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Ross Syndrome with Vitiligo

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Ross Syndrome with Vitiligo CASE REPORT Indian Journal of Clinical Practice, Vol. 30, No. 7, December 2019 Ross Syndrome with Vitiligo CHANDRAJEET SINGH RANAWAT*, DIVYA GOEL*, PRATIBHA SHEKHAWAT†, AMIT KUMAR* ABSTRACT Ross syndrome is rare clinical disorder of autonomic system with selective degeneration of cholinergic fibers of sweat glands. It is characterized by segmental anhidrosis or hypohidrosis associated with generalized areflexia or hyporeflexia and tonic pupil. Here, we report a case of a middle-aged man from Jaipur, Rajasthan who presented with complaints of segmental hypohidrosis for past 2 years. Keywords: Anhidrosis/hypohidrosis, hyporeflexia/areflexia, tonic pupil oss syndrome is a dysautonomic disorder or connective tissue disorders. There was no history of of peripheral nervous system with selective trauma to spine. Family history for similar kind Rdegeneration of cholinergic fibers of sweat glands, of illness was negative. characterized by a triad of segmental anhidrosis or On examination, patient was conscious and his vitals hypohidrosis, areflexia or hyporeflexia and tonic pupil. were stable. General physical examination revealed In 1958, it was first described by Alexander Ross.1,2 hypopigmented skin patch over periorbital and perioral The pathogenesis of Ross syndrome is still unknown. region. There was loss of sweating on left side of body It has been associated with multifactorial etiologies with hyperhidrosis on right side of body. On starch- like autoimmunity, viral infections, genetic factors iodine test, patient had anhidrosis on left side of body 1,3-6 and autonomic degeneration. The segmental (Fig. 1). hypo-/anhidrosis leads to compensatory segmental hyperhidrosis, which further causes disturbed On central nervous system (CNS) examination, left thermoregulation producing hyperthermia. pupil was dilated and poorly reactive to light (Fig. 2), whereas right pupil was normal in size and reactive There are very few reported cases of Ross syndrome to light, though accommodation reflex was normal from India till date. bilaterally. There was no ptosis and nystagmus and CASE REPORT fundus examination was normal. A 30-year-old male patient, a known case of vitiligo over face without any other chronic medical illness, presented with complaints of absence of sweating, burning sensation in left half of body, increased sweating in right half of body, tiredness and heat intolerance for last 2 years. There was no history suggestive of orthostatic hypotension, headache, syncope, palpitations, dyspnea *Senior Resident Neurology, Dept. of Neurology †3rd Resident Pathology, Dept. of Pathology SMS Medical College, Jaipur, Rajasthan Address for correspondence Dr Chandrajeet Singh Ranawat Quarter No. B, Old MREC Campus Gandhi Nagar, Jaipur, Rajasthan - 302 015 Figure 1. Starch-iodine test: Increased sweating on right side E-mail: [email protected] of body and decreased sweating on left side of body. 628 IJCP SUTRA: "Do not eat leftover food for more than 2 hours at room temperature." Indian Journal of Clinical Practice, Vol. 30, No. 7, December 2019 CASE REPORT sodium - 139 mEq/L [136-145 mEq/mL], SGOT - 30U/L [0-35 U/L], SGPT - 28 U/L [0-35 U/L], bilirubin - 0.9 mg/ dL [0.3-1.2 mg/dL]) were normal. Screening tests for autoimmune diseases (antinuclear antibody [ANA], anti-SS-A and anti-SS-B) were negative. Urine examination was normal. Chest X-ray, ultrasound abdomen and pelvis, spine X-ray and Figure 2. Asymmetric pupil, dilated left side pupil as magnetic resonance imaging (MRI) of cervical spine compared to right side pupil with periorbital vitiligo patches. and brain were normal. Nerve conduction study was normal. Cerebrospinal fluid routine examination was normal and viral polymerase chain reaction (PCR) for herpes simplex, cytomegalovirus, Epstein-Barr virus was negative. Biopsy done from the hypohidrotic skin on left side showed thinned out epidermis with total absence of eccrine sweat glands (Fig. 3). The patient was advised to avoid heat exposure and anticholinergic medications. Wet clothes were recommended during physical activities to prevent episodic hyperthermia. DISCUSSION The typical presentation of anhidrosis, tonic pupil and absent deep tendon reflexes with abnormal autonomic nervous system testing established the diagnosis of Ross syndrome in this patient. Anhidrosis with defective thermoregulation can lead to periodic hyperthermia Figure 3. Microscopically total absence of eccrine sweat that could be life-threatening. glands. Damage to postganglionic sympathetic fibers to sweat glands leads to segmental and progressive hypohidrosis.7 On motor system examination, bulk, power, tone were Multiple system atrophy, multiple sclerosis, diabetes normal; deep tendon reflexes were absent and bilateral mellitus, leprosy and polyneuropathies are the plantars were flexor. Sensory system examination was normal and cerebellar signs were absent. differential diagnoses of anhidrosis and need to be ruled out. On autonomic system examination, there was no postural drop in blood