Myositis in H1N1 Infection Compounds to Myasthenic Crisis.

ABSTRACT: Infection is an important trigger of myasthenic crisis (MC), and those infections manifest with pneumonia and muscle involvement may result in more frequent MC. We report two myasthenia gravis (MG) patients with H1N1 infection, and highlight the reasons for deterioration. Two patients with MG had H1N1 infection. The diagnosis of MG was confirmed by neostigmine, repetitive nerve stimulation, and anti-acetylcholine receptor antibody tests. H1N1 was confirmed by nucleic acid detection study, and myositis by creatinine kinase. The patient with pneumonia and myositis had MC needing mechanical ventilation for 10 days, and the other patient without myositis did not have MC. They were treated with oseltamivir 75 mg twice daily for 5 days, and the patients with MC received ceftriaxone intravenously. Both the patients were on prednisolone and azathioprine, and none received prior H1N1 vaccination. The lady with MC with myositis was discharged on day 27 in wheelchair bound state, and the other one patient without myositis or MC was discharged on 6th day with full recovery. These patients highlight the need for evaluation for myositis along with pneumonia in the MG patients with H1N1 infection. Vaccination in MG patients on immunosuppression may be useful.

Azathioprine-induced Veno-occlusive Hepatotoxicity in a Patient with Myasthenia Gravis.

INTRODUCTION: Myasthenia gravis (MG) is an autoimmune disorder of post-synaptic neuromuscular junction characterised by fatigable muscle weakness and is treated with prednisolone with or without other immunosuppressants, including azathioprine (AZA). Veno-occlusive hepatotoxicity of AZA is a rare complication in MG. CASE REPORT: We report a 35-year-old man with MG who was treated with pyridostigmine, prednisolone, and AZA for 5 years. He presented with abdominal pain and increased fatiguability for 7 days. His serum bilirubin and liver enzymes were elevated, and ultrasound revealed a dilated hepatic vein and portal vein suggestive of veno-occlusive liver disease. The clinical symptoms, liver functions, and ultrasound of the hepatobiliary system normalized after withdrawal of AZA. CONCLUSION: A possibility of AZA veno-occlusive hepatoxicity should be considered in an MG patient if presented with abdominal pain, elevated bilirubin and transaminases and ultrasound showing dilatation of hepatic veins. Physicians should be aware of this complication because this toxicity is reversible following dose reduction or withdrawal of AZA.

Tizanidine Induced Hypotension: Report of a Case and Review of the Literature.

INTRODUCTION: Spasticity is a common sequelae of stroke, and often these patients receive anti-spastic drugs such as baclofen or tizanidine. Stroke patients have multiple co-morbidities such as hypertension, diabetes, and seizure. Tizanidine is an α2 and imidazole receptor agonist at a spinal and supraspinal level resulting in reduced central sympathetic outflow and causing hypotension rarely, especially in those receiving beta-blockers or angiotensin-converting enzyme inhibitors. CASE PRESENTATION: We report a 56-year-old hypertensive male presenting with altered sensorium who had recurrent intracerebral hemorrhage with left spastic hemiplegia and focal seizures. He was on amlodipine, atenolol, telmisartan and oxcarbazepine. After 3 doses of tizanidine 2mg, his blood pressure dropped from 140/90 to 80/40 mmHg and pulse from 82 bpm to 44 bpm. His blood counts, serum chemistry, procalcitonin, and Trop I were normal. ECG revealed sinus bradycardia. After 8 hours of withdrawing tizanidine, his blood pressure became 110/70 mmHg, and on the next day, it became 140/82 mmHg. His attendants were taught physiotherapy to minimize spasticity. CONCLUSION: This patient highlights the need for close monitoring of patients receiving tizanidine co-medication with angiotensin-converting enzyme inhibitors and angiotensin receptor blockers. These drugs have a synergistic effect on reducing the renin-angiotensin-aldosterone system, thereby hypotension and bradycardia.

mRNA profiling of cytokines to understand paradoxical response in HIV-uninfected tuberculous meningitis.

