Bilateral Ulnar Nerves Research Articles

S45. To report abnormal nerve conduction studies in two patients with hypokalemic paralysis

Introduction In hypokalemic paralysis, hypokalemia-induced inactivity of the muscle fibre Na+K+ ATPase pump is supposed to be responsible for motor inexcitability, resulting reduced or non-recordable CMAP amplitudes. Hence, nerve conduction studies (NCS) mainly includes reversible reduction in compound muscle action potential (CMAP) amplitude. Other NCS abnormalities are very uncommon and suggest more widespread channel abnormality. We report two patients with hypokalemic paralysis with reversible NCS abnormalities. Methods Sensory-motor NCS of all four limbs (bilateral median, ulnar, tibial, peroneal and sural nerves) and other relevant blood investigations were performed in two young adult male patients during first episode of reversible quadriparesis. NCS was repeated 24 h after improvement of motor power. Secondary causes of hypokalemia were excluded by relevant investigations. Results Serum potassium (K+) was low ( Conclusion CMAP and F wave may be abnormal during hypokalemic paralysis due to abnormal Na+K+ ATPase at muscle fibres. However,other NCS abnormalities like abnormal SNAPs, prolonged DL, or non recordable proximal CMAP (specially when distal CMAP is recordable) are rarely reported in hypokalemic paralysis. These abnormalities are marker of nerve fibre abnormality and supposed to be normal in muscle disease. These abnormal and reversible NCS in present case series suggests that Na+K+ ATPase abnormalities are not restricted to muscle fibres but probably more widespread and involve nerve fibres also. However, larger study is required to evaluate this aspect of hypokalemic paralysis.

F56. Description of neurophysiological findings in mononeuritis multiple as a neurologic manifestation of borrelia burgdorferi infection - Case report

Introduction The infection for Borrelia burgdorferi is a zoonosis, in which the spirochaetes can be transmitted to human beings only by the bite of infected ixodid ticks, the spirochaete is maintained at high levels in populations of field mice or birds, the likelihood of having it is dependent on geography, recreational habits of the patient, and season. It is a multisistemic disease it can affect central nervous system. Neurological manifestations often referred to as Lyme neuroborreliosis are reported in up to 12% of patients with Lyme disease. In Europe and the United States seroprevalencia is 1–27%. This has not been determined in Mexico yet. In Mexico are few reports and series of cases published about Lyme neuroborreliosis, being a subdiagnosed disease in patients with neurological clinical manifestations. Methods Male 23 yo, carpenter. He started eight months earlier with symmetric paresthesias of both hands with ascending distribution from distal to proximal, presented inability to hold work tools with both hands, paresthesias in the plantar region and paresis of the lower limbs, ascending from distal to proximal, preventing him from walking and requiring help, finally prostration. PE: Generalized atrophy of distal predominance, interosseous atrophy of thenar and hypothenar, proximal upper limb 4/5, distal 3/5, proximal and distal lower limbs 4/5, absent bilateral patellar reflex. Bilateral plantar flexor response. Hypopallesthesia, altered arthrokinesia in both lower limbs and right hand. Unsteady gait with a broad base, short and slow steps with help. Romberg positive. Results Neurophysiological studies. Somatosensory evoked potentials (SSEP) of upper limbs with peripheral response integration at the level of the brachial plexus (N9) asymmetric by left prolongation and absence of responses from posterior cords (N13). SSEP of lower limbs with complete and bilateral absence of responses from popliteal fossa. N. median left, N. ulnar right, delay in nerve conduction velocity. Bilateral tibial with prolonged latency. N. median left with prolongation of peak latency. N. ulnar voltage asymmetry by right depression. F response of bilateral ulnar nerve did not elicit response. Bilateral tibial nerve with prolonged latencies and frequency of reduced evocation. The neurophysiological study showed evidence of multiple mononeuropathy that involves all four limbs asymmetrically. Ig G by Western blot versus Borrelia burgdorferi was positive in blood. It was prescribed ceftriaxone 1 g IV BID for 14 days, after this treatment showed neurological improvement, he continued with doxycycline 100 mg orally BID for a month. Conclusion Neuropathies in patients with chronic Lyme disease commonly present as non-vasculitic multiple mononeuritis, the motor deficit follows the multiple pattern. This case is based on an unusual neurological manifestation together with the exclusive predilection for affection of the central nervous system, having as main geographical and occupational risk factors.

