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Anais Brasileiros de Dermatologia 2016Ross syndrome is a rare disease characterized by peripheral nervous system dysautonomia with selective degeneration of cholinergic fibers. It is composed by the triad of...
Ross syndrome is a rare disease characterized by peripheral nervous system dysautonomia with selective degeneration of cholinergic fibers. It is composed by the triad of unilateral or bilateral segmental anhidrosis, deep hyporeflexia and Holmes-Adie's tonic pupil. The presence of compensatory sweating is frequent, usually the symptom that most afflicts patients. The aspects of the syndrome are put to discussion due to the case of a male patient, caucasian, 47 years old, with clinical onset of 25 years.
Topics: Cholinergic Fibers; Humans; Hyperhidrosis; Hypohidrosis; Male; Middle Aged; Nerve Degeneration; Peripheral Nervous System Diseases; Primary Dysautonomias; Syndrome
PubMed: 26982793
DOI: 10.1590/abd1806-4841.20163918 -
Brain and Nerve = Shinkei Kenkyu No... Jan 2016A 37 year-old Japanese male felt photophobia of both eyes one week following the onset of the common cold. His neurological examination revealed bilateral Adie's tonic... (Review)
Review
A 37 year-old Japanese male felt photophobia of both eyes one week following the onset of the common cold. His neurological examination revealed bilateral Adie's tonic pupils, no extraocular movement disorder, normal deep tendon reflexes, and no cerebellar signs. Based on markedly increased blood levels of anti-GQ1b IgG and anti-GT1a IgG antibodies, we diagnosed him as anti-GQ1b antibody syndrome. Bilateral Adie's tonic pupils were improved by IVIg drip infusion and methylprednisolone pulse therapy. This case suggests that we need to investigate anti ganglioside antibody when a patient presents with bilateral Adie's tonic pupils.
Topics: Adult; Antibodies; Eye; Humans; Infusions, Intravenous; Male; Syndrome; Tonic Pupil
PubMed: 26764303
DOI: 10.11477/mf.1416200354 -
Srpski Arhiv Za Celokupno Lekarstvo 2015Adie's (tonic) pupil is a neuro-ophthalmological disorder characterized by a tonically dilated pupil, which is unresponsive to light. It is caused by damage to...
INTRODUCTION
Adie's (tonic) pupil is a neuro-ophthalmological disorder characterized by a tonically dilated pupil, which is unresponsive to light. It is caused by damage to postganglionic fibers of the parasympathetic innervation of the eye, usually by a viral.or bacterial infection. Adie's syndrome includes diminished deep tendon reflexes.
OUTLINE OF CASES
We report data of a 59-year-old female with unequal pupil sizes. She complained of blurred vision and headache mainly while reading. She had a 35-year history of hepatitis B and liver cirrhosis. On exam, left pupil was mydriatic and there was no response to light and at slit lamp we saw segments of the sphincter constrict. We performed 0.125% pilocarpine test and there was a remarkable reduction of size in the left pupil. The second case is a 55-year-old female who was referred to the University Eye Clinic because of a headache and mydriatic left pupil. She had diabetes mellitus type 2, as well as hepatitis A virus 20 years earlier. On exam, the left pupil was mydriatic, with no response to light. Test with diluted pilocarpine was positive. Neurological examinations revealed no abnormality in either case so we excluded Adie's syndrome.
CONCLUSION
Adie's tonic pupil is benign neuro-ophthalmological disorder of unknown etiology. Most patients commonly present no symptoms and anisocoria is noticed accidentally. Although the etiology is unknown, there are some conditions that cause tonic pupil. It may be a part of a syndrome in which tonic pupil is associated with absent deep tendon reflexes.
Topics: Accommodation, Ocular; Adie Syndrome; Diabetes Complications; Female; Hepatitis B; Humans; Iris; Middle Aged; Pilocarpine; Pupil; Reflex, Pupillary; Tonic Pupil
PubMed: 26506756
DOI: 10.2298/sarh1508451k -
Neuro-ophthalmology (Aeolus Press) Oct 2015Adie's pupil is characterised by pupil dilatation, segmental iris palsy, light-near dissociation, and slow re-dilatation. Most commonly, tonic pupils are unilateral and...
Adie's pupil is characterised by pupil dilatation, segmental iris palsy, light-near dissociation, and slow re-dilatation. Most commonly, tonic pupils are unilateral and idiopathic, but can be caused by orbital disorders and autonomic neuropathies. There are only a few case reports of tonic pupils in patients with Sjögren's syndrome, caused by an autoimmune ciliary ganglionitis. The authors report on two cases with bilateral tonic pupils as the initial manifestation of primary Sjögren's syndrome. Both patients presented with blurred vision, bilateral tonic pupils, and sicca symptoms. The findings suggest that Sjögren's syndrome should be considered in patients presenting with bilateral Adie's tonic pupils.