Paradoxical reaction (PR) in tuberculous meningitis (TBM) is a major management issue. We report mRNA profiling of cytokines to understand PR in HIV-uninfected TBM patients. 72 patients with TBM were included, and their clinical, MRI, and mRNA profiling of tumor necrosis factor (TNF) α, interleukin (IL) 6, IL10 and interferon (IFN) γ genes in the peripheral blood mononuclear cells were done at admission and 6 weeks of antitubercular treatment. Cytokine profiling was done using reverse transcriptase polymerase chain reaction. PR was defined if repeat MRI at 6 weeks revealed new or increase in exudates, tuberculoma, hydrocephalus or infarctions. Outcome was defined at 6 months using modified Rankin Scale (mRS), and categorized as death, poor and good. 44 (61.1 %) patients had PR, and 28 (38.9 %) had paradoxical tuberculoma (PT). The expression of IL6 and TNFα genes were higher in PR and PT groups. Stage of meningitis and hydrocephalus at admission predicted PR. Patients with PR and PT had more frequently poor outcome. About three-fifth HIV-uninfected TBM patients have PR and two-fifth have PT. Paradoxical reaction is associated with higher expression of IL6 and TNFα. Patients with severe meningitis with hydrocephalus develop PR more frequently.

Stunting and wasting in neurological Wilson disease: Role of copper, zinc, and insulin-like growth factor-I.

OBJECTIVES: Copper (Cu) and zinc (Zn) are important trace elements for the growth and development of children. In Wilson disease (WD), impaired Cu metabolism may affect growth. This study was conducted to evaluate the height and weight of children with neurological WD and correlate these with serum Cu, Zn, and insulin-like growth factor-I (IGF-I). METHODS: This prospective cohort study was conducted in a tertiary care teaching institute. Children with neurologic WD were included. The height, weight, and body-mass index of each child were measured and categorized according to the revised national growth chart. Serum Cu, Zn, calcium, alkaline phosphatase, albumin, thyroid-stimulating hormone, and urinary-Cu were measured. Serum IGF-1 was measured by enzyme-linked immunosorbent assay. The relationship between height and weight with trace elements and IGF was analyzed using parametric or non-parametric tests. RESULTS: There were 52 children (5-18 years) with neurologic WD. Thirty-six (69.2%) children had normal height, 12 (23.1%) were tall, and 4 (7.7%) were stunted. Forty-six (88.5%) children had normal weight and six (11.5%) children were underweight. IGF-1 correlated with height, weight, duration of treatment, and serum Zn level. About 15.4% of children had stunting and/or wasting, which was associated with low levels of serum IGF-I, Zn, and calcium. CONCLUSIONS: Stunting and/or wasting occurs in 15.4% of children with neurologic WD and is associated with reduced serum IGF-I, Zn, and calcium concentration. Adjunctive Zn and calcium treatment may help in achieving normal growth.

Role of Nerve Conduction Studies in Hansen's Disease.

Background and Objective: To report the role of nerve conduction study (NCS) in diagnosis, monitoring, and prognosis of Hansen's disease (HD). Materials and Methods: In a hospital-based prospecive observational study, the patients with HD as per World Health Organization (WHO) criteria were included; muscle wasting power, reflexes, and sensations were recorded. Motor NCS of median, ulnar, and peroneal nerves and sensory NCS of ulnar, median, and sural nerves were recorded. Disability was graded using WHO grading scale. The outcome was assessed after 6 months using modified Rankin scale. Results: In the present study, 38 patients with a median age of 40 (15-80) years and five females were included. The diagnosis was tuberculoid in seven, borderline tuberculoid in 23, borderline lepromatous in two, and borderline in six patients. The disability was grade 1 and 2 in 19 patients each. Out of 480 nerves studied, NCS was normal in 139 sensory (57.4%) and 160 (67.2%) motor nerves. NCSs were axonal in seven sensory and eight motor nerves, demyelinating in three nerves, and mixed in one in seven patients who had lepra reaction. NCS findings did not correlate with disability (p = 1.0) or outcome (0.304) and provided additional information in 11 nerves (seven patients). Peripheral nerves were enlarged in 79. NCSs were normal in 32 (29.90%) in thickened nerves. Conclusion: In HD, NCS abnormalities correlated with respective sensory or motor abnormality but related with neither disability nor the outcome.

Topographic localization of migraine triggers and its association with headache frequency and severity.