Evidence that impaired motor conduction in the bilateral ulnar and tibial nerves underlies cervical spondylotic amyotrophy in patients with unilateral deltoid muscle atrophy.

Introduction The clinical entity of cervical spondylotic amyotrophy (CSA) is characterized by severe muscle atrophy in the upper extremities with insignificant sensory deficits in patients with cervical spondylosis. However, the pathogenesis of CSA is still unclear. Methods We assessed electrophysiological motor conduction through the corticospinal tract and ulnar and tibial nerves, which do not supply the deltoid or biceps muscles, of 18 patients with CSA, 12 patients with compressive cervical myelopathy, and 18 control subjects with cervical spondylotic radiculopathy. Motor evoked potentials following transcranial magnetic stimulation and M-waves and F-waves following electrical stimulation were measured from the bilateral abductor digiti minimi muscles (ADMs) and abductor hallucis muscles (AHs). The peripheral conduction time (PCT) was calculated from the latencies of the CMAPs and F-waves as follows: (latency of CMAPs + latency of F-waves - 1) / 2. The central motor conduction time (CMCT) was calculated by subtracting the PCT from the onset latency of the MEPs. Results The M-wave (M) latency and minimum F-wave (Fmin) latency from the ADM, and Fmin-M latency from the ADM/AH were significantly longer in the CSA group than in the other groups, on both the affected (p = 0.000-0.007) and unaffected sides (p = 0.000-0.033). F-wave persistence from the bilateral ADMs was significantly lower in the CSA group than in the other groups (p = 0.000-0.002). Among the CSA patients, there were no significant differences in these parameters between the affected and unaffected sides. The CMCT showed no significant differences between the CSA and control groups, but significant differences between the CSA and CCM groups (p = 0.000-0.004). Conclusions CSA patients with unilateral deltoid muscle atrophy had subclinical impairments of lower motor neurons and/or peripheral axons in the ulnar nerve, and subclinical impairments of peripheral axons in the tibial nerve. These motor impairments may have originally existed in these individuals before the onset of CSA.

Nerve Conduction and Heart Rate variability in Patients with Hypothyroidism at a Tertiary Care Centre in Eastern Nepal

Thyroid hormone effects on many organs including central and peripheral nervous systems. However, these hormones do not affect all systems/organs to a similar extent. Thus, we conducted this study to explore the effect of thyroid hormones on somatic nervous system assessed by Nerve conduction study and cardiac autonomic activity assessed by heart rate variability. The study included newly diagnosed hypothyroid patients and healthy controls. In all subjects NCS were performed in median, ulnar, tibial and sural nerves using Nihonkohden machine Cardiac autonomic control was assessed using Short-term Heart Rate Variability and parameters were analyzed by Time Domain and Frequency Domain methods. Both the groups were comparable in term of age, Body Mass Index, Pulse Rate, Systolic Blood Pressure and Diastolic Blood Pressure. Sensory parameters of NCS showed significant decrease in left median nerve SNAP amplitude (38.24±10.23 Vs 31.59±14.06, P=0.048) and nerve conduction velocity of bilateral median nerve in hypothyroid patients. In motor parameters of NCS, onset latencies of bilateral median nerves and right ulnar nerve were significantly increased in hypothyroid patients. All Time Domain measures of HRV and Frequency Domain measures; LF Power, HF Power and Total Power were significantly decreased (P<0.05) in hypothyroid patients. These HRV parameters are indicators of parasympathetic activity. In newly diagnosed hypothyroid patients, especially median nerve functions (both sensory and motor) and parasympathetic activity were decreased. It reflects that in hypothyroidism both autonomic nervous system and other somatic nerves are not affected in a similar extent.

