Screening for Hydroxychloroquine Retinal Toxicity in Indian Patients.

OBJECTIVE: Several studies have reported hydroxychloroquine (HCQ) retinal toxicity in East Asian patients. However, at present, there are limited data from Indian patients. The aim of this study was to investigate the prevalence and pattern of HCQ retinal toxicity in Indian population and to see if screening protocol for retinal toxicity in Indian patients should be any different to others. METHODS: This was a cross-sectional study involving adult patients with autoimmune rheumatic diseases who had received HCQ for at least 5 years or a cumulative dose of at least 500 g. Retinal toxicity was evaluated using fundus examination, visual fields 10-2 and 30-2 protocol, and spectral domain optical coherence tomography. RESULTS: Of 110 patients screened, retinal toxicity was found in 7 patients (6.36%). A parafoveal pattern was found in 4 patients, whereas a mixed parafoveal and perifoveal pattern was found in 3 patients. None of the patients had isolated perifoveal pattern. Except for the one patient, all the patients with retinal toxicity had more than 10 years (mean, 13 ± 4.89 years; range, 5-20 years) of HCQ usage with a mean cumulative dose of 1573.7 ± 771.5 g. The mean daily dose was 5 ± 1.6 mg/kg per day. CONCLUSIONS: Hydroxychloroquine retinal toxicity is more common than previously recognized in patients who have used the drug for more than 5 years. The toxicity manifests as a parafoveal or a mixed parafoveal and perifoveal pattern in Indian patients.

Anti-drug antibodies and low serum trough infliximab levels correlate with disease activity measures in spondyloarthritis patients on an as-needed infliximab treatment.

AIM: In India, many centers use infliximab at lower doses of 3-5 mg/kg without the loading dose for spondyloarthritis (SpA) patients. It is then continued on an as-required basis, rather than a fixed schedule. Our study was undertaken to see if the trough drug levels and anti-drug antibodies in patients with SpA treated with as-needed infliximab dosing correlated with the disease activity measures. METHODS: Thirty-five adult SpA patients in the age group 18-70 years were recruited. They had received three or more infusions of infliximab at 3-5 mg/kg over the past 6 to 12 months. Patient's serum tumor necrosis factor-α, trough infliximab levels and anti-drug antibodies were measured by enzyme-linked immunosorbent assay technique. The disease activity was quantified by Ankylosing Spondylitis Disease Activity Score - erythrocyte sedimentation rate/ C-reactive protein (ASDAS-ESR/CRP) scores. Correlation between quantitative variables was analyzed by the Spearman's correlation assay. The difference in mean trough infliximab and ASDAS between the drug antibody positive and negative patients was assessed using the Mann-Whitney U test. RESULTS: There was a significant negative correlation between the trough infliximab levels and the ASDAS-ESR (rs = -0.57, P < 0.01) and ASDAS-CRP scores (rs = -0.53, P < 0.01). Anti-drug antibodies were positive in 68.7% of the patients and in comparison to the antibody negative patients, had significantly higher ASDAS-ESR and ASDAS-CRP scores. CONCLUSIONS: Spondyloarthritis patients on low-dose, as-needed infliximab therapy, have both the trough infliximab and anti-drug antibodies correlate significantly with the measures of disease activity. We hypothesize that trough infliximab levels and anti-drug antibodies may be used to predict a suboptimal response due to secondary resistance in SpA patients.

Distal cord-predominant longitudinally extensive myelitis with diffuse spinal meningitis and dural abscesses due to occult tuberculosis: A rare occurrence.

Tuberculous myelitis usually involves thoracic and only rarely, distal cord. Longitudinal lesions more than three spinal segments long in tuberculosis (TB) are usually due to intramedullary tuberculomas and not infectious myelitis. We report a 17-year-old male with acute myelitis from D7 to conus medullaris, diffuse spinal meningitis, subdural and epidural abscesses, normal vertebrae, intervertebral discs, and brain imaging. Cerebrospinal fluid (CSF) showed raised proteins, lymphocytosis, hypoglycorrhagia, and positive TB-polymerase chain reaction. Chest X-ray was normal, and sputum was negative for acid-fast Bacilli. Chest computed tomography (CT) revealed endobronchial TB. The patient was successfully treated with antitubercular drugs and steroids. In endemic areas, a high index of suspicion should be kept for TB in patients with myelitis, especially those with spinal abscesses and a suggestive CSF report. In selected cases, there may be a role of CT scan inspite of normal X-ray.