OBJECTIVE: Internal biological rhythm with or without external trigger may precipitate migraine. Classifying exogenous and endogenous triggers to a topographic localization may help in understanding the migraine. We report topographic localization of migraine triggers and its influence on headache frequency and severity. METHODS: 588 migraineurs, aged 16-69 years were included. Various endogenous and exogenous triggers were categorized to topographic localization- hypothalamic, pituitary, auditory, visual, somato-sensory, olfactory and gustatory. The relationship of topographic localization of triggers with episodic versus chronic migraine, and moderate versus severe headache were analyzed using univariate followed by multivariate analysis. RESULTS: All migraineurs had triggers 584(99.9%) except 4(0.1%) patients. Presence of multiple triggers (99.4%), and combination of both endogenous and exogenous triggers (97.7%) was the rule. On topographic localization, hypothalamic trigger was the commonest (98.1%) followed by visual (84.1%), auditory (82.1%), somatosensory (76.1%), olfactory (26.2%), pituitary (24.1%), and gustatory (6.6%). 98.6% patients had combination of hypothalamic with pituitary triggers. Hypothalamic [Adjusted odds ratio (AOR) 4.50] and auditory triggers (AOR 0.34) independently predicted chronic migraine, and auditory (AOR 0.55) and gustatory (AOR 2.41) triggers predicted severity of headache. CONCLUSION: Hypothalamic triggers are the commonest suggesting an innate susceptibility of migraine. Auditory trigger may precipitate frequent and severe headache.

Prednisolone 20 mg vs 40 mg in complex regional pain syndrome type I: A randomized controlled trial.

BACKGROUND: High dose of corticosteroid has been found beneficial in complex regional pain syndrome type I (CRPS-I). We report the efficacy and safety of prednisolone 20 mg versus 40 mg in CRPS-I in an open label randomized controlled trial. METHODS: The patients with CRPS-I of the shoulder joint with a CRPS score of ≥8 were included. Their demographic details, comorbidities, and underlying etiology were noted. The severity of CRPS was assessed using a 0-14 CRPS scale, the pain using a 0-10 Visual Analogue Scale (VAS), and sleep quality using a 0-10. Daily Sleep Interference Scale (DSIS). Patients were randomized to prednisolone 40 mg/day (group I) or 20 mg/day (group II) for 14 days, then tapered to 10 mg in group I and to 5 mg in group II by 1 month. Thereafter both groups received prednisolone 5 mg/day for 2 months. The primary outcome was a >50% reduction in VAS score, and secondary outcomes were a reduction in CRPS score, DSIS score, and adverse events. RESULTS: Fifty patients were included, and their baseline characteristics were comparable. At one month, all the patients had >50% reduction in the VAS score. The effect size was 0.38 (95% CI 0.93-0.20; p = 0.20). On the Kaplan-Mayer analysis, the improvement in the VAS score (Hazard ratio-1.43, 95 % CI-0.80-2.56, p = 0.22) and the CRPS score (HR-0.79,95 % CI-0.45-1.39; p = 0.41) was insignificant between the two groups. The DSIS score improved in group II (HR-1.85,95 % Cl-1.04-3.31,p = 0.04). Group I patients needed frequent adjustment of antidiabetic drugs (14 vs 6; p = 0.04). CONCLUSION: The efficacy of prednisolone 20 mg is not inferior to 40 mg in CRPS-I, and is safe in diabetic patients. LIMITATIONS: This is an open label randomized controlled trial with small sample size without a placebo arm.

A Study of Dopaminergic Pathway in Neurologic Wilson Disease with Movement Disorder.

Movement disorder (MD) is an important manifestation of neurologic Wilson disease (NWD), but there is a paucity of information on dopaminergic pathways. We evaluate dopamine and its receptors in patients with NWD and correlate the changes with MD and MRI changes. Twenty patients with NWD having MD were included. The severity of dystonia was assessed using BFM (Burke-Fahn-Marsden) score. The neurological severity of NWD was categorized as grades I to III based on the sum score of 5 neurological signs and activity of daily living. Dopamine concentration in plasma and CSF was measured using liquid chromatography-mass spectrometry, and D1 and D2 receptor expression at mRNA by reverse transcriptase polymerase chain reaction in patients and 20 matched controls. The median age of the patients was 15 years and 7 (35%) were females. Eighteen (90%) patients had dystonia and 2 (10%) had chorea. The CSF dopamine concentration (0.08 ± 0.02 vs 0.09 ± 0.017 pg/ml; p = 0.42) in the patients and controls was comparable, but D2 receptor expression was reduced in the patients (0.41 ± 0.13 vs 1.39 ± 1.04; p = 0.01). Plasma dopamine level correlated with BFM score (r = 0.592, p < 0.01) and D2 receptor expression with the severity of chorea (r = 0.447, p < 0.05). The neurological severity of WD correlated with plasma dopamine concentration (p = 0.006). Dopamine and its receptors were not related to MRI changes. The central nervous system dopaminergic pathway is not enhanced in NWD, which may be due to structural damage to the corpus striatum and/or substantia nigra.