Peripheral neuropathy is not the end but the beginning

Background: Diagnosing the etiology of peripheral neuropathy is a challenging clinical situation especially in a patient with multiple potential causes. This case discusses how distribution of the neuropathy and accompanying manifestations can help unmask the original culprit. Case report: A 59-year-old male patient with a 15 year history of diabetes presented with severe peripheral neuropathy affecting the upper more than lower limbs. Six months prior to this presentation he had been diagnosed with hepatitis C infection for which he received interferon, ribavirin and sofosbuvir therapy. Physical examination revealed bilateral, thickened and tender ulnar and popliteal nerves, multiple patches of vitiligo and another well defined, hypopigmented, anesthetic lesions appearing over his back and buttocks. Six slit smears were positive for Mycobacterium leprae and ultrasound of both median nerves indicated swollen and hypoechoic areas along their course with positive intraneural Doppler uptake. Conclusion: Treatment with the interferon or immunosuppressive therapy could unmask leprosy. Leprosy should be included in the differential diagnosis of severe peripheral neuropathy.

Hereditary neuropathy with liability to pressure palsy in patients under 30 years old: Neurophysiological data and proposed electrodiagnostic criteria.

In young patients with mononeuropathy who lack family history and precipitating factors, hereditary neuropathy with liability to pressure palsy (HNPP) may be a possibility. Our objective is to propose neurophysiological criteria for HNPP in patients <30 years of age. We conducted a national multicenter retrospective clinical and neurophysiological study in patients under 30 with genetically confirmed HNPP. All of the 51 patients included in the study had at least 1 demyelinating pattern in 2 asymptomatic nerves, and 3 abnormalities were found in almost 90%, including slowed motor nerve conduction velocity across the elbow in at least 1 ulnar nerve (97.5%), increased distal motor latency (DML) in at least 1 fibular nerve (95.8%), and increased DML in both median nerves (89%). Age influenced DML slightly only in the fibular nerve. Dissemination of nerve involvement in HNPP incites to perform a complete nerve conduction study. including bilateral ulnar, fibular, and median nerves. Muscle Nerve 57: 217-221, 2018.

50. Peripheral neuropathy associated with Duodopa infusion: neurophysiological retrospective study

Subacute and chronic peripheral neuropathies (PN) have been reported in Parkinson’s disease (PD) patients treated with levodopa/carbidopa intestinal gel infusion (Duodopa), but only one study evaluated a long term follow up. We performed a retrospective electrophysiological study of 23 PD patients treated with Duodopa with a follow up period of 18 months. The electrophysiological assessments of 23 patients have been retrospectically collected and evaluated at baseline (before Duodopa infusion) and at 6, 12 and 18 months. Nerve conduction studies were obtained for the bilateral median, ulnar, tibial and peroneal motor nerves and for the bilateral median, ulnar and sural sensory nerves. During a follow-up period of 18 months, a statistically significant decrease in mean sural SAP amplitude was recorded. At baseline 5/23 patients showed PN. During the follow up, one patient developed a subacute PN and two patients developed a sensory-motor PN. Duodopa infusion was immediately halted in the subacute case, while the infusion therapy was not interrupted in chronic forms. All patients were supplemented with vitamin B12 and folate. Serial electrophysiological evaluations are mandatory in patients treated with Duodopa, given the possible risk of PN.

Autonomic neuropathy impairing quality of life after completion of MDT: Are we managing enough?

