Concurrence of Hyperhidrosis and Hypohidrosis in Ross Syndrome.

Ross Syndrome is a rare disorder characterized by tonic pupils, hyporeflexia, and abnormal segmental sweating. The pathophysiology of the disease remains unclear, with either hypohidrosis or hyperhidrosis reported in individual patients. We present the case of a man, aged 57 years, who presented with hyperhidrosis in his right extremities, anhidrosis in the left extremities, and changes in his pupils. The disease was not associated with markers of autoimmune disease, which supports recent research findings on the role of neurodegeneration. The patient's son was exhibiting similar symptoms, which implicates genetic inheritance in the process. A multidisciplinary approach is crucial for the diagnosis and ultimate management of patients with Ross Syndrome.

Ross Syndrome Presenting as Heat Exhaustion: A Report of Two Cases.

Ross syndrome is a rare clinical disorder of sweating associated with tonic pupil and areflexia. There are very few case reports of Ross syndrome in dermatology literature, most presenting with patchy hyperhidrosis. Here, we report two isolated cases who had presented to the emergency department with heat exhaustion. Multidisciplinary evaluations of the first case revealed focal anhidrosis, patchy hyperhidrosis, postural hypotension, absent deep tendon reflex, and tonic pupil while the second case had similar features except for postural hypotension, prompting the diagnosis of Ross syndrome. Presentation of these two patients highlights the importance of a high index of suspicion of dysautonomic disorder, interdisciplinary workup of a case of patchy anhidrosis, or hyperhidrosis, which may get missed in busy outpatient department (OPD) visit.

Two Cases of Tonic Pupil: Ross and Ross Syndrome Plus.

Ross syndrome is a rare disorder of the peripheral autonomic nervous system characterized by a triad of tonic pupils with light-near dissociation, segmental anhidrosis, and areflexia. Though having a benign course, the disease can cause significant social embarrassment. Both our cases presented with complaints of segmental facial hyperhidrosis. The first case with a one-year history had findings of segmental anhidrosis up to T4 thoracic level, left tonic pupil, and absent right ankle reflex. While the second case with a history of five years had bilateral tonic pupil, absent lower limb reflexes, anhidrosis of left face, neck, and upper trunk up to T4 level, apart from having associated Horner's syndrome. Minor's (starch-iodine) test and dilute pilocarpine test were helpful for diagnosis in both cases, indicating areas of anhidrosis and pupillary cholinergic denervation hypersensitivity respectively. Both cases were provided counseling and managed conservatively.

Clinical presentation and autonomic profile in Ross syndrome.

BACKGROUND: Ross syndrome is a rare disorder characterized by tonic pupils, hyporeflexia, and segmental anhidrosis. We sought to characterize the clinical presentation, associated autoimmune disorders, and autonomic profile in patients with Ross syndrome to further elucidate its pathophysiology. METHODS: We performed a retrospective chart review of all patients who underwent a thermoregulatory sweat test (TST) between 1998 and 2020 and had confirmation of the diagnosis of Ross syndrome by an autonomic disorders specialist. Standardized autonomic reflex testing was reviewed when available. RESULTS: Twenty-six patients with Ross syndrome were identified. The most common initial reported manifestation was an abnormal segmental sweating response in 16 patients (described as hyperhidrosis in 12 patients and anhidrosis in 4 patients) while a tonic pupil was the initial manifestation in 10 patients. Other commonly reported symptoms included fatigue, chronic cough, and increased urinary frequency. An associated autoimmune disorder was identified in one patient. Positive autoantibodies were found in a minority of patients often with unclear clinical significance. Distributions of anhidrosis encountered were segmental (n = 15), widespread (n = 7), and global (n = 4). Well-circumscribed small areas of preserved sweating within areas of anhidrosis were observed in the majority of patients (88.5%). Anhidrosis progressed slowly over time and sudomotor dysfunction was predominantly (post)ganglionic. Cardiovagal and adrenergic functions were preserved in most patients. CONCLUSIONS: The pattern of autonomic dysfunction in Ross syndrome is suggestive of a limited autonomic ganglioneuropathy. Sudomotor impairment is prominent and should be the focus of symptomatic management; however, clinicians should be aware of symptoms beyond the classic triad.

Ross Syndrome: A Patient with a 23-Year History.

We present a 60-year-old female with a 23-year history of anhidrosis with concomitant heat intolerance. At examination, we found a right-sided tonic pupil, absent tendon reflexes, and a segmental patch of compensatory hyperhidrosis in the left lower quadrant of her trunk. To further confirm this finding, a minor test (a starch-iodine test, which is used to evaluate the sudomotor function, sweating) was performed. Nerve conduction studies revealed evidence of a mild distal sensory polyneuropathy of the axonal type. Tilt-table testing showed signs of orthostatic hypotension with evidence of reduced sympathetic function. Skin biopsy excluded pathology in the sweat glands. Our patient met the criteria for a diagnosis of Ross syndrome. This case describes the long-term outcome of this rare entity and highlights how careful examination and bedside autonomic testing can confirm the diagnosis of autonomic neurological disorders.

Ross Syndrome.

Ross syndrome is a rare disorder of thermoregulation and includes a triad of tonic pupil, anhidrosis/hypohidosis and areflexia. Here we describe one such case in a 40-year-old woman. A general awareness among physicians, dermatologists and ophthalmologists regarding this disease can alleviate unnecessary anxiety and avoid unnecessary investigations.

Clinical and laboratory correlates of selective autonomic dysfunction due to Ross syndrome.