Effect of Exercise on ß-Endorphin and Its Receptors in Myasthenia Gravis Patients.

This is a prospective observational study evaluating the change in ß-endorphin (BE) and its receptors following exercise in patients with myasthenia gravis (MG) and their association with clinical improvement. Fifteen patients with mild to moderate MG, aged 16-70 years, who were able to do 6-Minute Walk Test (6-MWT) and had MG Quality of Life-15 (MGQoL-15) ≤ 45 without any contraindication for exercise were included. The patients walked 30 min daily for 3 months. The primary outcome at 3 months was > 50% improvement in MGQoL-15 from the baseline, and the secondary outcomes were MG Activities of Daily Living (MGADL), Hospital Anxiety and Depression Scale (HADS), number of steps, and distance covered on 6-MWT and adverse events. Plasma BE level, µ-opioid receptor (MOR), and δ-opioid receptor (DOR) were measured on admission and at 1 and 3 months. Twelve age- and gender-matched healthy controls who were not on regular exercise were included for comparison of BE, MOR, and DOR levels. Plasma BE level (P = 0.007) and DOR expression (P = 0.001) were lower in MG patients compared to the healthy controls. After 3 months of exercise, 6 patients improved. Plasma BE, MOR, and DOR levels increased in the first and decreased in the third month. MGQoL-15 (P < 0.001), HADS (P < 0.0001), number of steps (P < 0.007), distance (P = 0.030), and MGADL (P < 0.001) significantly improved compared to baseline. At 3 months, MGQoL-15 was associated with HADS score (P = 0.001), reduced depression (P = 0.013), MGADL (P = 0.035), and distance travelled on the 6-WMW test (P = 0.050). The improvement in depression was associated with higher BE level.

A Comparative Study of Diagnosis and Treatment of Pott's Spine Amongst Specialists and Super Specialists in India.

Background and Objective: There is a paucity of guidelines about the diagnosis and management of Pott's spine. In this study, we report the pattern of practice of diagnosis and treatment of Pott's spine among the specialists and super-specialists in India. Subject and Methods: Response to a 22-item questionnaire regarding the diagnosis and treatment of Pott's spine has been reported. The responses were compared between medical and surgical specialists, residents and consultants, and specialists and super-specialists. There were 84 responders: 42 physicians and 42 surgeons; 48 residents and 36 faculty or consultants; 53 specialists and 31 super-specialists. Results: Thirty-eight responders rarely recommended biopsy whereas others recommended biopsy more frequently, especially the surgeons (P < 0.007). Twenty-five responders recommended immobilization even in an asymptomatic patient whereas 38 would immobilize those with neurological involvement only. All but 4 responders would repeat imaging at different time points. The response of medical treatment was judged at 1 month by 53, and 3 months by 26 responders. Surgery was recommended in a minority of patients-in those with neurological involvement or abscess. Surgeons more frequently biopsied, immobilized the patients, and recommended surgery compared to the physicians. The residents also recommended biopsy and recommended immobilization more frequently compared to consultants or faculty members. Super-specialists more frequently recommended biopsy compared to specialists. Conclusion: There is marked variation in investigations and treatment of Pott's spine patients, suggesting the need for consensus or evidence-based guidelines.

Prognostic Significance of Serial Nerve Conduction in GB Syndrome.

Background and Aims: We evaluated dynamic changes in neurophysiology of Guillain-Barré syndrome (GBS) at different time points and the role of demyelination and axonal burden in predicting outcome. Methods: Nerve conduction study (NCS) was done in 44 GBS patients at admission and at 1 and 3 months, and were categorized into acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), equivocal and in-excitable motor nerve (IMN). The demyelinating and axonal burden on motor NCS at admission, 1 and 3 months were computed and correlated with disability at 3 and 6 months. Disability was assessed using Clinical Grading Scale. Results: Twenty-four (54.3%) had AIDP, 5 (11.4%) AMAN, 12 (27.3%) equivocal and 3 (6.8%) had IMN at admission. Maximum instability was noted in equivocal group; majority of whom became AIDP at three months. Neurophysiological subtypes at different time points did not correlate with 6 months disability, but demyelination burden at admission (r = -0.42; P = 0.005) and axonal burden at one month (r = 0.43; P = 0.04) correlated with six months disability. Conclusion: Inverse correlation of axonal burden at one and three months with disability suggests role of secondary axonal damage in predicting outcome. Repeat NCS at one month helps in categorizing GBS and also in prognostication.