A 26 year old male, treated case of lepromatous leprosy, presented with severe heat intolerance, loss of sensation and sweating over distal parts of both upper and lower limb of 12 years’ duration. On examination, there was definite sensory loss over the extremities in glove and stocking pattern and thickening of the bilateral ulnar, common peroneal and radial nerves. There were three trophic ulcers over the plantar aspect of the right foot, atrophy of the small muscles of hand, reabsorption of the distal index, middle and ring fingers along with total clawing of both hands. A slit skin smear for acid fast bacilli revealed fragmented granular bacilli. A starch-iodine test was used to document the pattern of sweating which coincided with the glove and stocking pattern of sensory loss found in lepromatous leprosy. Even though the patient had completed WHO MDT and bacilli were dead, the damage to the autonomic system was extensive in the patient leading to widespread loss of sweating and severe heat intolerance affecting his quality of life. Also the patient had motor deformity in the form of total clawing which added to his disability. Therefore all cases should be followed up even after completion of MDT, counselled regarding the course of the disease, and trained to tackle the consequences of nerve damage in their daily life. We report the case as autonomic dysfunction that has been rarely documented in leprosy patients and also we emphasise the use of the starch-iodine test in such cases along with management of the disease in part by using hydro-oleo therapy.

To Compare Sensory Threshold, Pain Threshold and Vibration Perception in Road Diggers and Controlled Group between Age Group 35–45 Years

To assess the sensory threshold and pain threshold in road diggers and controlled group, total 30 individuals in which 15 Controlled Group and Road Diggers, Nerve muscle Stimulator was used for bilateral ulnar and median nerves. The vibration perception was assessed using tunning fork of 128Hz and 256Hz, which were hit and placed on the patient's mastoid process again the tunning fork were hit and placed on the ulnar styloid process, then the patient were asked the difference between the perceptions as comparing to mastoid process.

Quantitative Muscle Ultrasonography in Carpal Tunnel Syndrome.

ObjectiveTo assess the reliability of quantitative muscle ultrasonography (US) in healthy subjects and to evaluate the correlation between quantitative muscle US findings and electrodiagnostic study results in patients with carpal tunnel syndrome (CTS). The clinical significance of quantitative muscle US in CTS was also assessed.MethodsTwenty patients with CTS and 20 age-matched healthy volunteers were recruited. All control and CTS subjects underwent a bilateral median and ulnar nerve conduction study (NCS) and quantitative muscle US. Transverse US images of the abductor pollicis brevis (APB) and abductor digiti minimi (ADM) were obtained to measure muscle cross-sectional area (CSA), thickness, and echo intensity (EI). EI was determined using computer-assisted, grayscale analysis. Inter-rater and intra-rater reliability for quantitative muscle US in control subjects, and differences in muscle thickness, CSA, and EI between the CTS patient and control groups were analyzed. Relationships between quantitative US parameters and electrodiagnostic study results were evaluated.ResultsQuantitative muscle US had high inter-rater and intra-rater reliability in the control group. Muscle thickness and CSA were significantly decreased, and EI was significantly increased in the APB of the CTS group (all p<0.05). EI demonstrated a significant positive correlation with latency of the median motor and sensory NCS in CTS patients (p<0.05).ConclusionThese findings suggest that quantitative muscle US parameters may be useful for detecting muscle changes in CTS. Further study involving patients with other neuromuscular diseases is needed to evaluate peripheral muscle change using quantitative muscle US.

A Unique Case of Bilateral High Ulnar Nerve Laceration

High ulnar nerve injuries are now commonly treated with a distal nerve transfer, in which the anterior interosseuos nerve to pronator quadratus is coapted to the deep ulnar nerve motor branch. Herein, the authors describe a case of bilateral ulnar nerve laceration that provides an opportunity to directly compare two operative techniques on different extremities of the same patient. The patient sustained bilateral ulnar nerve injuries 6 cm superior to the medial epicondyle. The right ulnar nerve was proximally repaired with primary neurorraphy and a distal motor nerve transfer. The left ulnar nerve underwent primary nuerorraphy without a nerve transfer because the median nerve was mistakenly tied off to control bleeding. Physical examination and nerve conduction studies two years postsurgical intervention demonstrated an undeniable difference in cosmesis and function. The clinical findings suggest a superior result with a combined primary neurorraphy and a distal nerve transfer compared with a primary neurorraphy alone.