A rare case of occult abdominal tuberculosis with Poncet's disease mimicking Adult onset Still's disease.

A 50-year-old female presented with fever, symmetrical arthralgias, rash, painful oral ulcerations and alopecia since 8 weeks. Examination showed mild hepatospleenomegaly. Investigations revealed leucocytosis, neutrophilia, elevated sedimentation rate and raised ferritin levels (3850 ng/ml). Computerized tomography (CT) abdomen showed hepatospleenomegaly, mild ascitis and mild bilateral pleural-effusion. After ruling out occult infections, tuberculosis, malignancies and autoimmune diseases by appropriate investigations, and due to raised ferritin levels, adult onset stills disease (AOSD) was diagnosed. Patient responded to oral steroids initially, but after 7 days developed severe abdominal pain. Repeat CT showed multiple enlarged, necrotic and matted retroperitoneal lymph nodes with caseating granuloma on histopathology suggesting tuberculosis. Patient was given four-drug anti-tubercular treatment and she improved. Thus our patient of occult abdominal tuberculosis with reactive arthritis (Poncet's disease) presented with hyperferritinemia mimicking AOSD. We postulate that extreme hyperferritinemia can be seen in tuberculosis and tuberculosis must be conclusively ruled out before diagnosing AOSD in tropics.

Polyneuritis cranialis with generalized hyperreflexia as a presenting manifestation of thyrotoxicosis.

A 22-year-old male student with no past medical illness, presented with acute onset dysarthria, binocular diplopia, and dysphagia over 10 hours. On examination, he had tachycardia, hypertension, generalized hyper-reflexia, and bilateral pupil sparing oculomotor, troclear, abducens, trigeminal, facial, glossopharyngeal, and vagus nerve palsy. Rest examination was unremarkable. Facial nerve conduction study (NCS) showed decreased amplitude bilaterally and neurogenic pattern on electromyography. Limb NCS, repetitive nerve stimulation, neostigmine test, brain magnetic resonance imaging, cerebrospinal fluid, and biochemical tests were normal. Only positive tests were low thyroid-stimulating hormone (TSH) (<0.01), high free T3 (19.2 pmol/L), and high free T4 (39.2 pmol/L). Thyroid ultrasonography, anti-thyroid peroxidase, and anti-thyroglobulin antibody were normal. Patient was treated with anti-thyroid drugs, with which he completely recovered in 2 months. Though many cases with thyrotoxic myopathy have been reported, only few mention neuropathic cause of dysphagia or polyneuritis cranialis. Getting done thyroid function tests may be helpful in patients with polyneuritis cranialis of uncertain etiology.

Post-Plasmodium vivax malaria cerebellar ataxia and optic neuritis: A new form of delayed cerebellar ataxia or cerebellar variant of acute disseminated encephalomyelitis?

Acute disseminated encephalomyelitis (ADEM) is commonly seen after viral and bacterial infections, immunization, and Plasmodium falciparum (PF) malaria. Plasmodium vivax (PV) rarely causes ADEM. We report a 14-year-old female patient who presented with acute onset bilateral cerebellar ataxia and optic neuritis, 2 weeks after recovery from PV. Magnetic resonance imaging showed bilateral cerebellar hyperintensities suggestive of ADEM. No specific viral etiology was found on cerebrospinal fluid examination. Patient responded well to treatment without any sequelae. Thus, PV too is an important cause of ADEM along with PF. Two of the previously reported cases had co-infection with falciparum malaria. The only other two reported cases, as also this patient, are from Asia. A geographical or racial predisposition needs to be evaluated. Also, a possibility of post-PV delayed cerebellar ataxia, which is classically described post-PF infection, may be considered as it may be clinically, radiologically, and prognostically indistinguishable from a milder presentation of ADEM.

Guillain-Barre syndrome in acute lymphoblastic leukemia: Causal or coincidental.

