Myasthenia gravis with Holmes-Adie syndrome:a case report / 中华神经科杂志

Holmes-Adie syndrome (HAS) is a clinical syndrome mainly characterized by tonic pupil and disappearance of tendon reflex. It is mostly idiopathic and can also be seen in cerebral diseases, such as trauma, infection and tumors. However, it is rarely reported to be accompanied with myasthenia gravis (MG). We report a case of MG and HAS, whose clinical manifestations were fluctuation of limb weakness, breathing difficulties, right ptosis. Her pupils were unequal: the left pupil was 3 mm, the right pupil was 2 mm, direct and indirect light reflex was slow in left pupil, and right pupil was sensitive to light reflex. The left eye pupil shrank to 2 mm after dripped pilocarpine diluent for 10 minutes, while the right pupil was still 2 mm. Chest CT examination revealed thymoma. After treatment with thymectomy, glucocorticoid, immunoglobulins and tacrolimus, her symptoms of MG were improved, but the left pupil diameter and light reflex were not changed. Combined with the patient's symptoms, physical signs and examinations, this patient was diagnosed as MG accompanied with HAS.

Adie-Holmes Syndrome / Síndrome de Adie-Holmes

The Holmes-Adie syndrome is characterized by the presence of tonic pupil associated with absence or diminution of deep tendon reflexes. In some cases there may be autonomous nerve dysfunction. The mechanism that causes the disorder is not fully known, but is believed to be caused by denervation of the postganglionic supply to the sphincter of the pupil and the ciliary muscle which can occur following viral disease. Typically it affects young adults and is unilateral in 80% of cases, although it may develop in the contralateral eye in months or years. We report a case of a woman presenting typical signs of this syndrome, in which pharmacological test was essential for diagnosis.

A Síndrome de Holmes-Adie É caracterizada pela presença de pupila tônica associada à diminuição ou ausência dos reflexos tendíneos profundos. Em alguns casos pode haver disfunção nervosa autônoma. O mecanismo que causa a desordem não é totalmente conhecido, mas acredita-se que seja causada pela desnervação do suprimento pós-ganglionar para o esfíncter da pupila e para o músculo ciliar, que pode ocorrer após doença viral. Tipicamente afeta adultos jovens e é unilateral em 80% dos casos, embora possa se desenvolver no olho contralateral em meses ou anos. Nós relatamos o caso de uma mulher apresentando sinais típicos desta síndrome, em que o teste farmacológico foi fundamental para o diagnóstico.

Fixed Pupillary Light Reflex due to Peripheral Neuropathy after Liver Transplantation / 대한구급학회지

A 46-year-old female patient was admitted to the intensive care unit (ICU) after liver transplantation. About an hour later after the ICU admission, she had no pupillary light reflex. Both pupils were also fixed at 5 mm. Patients who undergo liver transplantation are susceptible to neurologic disorders including hepatic encephalopathy, thromboembolism and intracranial hemorrhage. Abnormal pupillary light reflex usually indicates a serious neurologic emergency in these patients; however, benign neurologic disorders such as peripheral autonomic neuropathy or Holmes-Adie syndrome should also be considered. We experienced a case of fixed pupillary light reflex after liver transplantation diagnosed as peripheral autonomic neuropathy.

Fixed Pupillary Light Reflex due to Peripheral Neuropathy after Liver Transplantation / 대한중환자의학회지

A 46-year-old female patient was admitted to the intensive care unit (ICU) after liver transplantation. About an hour later after the ICU admission, she had no pupillary light reflex. Both pupils were also fixed at 5 mm. Patients who undergo liver transplantation are susceptible to neurologic disorders including hepatic encephalopathy, thromboembolism and intracranial hemorrhage. Abnormal pupillary light reflex usually indicates a serious neurologic emergency in these patients; however, benign neurologic disorders such as peripheral autonomic neuropathy or Holmes-Adie syndrome should also be considered. We experienced a case of fixed pupillary light reflex after liver transplantation diagnosed as peripheral autonomic neuropathy.