Ross syndrome is diagnosed by the presence of the characteristic triad of segmental anhidrosis, depressed deep tendon reflex, and tonic pupils. It is a rare, misdiagnosed autonomic disorder with less than 80 cases reported in the world literature. Two representative cases of Ross syndrome are presented with their laboratory correlates and relevant review of literature. Both cases (aged 35 and 58) presented with complaint of decreased sweating over one half of the face and ipsilateral upper limb and trunk and contralateral lower limb. There was compensatory increased sweating and hyperpigmentation over the remaining parts of the body. The duration of symptoms was 2 years and 15 days. The patients had variegated skin color as per the above distribution and hyporeflexia in lower limbs. One patient also had Holmes-Adie pupil. Iodine test showed hypohidrosis in the described areas, which was confirmed by skin biopsy in both cases. The patients were treated symptomatically with incomplete relief. The authors aim to highlight this rare disorder that can be one of the causes of pathological sweating encountered in general practice and the challenges in its management.

Absent cardiac and muscle sympathetic nerve activities involvement in Ross syndrome: A follow-up study.

PURPOSE: Ross syndrome (RS) is characterized by selective involvement of post-ganglionic skin sympathetic nerve fibres. We report a follow-up study in 4 patients to clarify whether in RS autonomic dysfunction spreads affecting also cardiovascular system. METHODS: The patients underwent cardiovascular reflexes (CVR) and microneurography recording of muscle sympathetic nerve activity (MSNA) for a follow-up mean period of 5years. RESULTS: CVR and MSNA were normal at baseline and unchanged over the follow-up. CONCLUSIONS: Cardiovascular autonomic system is spared in RS differently from skin autonomic activity dysfunction which progress over time. However, before drawing any definite conclusion, a large cohort of patients needs to be studied.

Ross Syndrome: A Case Report and Review of Cases from India.

Ross syndrome is a rare dysautonomia characterized by a clinical complex of segmental anhidrosis or hypohidrosis, areflexia, and tonic pupils. A very few cases (≃50) have been reported in literature since its original description in 1958. Here, we report the case of a middle-aged homemaker from Odisha, India, who presented with complaints of segmental hypohidrosis for the past 7 years.

Ross Syndrome with Segmental Anhidrosis and Anisocoria: Application of Finger Winkle Test

Ross syndrome is characterized by a triad of segmental anhidrosis, tonic pupil, and generalized areflexia. Selective postganglionic autonomic denervation could be the differential diagnostic point for other diseases of the autonomic nervous system. Here we report a patient with regional anhidrosis in his left hand and sole, and anisocoria. An evaluation of sweating and the pupillary response together with generalized areflexia confirmed the diagnosis of Ross syndrome. The finger wrinkle test is a simple and useful tool for revealing segmental sympathetic denervation.

[Dysautonomic syndrome of the face with Harlequin sign and syndrome: Three new cases and a review of the literature]. / Syndromes dysautonomiques du visage avec signe et syndrome d'Harlequin : étude de trois cas et revue de la littérature.

INTRODUCTION: Harlequin phenomenon is characterized by a strictly unilateral erythrosis of the face with flushing and hyperhydrosis, and controlaterally a pale anhydrotic aspect. This syndrome can occur alone or associated to other dysautonomic phenomena such as Horner syndrome, Adie syndrome or Ross syndrome. PATIENTS AND METHODS: We report three cases: two patients presented a Harlequin sign, associated with Horner syndrome for one and Ross syndrome for the second. The etiologic investigation was normal, allowing recognizing the idiopathic nature of the disorder. For the third patient, Harlequin syndrome was observed in a neoplastic context due to breast cancer, metastatic dissemination, and bone metastases involving the right side of the T2 body. DISCUSSION: We reviewed the literature: 108 cases have been described. This syndrome occurred alone in 48 patients and was associated with other dysautonomic syndromes such as Horner syndrome in 38 patients, Holmes Adie syndrome in six, and Ross syndrome in six; both Ross and Holmes Adie syndrome were associated five cases and associations were not reported in five patients. The pathophysiological mechanisms of this autonomic cranial neuropathy, the possible etiologies, and therapeutic management were discussed. CONCLUSION: Harlequin phenomenon with flushing and unilateral hyperhydrosis is rare, occurring alone or in combination with other autonomic syndromes of the face. Idiopathic in two-thirds of cases, Harlequin phenomenon does not require specific treatment; sympathectomy may be discussed in the severe cases with a significant social impact.

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.

Ross syndrome with ana positivity: a clue to possible autoimmune origin and treatment with intravenous immunoglobulin.

A 28-year-old serving soldier presented with patchy areas of absence of sweating and blurring of vision. On examination he was found to have segmental anhidrosis, right sided tonic pupil and absent ankle jerks. Investigations revealed ANA positivity with no other abnormalities. He was treated with Intravenous immunoglobulin. This case of Ross syndrome is reported for its rarity as well as a clue to its probable autoimmune origin and treatment option with intravenous immunoglobulins.

A Case of Ross' Syndrome with Horner's Syndrome

Ross syndreome is a rare peripheral nervous system disorder defined by Adie' tonic pupil, hyporeflexia, and segmental anhidrosis. The pathogenesis is believed to result from damage to the ganglion cells or postganglionic fibers, including sympathetic, parasympathetic and dorsal root ganglion cells. A 46 year-old woman complained of right ptosis and decreased sweating of right face. The pupil showed anisocoria, which was evaluated under the variable conditions and recorded with the pupillography using Infrared Scanning Laser Photography(HRA , Heidelberg engineering, Germany). The neurologic examinations including starch-iodine test demonstrated areflexia of lower limbs and hypohidrosis of right face. The above examinations provided evidences that the sympathetic and parasympathetic ganglion cells or the post ganglionic fibers were involved and the diagnosis of Ross syndrome with Horner syndrome was made.