pressure. On standing, 30/15 No specific treatment is available for this disease. RR interval ratio on ECG was <1 (normal value = 1.04). Treatment depends on symptomatology. If Sympathetic skin response in upper limbs showed hyperhidrosis is the main complaint, then botulinum 8 9 prolonged latency suggestive of small fiber neuropathy. toxin, iontophoresis, aluminum chloride, 0.5% glycopyrrolate,10 thoracic sympathectomy11 and Investigations systemic anticholinergics can be tried with varying results. For management of hypohidrosis, avoiding hot Routine investigations including hematological environment and wearing wet clothes during strenuous (Hb - 14.0 g/dL [12-15.5 g/dL], WBC count - 6,410 activities is advised. cells/dL [4,000-11,000 cells/dL], MCV - 85.7 fL, platelet count - 2.62 lac/mL [1.5-4.5 lac/mL]) and biochemistry CONCLUSION profile (urea - 23 mg/dL [8-45 mg/dL], creatinine - 1.00 mg/dL [0.7-1.3 mg/dL], fasting blood sugar - 79 mg/dL Ross syndrome is a rare and progressive disorder of [<100 mg/dL], TSH - 1.63 mu/mL [0.5-5.0 mu/mL], vitamin unknown etiology. Treatment options are limited but B12 level - 645 pg/mL [211-800 pg/mL], serum correct diagnosis helps to alleviate the anxiety of patient. IJCP SUTRA: "Cheaper fruits and vegetables brought in morning from abundant stock will always be fresh, locally grown and seasonal." 629 CASE REPORT Indian Journal of Clinical Practice, Vol. 30, No. 7, December 2019 REFERENCES spinal deformity: a Ross’s syndrome variant or inverse Horner’s syndrome? Indian J Dermatol Venereol 1. Ross AT. Progressive selective sudomotor Leprol. 2004;70(1):29-32. denervation; a case with coexisting Adie’s syndrome. Neurology. 1958;8(11):809-17. 7. Wolfe GI, Galetta SL, Teener JW, Katz JS, Bird SJ. Site of autonomic dysfunction in a patient with Ross’ 2. Nolano M, Provitera V, Perretti A, Stancanelli A, syndrome and postganglionic Horner’s syndrome. Saltalamacchia AM, Donadio V, et al. Ross syndrome: Neurology. 1995;45(11):2094-6. a rare or a misknown disorder of thermoregulation? A skin innervation study on 12 subjects. Brain. 2006; 8. Bergmann I, Dauphin M, Naumann M, Flachenecker P, 129(Pt 8):2119-31. Müllges W, Koltzenburg M, et al. Selective degeneration of sudomotor fibers in Ross syndrome and successful 3. Luong M, Jomir L, Labauge P, Dandurand M, Meunier L, Stoebner PE. Ross syndrome with sweating anomaly treatment of compensatory hyperhidrosis with botulinum associated with Sjögren syndrome: an infrared thermo- toxin. Muscle Nerve. 1998;21(12):1790-3. graphic case study. Acta Derm Venereol. 2011;91(1):80-1. 9. Reinauer S, Schauf G, Hölzle E. Ross syndrome: treatment 4. Vasudevan B, Sawhney M, Vishal S. Ross syndrome with of segmental compensatory hyperhidrosis by a modified ANA positivity: a clue to possible autoimmune origin iontophoretic device. J Am Acad Dermatol. 1993;28(2 Pt 2): and treatment with intravenous immunoglobulin. Indian 308-12. J Dermatol. 2010;55(3):274-6. 10. Bajaj V, Haniffa M, Reynolds NJ. Use of topical 5. Nagane Y, Utsugisawa K. Ross syndrome associated with glycopyrrolate in Ross syndrome. J Am Acad Dermatol. cytomegalovirus infection. Muscle Nerve. 2008;38(1): 2006;55(5 Suppl):S111-2. 924-6. 11. Baran A, Balbaba M, Demir CF, Ozdemir HH. A case of 6. Sawhney MP, Sharma YK, Singh N. Segmental Ross syndrome presented with Horner and chronic cough. anhidrosis with hyporeflexia associated with congenital J Neurosci Rural Pract. 2014;5(4):394-7. ■ ■ ■ ■ Omega-3 Oils Boost Attention as Much as ADHD Drugs in Some Children Omega-3 fish oil supplements can improve attention in children with attention deficit hyperactivity disorder (ADHD) just as much as drug treatments, but only in those whose blood levels of omega-3 are low, trial results showed recently. In this study, published in the journal Translational Psychiatry, researchers conducted a randomized controlled trial with 92 children aged 6-18 with ADHD. Children with the lowest blood levels of eicosapentaenoic acid (EPA) showed improvements in focused attention and vigilance after taking the omega-3 supplements. While the amount of improvement in attention and vigilance from methylphenidate is generally 0.22-0.42, the effect seen in children with low levels of EPA in the trial was larger - at 0.89 for focused attention and 0.83 for vigilance… (Reuters) Sleeve Gastrectomy Beneficial at Lower BMI In a state-wide registry study of patients who had sleeve gastrectomy, less obese individuals — those with an initial body mass index (BMI) <35 kg/m2 — had similar and even sometimes better early outcomes compared to patients with a BMI ≥35 kg/m2, researchers report. At 1 year, more than half of patients in both groups discontinued medications for diabetes, hypertension or hyperlipidemia, and patients in both arms had similar complication rates, investigators said at a session of top papers of the American Society of Metabolic and Bariatric Surgery (ASMBS), during Obesity Week 2019. Statins Not Linked to Cognitive Decline, Study Shows Statins were not linked to any greater decline in cognition or memory over 6 years, a longitudinal study of elderly adults in Australia showed.
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