PubMed: 27928364
DOI: 10.3109/01658107.2015.1067231 -
The Pan African Medical Journal 2015
Topics: Adie Syndrome; Adolescent; Diagnosis, Differential; Female; Humans
PubMed: 26213604
DOI: 10.11604/pamj.2015.20.330.4437 -
The Breast Journal 2015Here, we present a case of tonic pupil associated with occult breast cancer as a paraneoplastic neuro-ophthalmology syndrome. A 45-year-old woman developed progressive...
Here, we present a case of tonic pupil associated with occult breast cancer as a paraneoplastic neuro-ophthalmology syndrome. A 45-year-old woman developed progressive photophobia and blurred vision due to unilateral Adie's tonic pupil. Magnetic resonance image of her brain and neurological examination (including deep tendon reflexes) were normal at first visit. Follow-up examinations performed by ophthalmologist every 6 month without any change in her condition. After 2 years, patient discovered a mass in her breast which identified to be malignant after diagnostic procedures. Despite surgical and medical treatment for cancer, no change in the ocular condition was happened.
Topics: Breast Neoplasms; Female; Humans; Middle Aged; Paraneoplastic Syndromes, Nervous System; Tonic Pupil; Treatment Outcome
PubMed: 26174775
DOI: 10.1111/tbj.12451 -
Dermatology Online Journal Apr 2015Harlequin syndrome (HS) is a rare disorder of the sympathetic nervous system which presents with unilateral decreased sweating and flushing of the face, neck, and chest...
Harlequin syndrome (HS) is a rare disorder of the sympathetic nervous system which presents with unilateral decreased sweating and flushing of the face, neck, and chest in response to heat, exercise, or emotional factors. The contralateral side displays a compensatory overreaction to provide normal heat regulation of the face as a whole. In the literature, most of the cases are primary in nature and no underlying cause could be identified. Harlequin sign is used to denote these symptoms in patients who also exhibit associated oculosympathetic paresis, such as Horner syndrome, Adie syndrome, and Ross syndrome.We report a rare case of a 13-year-old boy who presented with complaints of flushing and sweating of the left side of the face after exertion, while the right side remained dry and maintained its normal color. No structural abnormality was identified on detailed work up. Thus, diagnosis of classic idiopathic HS was made. Despite the rarity of this syndrome, dermatologists should be acquainted with this distinctive entity and should refer the patient for complete ophthalmological and neurological examination.
Topics: Adolescent; Autonomic Nervous System Diseases; Exercise; Face; Flushing; Humans; Hypohidrosis; Male; Stress, Psychological
PubMed: 25933078
DOI: No ID Found -
Turkish Journal of Emergency Medicine Mar 2015Holmes-Adie syndrome (HAS) is a rare syndrome characterized by tonic pupil and the absence of deep tendon reflexes. HAS was first described in 1931 and is usually...
Holmes-Adie syndrome (HAS) is a rare syndrome characterized by tonic pupil and the absence of deep tendon reflexes. HAS was first described in 1931 and is usually idiopathic, with incidences reported to be 4-7 per 100,000. Although tonic pupil is usually unilateral, it can also be bilateral. Enlarged and irregular pupil is usually noticed by the patient. Light reflex is weak or unresponsive. Another characteristic of HAS is the absence of deep tendon reflexes, and unilateral involvement is more common. This case report emphasizes that HAS should be considered in the differential diagnosis of patients presenting to the emergency department with anisocoria, and the dilute pilocarpine test can be used in diagnosis.
PubMed: 27437522
DOI: 10.5505/1304.7361.2015.59144 -
Journal of Neurosciences in Rural... Oct 2014Ross syndrome is a rare sweating disorder associated with Adie's tonic pupil, decreased or diminished tendon reflex and unknown etiology. Although autonomic disturbances...
Ross syndrome is a rare sweating disorder associated with Adie's tonic pupil, decreased or diminished tendon reflex and unknown etiology. Although autonomic disturbances affecting sudomotor and vasomotor functions are seen commonly, they are rarely symptomatic. While Ross syndrome is typically characterized with dilated tonic pupil, it may be rarely manifested with miotic pupils (little old Adie's pupil), which can make diagnosis difficult. In this article, we aim to specify the atypical clinical manifestations of syndrome by means of Ross syndrome manifested by autonomic symptoms, Horner syndrome, chronic cough together with bilateral little old Adie's pupil.
PubMed: 25288844
DOI: 10.4103/0976-3147.139997 -
Journal of Anesthesia Apr 2015
Topics: Adie Syndrome; Anesthesia, General; Anisocoria; Female; Humans; Middle Aged; Neurologic Examination; Reflex, Pupillary
PubMed: 25138821
DOI: 10.1007/s00540-014-1902-y