Síndrome disautonómico de Ross: variaciones de un tema / Disautonomic Ross syndrome: variations about one theme

El síndrome de Ross fue descrito en 1958 como una afección degenerativa del sistema nervioso autónomo definido por la tríada de anhidrosis generalizada, disminución de los reflejos tendinosos y pupila tónica. Desde su descripción inicial se han descrito cerca de cuarenta casos. Comunicamos tres pacientes con variantes de interés que incluyen la presencia de espasmos cíclicos espontáneos del esfínter de iris, el desarrollo conjunto de síndrome de Holmes-Adie en un lado y síndrome Horner posganglionar en el otro, trastornos del desarrollo piloso en el lado de la anhidrosis, alteraciones de la motilidad intestinal, lengua sin papilas gustativas y disfunción sexual.

Ross Syndrome was described in 1958 as a degenerative condition of the autonomic nervous system defined by a triad of generalized anhidrosis, reduction of tendon reflexes and tonic pupil. Since its initial description about 40 cases have been described. We communicate three cases with variants of interest involving the presence of the simultaneous development of syndrome of Holmes-Adie on one side and Horner syndrome in the other, disorders of pilous follicle development on the side of anhidrosis, spontaneous disturbances of intestinal motility, tonque without papillae and sexual dysfunction.

Probable Paroxysmal Hemicrania Associated with Holmes-Adie Syndrome

No abstract available.

Tonic pupil presenting after surgical removal of an orbital cavernous haemangioma

Orbital trauma and surgery are recognised aetiological factors of tonic pupil. Tonic or Adie's pupil is an efferent pupil defect in which light reactions to one or more segments of the iris sphincter are lost due to the postganglionic parasympathetic nerves damage from ciliary ganglion. There is loss of part or all of the light reflex and decrease in accommodative functions at near. We report a case of tonic pupil in a 42-year-lady after a successful surgical removal of an orbital cavernous haemangioma.

Two Cases of Holmes-Adie Syndrome

BACKGROUND: Adie's tonic pupil consists of mydriasis, accommodation defect, decreased light reflex, and tonic near reflex. When tonic pupil is combined with hypo- or areflexia, it is called Holmes-Adie syndrome. CASE: Fifty-two-year-old and 37-year-old female patients showed unilateral dilated irregular pupil, decreased light reflex, and decreased or absent DTR. When 0.125% pilocarpine was dropped on the dilated pupil, the pupil was constricted after pilocarpine application. Somatosensory evoked potentials were within normal ranges, and H-reflex responses were absent or delayed.

A Case of Primary Sjogren's Syndrome Presenting as Adie's Syndrome / 대한류마티스학회지

Sjogren's syndrome is a hererogenous autoimmune disease characterized by progressive destruction of the exocrine glands and accompanied by a variety of autoimmune phenomena. Sjogren's syndrome patients can develop symptoms of ocular and oral dryness as well as extraglandular complications including central and peripheral nervous system disease. Sometimes neuropathy precedes the diagnosis of Sjogren's syndrome. Adiets syndrome is characterized by tonic pupil and the absence of tendon reflex. Sweating abnormality and chronic peripheral polyneuropathy can also be present. We report a case of primary Sjogren's syndrome preceded by Adie's syndrome with peripheral neuropathy. A 26-year-old woman was admitted for photophobia and paresthesia. On examination, her pupils were anisocoric and did not react to light but constricted promptly to pilocarpin. Sensation decreased on her left side of body and deep' tendon reflexes were absent. Biopsy of minor salivary gland demonstrated infiltration by lymphocyte consistent with Sjogren's syndrome, but Schirmer test was negative. So she was diagnosed as Adie's syndrome with peripheral neuropathy. Five month later she complained of dry eye and dizziness. Rose bengal staining was positive. Sjogren's syndrome was diagnosed and she was discharged with local therapy for the sicca symptoms.

Holmes-Adie syndrome with sweating abnormality (report of 1 case) / 临床神经病学杂志

Objective To investigate the clinical manifestation, pathological features and pathogenesis of Holmes-Adie syndrome (HAS). Methods The clinical data of one HAS patient with sweating abnormality and combined with literatures were analysed. Results The patient had the typical characteristics of HAS, including Adie pupil, areflexia of lower limb and the symptoms of autonomic nerve dysfunction. The characteristic pupil of HAS was proved by slit lamp of ophthalmology. Cranial MRI showed lacunar infarction. A partial or total loss of neurons of the ciliary ganglion was the main pathological characteristic of HAS. Conclusions The etiology of HAS remains unclear, and has characteristic pupil finding, which was associated with autonomic nerve dysfunction to a certain extent. HAS still need to differentiate from Ross syndrome and Harlequin syndrome.