Treatment response and complications during management of Pott's spine.

The global incidence of TB in 2016 was 10.4 million and India accounts for a quarter of the global burden of TB. It is estimated that there are 2.79 million people with TB in India. About 10% of extra pulmonary TB involves bone and joints. Spinal TB accounts for half the cases of skeletal TB. The incidence of spinal TB is 1-4% of total TB cases, then it is estimated that only in India approximately 60,000 spinal TB cases exist. To report the pattern of recovery and predictors of outcome of Pott's spine. The intervention comprised of four drug antitubercular treatment, rest, immobilization, and ultrasonography or computerized tomography guided aspiration or biopsy as indicated outcome measures were six months Nurick grade, and mRS and complications like drug induced hepatitis (DIH) and paradoxical worsening. Seventy-three patients with Pott's spine, median age 36 (11-73) years, 32 (43.8%) females were included. The neurological signs were present in 44 (64.4%) patients. At six months, median Nurick grade improved from 4 to 2 and;and 70% patients had a good outcome as defined by mRS.The predictors of poor outcome were weight loss, non-ambulatory state on admission and paradoxical worsening. It is concluded that neurological involvement in Pott's spine was present in 64% patients, paradoxical worsening (deterioration in symptoms after one month of ATT) in 11% and DIH in 16%. Weight loss, non-ambulatory state on admission and paradoxical worsening predicted poor outcome.

Blood-CSF-barrier permeability in tuberculous meningitis and its association with clinical, MRI and inflammatory cytokines.

Blood -cerebrospinal fluid-barrier (BCB) disruption in tuberculous meningitis (TBM) may be mediated by inflammatory cytokines, and may determine clinico-radiological severity and outcome. We report BCB permeability in TBM and its relationship with inflammatory cytokines (TNF-α, IL-1ß and IL-6), clinical severity, MRI changes and outcome. 55 TBM patients with a median age of 26 years were included. Their clinical, cerebrospinal fluid (CSF) and MRI findings were noted. The severity of meningitis was graded into stages I to III. Cranial MRI was done, and the presence of exudates, granuloma, hydrocephalus and infarctions was noted. BCB permeability was assessed by a ratio of CSF albumin to serum albumin (Qalb). The concentration of TNF-α, IL-1ß and IL-6 in CSF were measured by cytokine bead array. The Qalb in the patients was more than the mean + 2.5 SD of controls. In TBM, Qalb correlated with TNF- α (r = 0.47; p = 0.01), CSF cells (r = 0.29; p = 0.02) and exudate on MRI (0.18 ± 0.009 Vs 0.13 ± 0.008; p = 0.04). There was however no association of Qalb with demographic variables, stage, tuberculoma, infarction and hydrocephalus. At 6 months, 11(20%) died, 10(18.2%) had poor and 34(61.8%) had a good recovery. BCB permeability in TBM correlated with TNF-α, CSF pleocytosis and exudates but not with severity of meningitis and outcome.

Racemose neurocysticercosis simulating tuberculous meningitis.

We report a patient with racemose neurocysticercosis, highlighting the diagnostic and management issues. A 37-year-old male had headaches, fever, and seizures for 8 months. He had a positive tuberculin test, cerebrospinal fluid pleocytosis, and hydrocephalus and exudates on MRI. His symptoms rapidly resolved following antitubercular and prednisolone treatment. After 2 months, he was readmitted with headache and vomiting, and his brain MRI revealed communicating hydrocephalus with a cyst in the lateral ventricle and subarachnoid space, which was confirmed as neurocysticercosis on the third ventriculostomy. The patient was managed with dexamethasone and a ventriculoperitoneal shunt. This case highlights that meningitis symptoms, CSF pleocytosis, and positive tuberculin tests may not always suggest tubercular etiology.

Oxidative stress and ER stress are related to severity of tubercular infection.