Carpal tunnel syndrome in pediatric mucopolysaccharidoses

BackgroundCarpal tunnel syndrome (CTS) is rare in children but is a recognised complication of the mucopolysaccharidoses (MPS). Clinicians should have a low threshold of suspicion for CTS in this group as symptoms may be atypical or minimal. If untreated, CTS can cause significant loss of hand function. We present findings in 11 children with mucopolysaccharidoses and suspected CTS, and propose guidelines for screening for CTS in children with these disorders.MethodsClinical and electrodiagnostic data of 11 children with mucopolysaccharidoses, who were suspected on clinical grounds to have CTS, was reviewed. All subjects underwent motor and sensory conduction studies of bilateral median and ulnar nerves. The presence of carpal tunnel syndrome and its severity was determined. Subsequent details of intervention(s) and recurrence were noted.ResultsThree children had MPS I, five had MPS II, one had MPS III and two had MPS IV. Seven had motor and three sensory features referable to median nerve compression. Nine of the eleven children (2/3 with MPS I, 5/5 with MPS II, 0/1 with MPS III, 2/2 with MPS IV) had median neuropathies at the wrist, (eight bilateral, one unilateral) which were mild in three, moderate in five, and severe in one. Three children presented with symptoms at five years age. Six underwent median nerve decompression. Four of these had recurrent symptoms several years after surgery, which was confirmed on nerve conduction studies in two cases. To the best of our knowledge, this is the first report of carpal tunnel syndrome in MPS IV. ConclusionSome children with mucopolysaccharidoses experience early development of at least moderately severe carpal tunnel syndrome. We recommend screening for median neuropathies at the wrist from age 5 years for children with mucopolysaccharidoses, particularly types I, II and IV, regardless of their symptoms of CTS, and of the treatment received for mucopolysaccharidosis.

Leprosy Mimicking Psoriasis.

Leprosy Mimicking Psoriasis.

The prevalence of median neuropathy at wrist in systemic sclerosis patients at Srinagarind Hospital

Objectives: To determine the prevalence and factor related with median neuropathy at wrist (MNW) in systemic sclerosis patients. Study Design: Cross-sectional study. Setting: Srinagarind Hospital, Khon Kaen, Thailand. Participants: Systemic sclerosis patients who attended the Scleroderma Clinic, Srinagarind Hospital. Materials and Methods: Seventyfive systemic sclerosis patients were prospectively evaluated by questionnaire, physical examination, and electrodiagnostic study. The questionnaire consisted of the symptoms, duration, and type of systemic sclerosis. The physical examination revealed skin score of systemic sclerosis, pinprick sensation of median nerve distribution of both hands, and weakness of both abductor pollicis brevis muscles. The provocative test which were Tinel's sign and Phalen's maneuver were also examined. Moreover, electrodiagnostic study of the bilateral median and ulnar nerves was conducted. Results: The prevalence of MNW in systemic sclerosis patients was 44% - percentage of mild, moderate, and severe were 28%, 9.3%, and 6.7%, respectively. The prevalence of asymptomatic MNW was 88%. There were no association between the presence of MNW and related factors of systemic sclerosis. Conclusions: MNW is one of the most common entrapment neuropathies in systemic sclerosis patients. Systemic sclerosis patients should be screened for early signs of MNW.

Is Further Examination Necessary in Patients with Behcets Disease Without Any Neurological Signs or Symptoms?

Background and Purpose: A mean percentage of 5% was reported for neurological involvement in researches related to Behcet's disease. The neurological involvement may be characterized by headache, neuropsychiatric symptoms, brain stem involvement, cerebral venous thrombosis or peripheral nervous system involvement. Sometimes neurological involvement may be seen without any neurological sign or symptom. In our study, we aimed to evaluate the cognitive, psychiatric and neurophysiological responses in Behcet's disease without neurological sign and symptom. Methods: The study was performed on patients with Behcet's disease without neurological signs and symptoms and healthy volunteers that would make the control group. The patients were assessed with Mini Mental State Examination, Hamilton Rating Scale for Depression, nerve conduction study and visually evoked potential. Results: A total of 27 male patients (age: 24.88±5.45 years) were enrolled in the study. Neurological examinations of all the patients were normal. Mean Mini Mental State Examination score of the patients was 29.62±0.79 (2 patients had loss of focus, 2 patients had disturbance in the copying function) and 2 patients had mild depression in Hamilton Rating Scale for Depression. In the nerve conduction study, 2 patients had right sensory-motor carpal tunnel syndrome, 1 patient had right sensory-motor carpal tunnel syndrome - bilateral ulnar nerve entrapment. In the visually evoked potential examination, 8 patients had prolonged P100 latency, decreased amplitude and disturbance of wave morphology. Conclusion: Visually evoked potential examination may be used as a conductive method to detect the subclinical neurological pathologies in Behcet's disease. The possible silent neurological involvement should be evaluated with further neuro-screening methods.

