients with primary Sjogren's syndrome: clinical, immunological, and electromyographic findings. (8/15)

A pure sensory neuropathy caused by lymphocytic infiltration of the dorsal root ganglia has been reported in a few patients with Sjogren's syndrome. The clinical, immunological, and electromyographic findings of five patients with this type of neuropathy and primary Sjogren's syndrome were reviewed. Typical clinical indications were the presence of a chronic asymmetrical sensory deficit, initial disease in the hands with a predominant loss of the vibratory and joint position senses, and an association with Adie's pupil syndrome or trigeminal sensory neuropathy. The simultaneous impairment of the central and peripheral evoked cortical potentials suggested that there was a lesion of the neuronal cell body. The neuropathy preceded the diagnosis of Sjogren's syndrome in four patients. Four patients were positive for Ro antibodies, but systemic vasculitis or malignancy was not found after a mean follow up of six years. These findings indicate that in patients with a sensory neuropathy the diagnosis of Sjogren's syndrome has to be considered, even if the patient denies the presence of sicca symptoms, and that appropriate tests must be carried out.  (+info)

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