To report the markers of oxidative stress and endoplasmic reticulum (ER) stress in tuberculosis of differing severity. Ninety patients with tuberculosis, 30 each with pulmonary tuberculosis (PTB), Pott's spine (PS) and tuberculous meningitis (TBM) were included. The diagnosis and severity of the respective group was based on pre-defined criteria. Six-months outcome and complications (Hyponatremia, paradoxical worsening and Drug induced hepatitis(DIH)) were recorded. Serum Melanodehyde (MDA) , glutathione (GSH), total antioxidant capacity (TAC), ER stress markers ATF-4,GRP-78 and CHOP, were measured using spectrophotometry and real time PCR. The oxidative and ER stress markers were correlated with different subgroups, severity of TBM, complications and outcome. The severity of TBM correlated with alteration in oxidative and ER stress markers. MDA was related to hyponatremia (P = 0.045), paradoxical worsening (P = 0.035) and DIH (P = 0.038), TAC correlated with paradoxical worsening (P = 0.047) and DIH (P = 0.015). In PS, MDA correlated with paradoxical worsening (P = 0.032) and DIH (P = 0.032); and in PTB, MDA correlated with hyponatremia (P = 0.025) and DIH (P = 0.037). Changes in stress marker levels were more marked in TBM compared to PS and PTB. Outcome of TBM correlated with MDA (P = 0.002), PS to MDA(P = 0.004), TAC(P = 0.05) CHOP(P = 0.004), GRP78(P = 0.001), ATF4(P = 0.045) and PTB to MDA(P = 0.0450), TAC(P = 0.014), CHOP(P = 0.025) and GRP78(P = 0.035). Oxidative and ER stress markers seem to be related to severity of TB, its complications and outcome.

Changing Spectrum of Acute Encephalitis Syndrome in India and a Syndromic Approach.

Acute encephalitis syndrome (AES) refers to an acute onset of fever and clinical neurological manifestation that includes mental confusion, disorientation, delirium, or coma, which may occur because of infectious or non-infectious causes. Cerebrospinal fluid (CSF) pleocytosis generally favors infectious etiology, and a normal CSF favors an encephalopathy or non-infectious AES. Among the infectious AES, viral, bacterial, rickettsial, fungal, and parasitic causes are the commonest. Geographical and seasonal clustering and other epidemiological characteristics are important in clinical decision making. Clinical markers like eschar, skin rash, myalgia, hepatosplenomegaly, thrombocytopenia, liver and kidney dysfunction, elevated serum CK, fronto-temporal or thalamic involvement on MRI, and anterior horn cell involvement are invaluable clues for the etiological diagnosis. Categorizing the AES cases into neurologic [Herpes simplex encephalitis (HSE), Japanese encephalitis (JE), and West Nile encephalitis (WNE)] and systemic (scrub typhus, malaria, dengue, and Chikungunya) helps in rational utilization of diagnostic and management resources. In neurological AES, cranial CT/MRI revealing frontotemporal lesion is consistent with HSE, and thalamic and basal ganglia lesions are consistent with JE. Cerebrospinal fluid nucleic acid detection test or IgM antibody for JE and HSE are confirmatory. Presence of frontotemporal involvement on MRI indicates acyclovir treatment pending virological confirmation. In systemic AES, CT/MRI, PCR for HSE and JE, and acyclovir therapy may not be useful, rather treatable etiologies such as malaria, scrub typhus, and leptospirosis should be looked for. If smear or antigen for malaria is positive, should receive antimalarial, if negative doxycycline and ceftriaxone should be started pending serological confirmation of scrub typhus, leptospira, or dengue. A syndromic approach of AES based on the prevalent infection in a geographical region may be developed, which may be cost-effective.

Paroxysmal spinal hemidystonia in neuromyelitis optica.

Paroxysmal dystonia occurs because of genetic or structural lesion in the basal ganglia or thalamus, and there is paucity of reporting in spinal pathology. We report a patient with paroxysmal hemidystonia admitted to a tertiary care hospital, India, and review the literature on spinal dystonia in neuromyelitis optica (NMO). A 19-year-old woman presented with recurrent visual loss and quadriparesis. She developed paroxysmal hemidystonia after 18 days of a second episode of quadriplegia, during which her muscle power improved to Grade 3. Magnetic resonance imaging (MRI) of her spine showed central T2 hyperintensity extending from C2 to C7 vertebral level, and a cranial MRI was normal. Tibial somatosensory evoked potentials were unrecordable. Aquaporin-4 antibody was positive in serum, confirming the diagnosis of NMO. Paroxysmal hemidystonia responded to carbamazepine 200 mg thrice daily. Paroxysmal dystonia may occur in a patient with myelitis and may respond to carbamazepine.