Guillain-Barre syndrome (GBS) is rarely reported in children with acute lymphoblastic leukemia (ALL) and may be difficult to differentiate from vincristine induced neuropathy. Only few case reports highlighted GBS with ALL. We report a 10-year-old male child who was a diagnosed case of ALL since 3 month on chemotherapy. At 3(rd) week of chemotherapy, he developed rapidly progressive ascending motor quadriparesis over 2 days. Clinical and electrophysiology revealed acute motor axonal neuropathy (AMAN) variant of GBS. He was treated with intravenous immunoglobulin (2 g/kg) without discontinuing chemotherapy. Complete recovery took 12 weeks despite immunotherapy, and it was corroborating to slow remission. We concluded that AMAN variant is usually present in B-cell type ALL, may be causal for GBS and it takes 6-16 weeks to complete recovery which may correspond to remission of ALL. However, it needs to be studied. We also present a meta-analysis of previously reported cases of GBS in ALL.

Carpal tunnel syndrome: Analyzing efficacy and utility of clinical tests and various diagnostic modalities.

BACKGROUND: Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy, but not adequately studied in India. OBJECTIVES: To study clinical tests, nerve conduction studies (NCS), ultrasonography (USG), and magnetic resonance imaging (MRI) in diagnosing CTS. MATERIALS AND METHODS: We diagnosed CTS in 54 patients (93 hands) out of 60 screened patients with symptoms compatible with CTS, including 19 control patients (23 hands). We conducted provocative tests and calculated Boston Carpal tunnel Questionnaire (BCTQ) symptom (S) and function (F) scores. NCS positive patients were classified into mild, mild-to-moderate, moderate, severe, and all-CTS groups. Median nerve anteroposterior, transverse, circumference (CIR), and cross-sectional area (CSA) at inlet (I), middle (M), and outlet (O) each was measured by USG in all patients. MRI was done in 26 patients (39 hands). RESULTS: Phalen, hand elevation and pressure provocation tests had higher sensitivity, Tinel's test had higher specificity and tethered median nerve and tourniquet tests had low sensitivity and moderate specificity. USG had low sensitivity but high specificity, and MRI had moderate sensitivity. USG in patients compared to controls was significantly abnormal in CSA-I, CIR-I, and CSA-O. Significant correlation was found between BCTQ-S and NCS and BCTQ-S and CIR-O. CIR-M, CIR-O, CSA-M, and CSA-I had correlation with NCS. MRI was significant in moderate and in moderate + severe groups combined and associated pathologies were detected in 59% patients. CONCLUSION: NCS remain gold standard but USG and MRI help increase sensitivity and detect mass lesions amenable to surgery.

Disseminated neurocysticercosis presenting as isolated acute monocular painless vision loss.

Neurocysticercosis, the most common parasitic infection of the nervous system, is known to affect the brain, eyes, muscular tissues and subcutaneous tissues. However, it is very rare for patients with ocular cysts to have concomitant cerebral cysts. Also, the dominant clinical manifestation of patients with cerebral cysts is either seizures or headache. We report a patient who presented with acute monocular painless vision loss due to intraocular submacular cysticercosis, who on investigation had multiple cerebral parenchymal cysticercal cysts, but never had any seizures. Although such a vision loss after initiation of antiparasitic treatment has been mentioned previously, acute monocular vision loss as the presenting feature of ocular cysticercosis is rare. We present a brief review of literature along with this case report.

Neuroretinitis with abnormal brain imaging in Ask-Upmark kidney: A novel case report.

We report a 13-year-old female patient having vertigo and visual blurring since 2 weeks with blood pressure being 180/106 mmHg. Fundus examination showed optic disc edema with macular star. After ruling out infective causes, idiopathic neuroretinitis was diagnosed. Her brain magnetic resonance imaging (MRI) scan revealed three periventricular morphologically multiple sclerosis (MS)-like white matter lesions. Renal ultrasound and renal arteriogram showed a shrunken left kidney, small non-stenotic left renal artery and reduced vessels in upper pole of left kidney, consistent with Ask-Upmark kidney (AUK). Her symptoms improved with antihypertensive drugs. Follow-up MRI at 1 year revealed no interval change, while fundus had normalized. Neuroretinitis typically has normal brain MRI and rules out MS. However, our patient having AUK-induced hypertension had neuroretinitis and MS-like brain lesions and did not fulfill diagnostic criteria for MS. Thus we postulate that MS-like lesions can be part of neuroretinitis, especially in hypertensive patients.