Comparison of Automated Versus Traditional Nerve Conduction Study Methods for Median Nerve Testing in a General Worker Population

ObjectiveTo investigate the validity of automated nerve conduction studies compared to traditional electrodiagnostic studies (EDS) for testing median nerve abnormalities in a working population. DesignAgreement study and sensitivity investigation from 2 devices. SettingField research testing laboratory. ParticipantsActive workers from several industries participating in a longitudinal study of carpal tunnel syndrome. MethodsSixty-two subjects received bilateral median and ulnar nerve conduction testing across the wrist with a traditional device and the NC-stat automated device. We compared the intermethod agreement of analogous measurements. Main outcome measurementNerve conduction study parameters. ResultsMedian motor and sensory latency comparisons showed excellent agreement (intraclass correlation coefficients 0.85 and 0.80, respectively). Areas under the receiver operating characteristic curves were 0.97 and 0.96, respectively, using the optimal thresholds of 4.4-millisecond median motor latency (sensitivity 100%, specificity 86%) and 3.9-millisecond median sensory latency (sensitivity 100%, specificity 87%). Ulnar nerve testing results were less favorable. ConclusionThe automated NC-stat device showed excellent agreement with traditional EDS for detecting median nerve conduction abnormalities in a general population of workers, suggesting that this automated nerve conduction device can be used to ascertain research case definitions of carpal tunnel syndrome in population health studies. Further study is needed to determine optimal thresholds for defining median conduction abnormalities in populations that are not seeking clinical care.

11. Carpal tunnel syndrome in pediatric mucopolysaccharidoses

Background Carpal tunnel syndrome is rare in children but is a recognised complication of the mucopolysaccharidoses. Clinicians should have a low threshold of suspicion for carpal tunnel syndrome in this group as symptoms may be atypical or minimal, especially in those with intellectual disabilities secondary to mucopolysaccharidoses. If untreated, carpal tunnel syndrome can cause significant, potentially permanent loss of hand function. We present findings in 11 children with mucopolysaccharidoses and suspected median neuropathies at the wrist, and propose guidelines for screening for carpal tunnel syndrome in children with these disorders. Methods Clinical and electrodiagnostic data of 11 children with confirmed mucopolysaccharidoses by enzymatic ± molecular testing, who were suspected on clinical grounds to have carpal tunnel syndrome, was reviewed. All subjects underwent motor and sensory conduction studies of bilateral median and ulnar nerves. The presence of carpal tunnel syndrome and its severity was determined. Subsequent details of intervention (s) and recurrence were noted. Results Three children had Hurler syndrome (MPS I), five had Hunter syndrome (MPS II), one had Sanfilippo syndrome (MPS III) and two had Morquio syndrome (MPS IV). Seven had motor and three sensory features referable to median nerve compression. Nine of the eleven children (2/3 with MPS I, 5/5 with MPS II, 0/1 with MPS III, 2/2 with MPS IV) had median neuropathies at the wrist (eight bilateral, one unilateral), which were mild in three, moderate in five, and severe in one. Three children presented with symptoms at 5 years age. Six underwent median nerve decompression. Four of these had recurrent symptoms several years after surgery. Recurrent carpal tunnel syndrome was confirmed on nerve conduction studies in two cases. To the best of our knowledge, this is the first report of carpal tunnel syndrome in MPS IV. Conclusion Some children with mucopolysaccharidoses experience early development of at least moderately severe carpal tunnel syndrome. We recommend screening for median neuropathies at the wrist from age 5 years for children with mucopolysaccharidoses, particularly types I, II and IV, regardless of whether they are symptomatic or asymptomatic of carpal tunnel syndrome and of the treatment received for their mucopolysaccharidosis.

Histoid leprosy in type II reaction with neural abscess: Treated with ulnar nerve decompression and anterior transposition

A 40-year-old male, known case of histoid leprosy on multidrug therapy since 10 months, presented with erythema nodosum leprosum since 15 days. Examination findings revealed multiple, irregular, bilateral, symmetrically distributed shiny nodules and plaques all over the body. Bilateral radial, ulnar, radial cutaneous, and lateral popliteal nerves were thickened. The right ulnar nerve was exclusively tender indicative of an abscess, for which decompression and anterior transposition of ulnar nerve was performed. Steroids were given parenterally followed by oral steroids in a dose of 60 mg/day and tapered gradually with good prognosis. In addition, thalidomide in a dose of 300 mg/day was also started.

Bimelic Symmetric Hirayama’s Disease: Outgrowing its ‘Brachial Monomelic Amyotrophy’ Origins

A 18 year old boy presented to us with history of insidious onset, gradually progressive weakness of bilateral upper limbs which started 2 years back but had been static for the past one year. He had difficulty in buttoning his shirt and holding a pen. There was no difficulty in raising his arms while wearing his clothes. He also noticed gradual thinning of both his hands and distal part of forearm near his wrist. There was no history suggestive of lower limb, trunk or cranial nerve involvement. There was no sensory loss and no bowel bladder involvement. There was history of pain over neck on prolonged flexion. There was no history of similar disease in family. On examination bilateral wasting of small muscles of hand and muscles over the medial aspect of forearm. Tone was reduced in bilateral wrist and elbow joints. Bilateral biceps, triceps and supinator jerks were absent. All other central nervous system examination was within normal limits. Muscle enzymes CPK and LDH were normal. NCV of upper limbs showed no response in bilateral ulnar and median nerves. EMG showed denervation potentials and fasciculations. MRI cervical spine showed loss of dural attachment posteriorly with increase in posterior epidural space and engorged venous plexuses at lower cervical and upper dorsal spine. There was antero-posterior flattening of spinal cord from C5 to D3 segment. Based on the clinical presentation and MRI findings a diagnosis of bimelic symmetric Hirayama’s disease was made. Patient was prescribed a cervical collar. At 6 months follow-up patient showed no progression of weakness or atrophy. Hirayama’s disease is a benign non-progressive illness affecting young males causing lower motor neuron type of upper limb weakness without sensory involvement due to forward displacement of the posterior wall of the lower cervical dural canal during neck flexion [1]. The disease was thought to be unilateral or asymmetric in onset if bilateral leading to it being called brachial monomelic amyotrophy. But it is recognised now that up to 10% of patients may have bilateral symmetric involvement [2]. Syringomyelia, spinal cord tumors, multifocal motor neuropathy, and toxic neuropathies are close differentials in cases with bilateral symmetric onset. Early diagnosis and use of a cervical collar prevents progression of disability in Hirayama’s disease [3].

Necrotic Bullous Erythema Nodosum Leprosum—a Case Report from a Tertiary Care Centre In Eastern India

A patient with lepromatous leprosy while on multidrug therapy (MDT) presented with crops of brightly erythematous tender nodules on both upper and lower extremities along with bullous necrotic lesion over face and trunk of one week duration. The lesions were associated with high grade fever. Bilateral ulnar, radial cutaneous and lateral popliteal nerves were thickened. Slit skin smear showed a bacterial index (BI) of 4+ with morphological index 40% and histopathology was consistent with type II reaction. Systemic naproxen, corticosteroid and clofazimine were administered and the patient became symptoms free after a week. On 21 st