Absent Deep Tendon Reflexes Research Articles

Endoscopic Third Ventriculostomy with Choroid Plexus Coagulation for Treatment of Hydrocephalus in X-linked Myotubular Myopathy: A Novel Approach

X-linked myotubular myopathy (XLMTM) is a rare x-linked disease. It manifests in male new-borns with multiple comorbidities including severe hypotonia, absent deep-tendon reflexes, and respiratory weakness leading to ventilator dependence. The affected individuals usually die in early infancy but some individuals have survived into the second decade of life. Macrocephaly and hydrocephalus are common in XLMTM with some reporting it in up to 70% and 50% of cases, respectively. Hydrocephalus is usually communicating in nature and may require surgical intervention if it is progressive. Prior cases have also used ventriculoperitoneal shunts as the primary hydrocephalus treatment modality. Here we have described a case of treating XLMTM associated communicating hydrocephalus via endoscopic third ventriculostomy with choroid plexus coagulation. A 23-month-old male with XLMTM presented with macrocephaly and hydrocephalus. Previous cases of hydrocephalus in XLMTM were treated with the placement of a ventriculoperitoneal shunt. However, in this case, it was contraindicated due to extensive multisystem comorbities which restricted treatment options. Due to the lack of another feasible compartment for the placement of the cerebrospinal fluid (CSF) diversion, it was decided that an endoscopic third ventriculostomy with choroid plexus coagulation will be performed. During the patient’s postoperative course, his head circumference stabilized at 50 cm and serial brain imaging indicated the decreasing size of the ventricular system. He also became more awake and interactive. His family withdrew care when he was 33-months-old, due to complications not related to the neurosurgery procedure. This case was the first time endoscopic third ventriculostomy with choroid plexus coagulation was successfully performed for the treatment of hydrocephalus in X-linked myotubular myopathy.

Autosomal Recessive Limb Girdle Muscular Dystrophy In A Complex Consanguineous Family: The First Cases Series In Indonesia

ABSTRACTBackground: Limb girdle muscular dystrophy (LGMD) is a neuromuscular abnormality with clinical heterogeneity and various severity, where over 30 subtypes have been identified. Meanwhile, molecular diagnosis of LGMD is not commonly carried out in Indonesia. We present a large pedigree of familial LGMD, with over 14 years of follow-up.Case Presentation: A 12-year old female patient came with muscle weakness. She had toe walking since age of 6, followed by calf hypertrophy for over three years. Family history revealed complex consanguinity. Her younger sister and her parents’ cousin had similar condition, with the latter was already bedridden.Physical examination results were waddling gait, lordotic spine, and absent deep tendon reflexes. Muscle biopsy showed sign of dystrophic process. Immunoperoxidase staining of some proteins resulted normal. Single nucleotide polymorphism (SNP) array in two siblings revealed homozygosity on chromosome 15 containing CAPN3 gene of LGMD2A subtype.Recently, the patient is wheelchair bound and undergoes rehabilitation. Her sister is still able to walk with abnormal gait, while her parents’ cousin had passed away in age 55. From the multiple consanguinity, it could be concluded as autosomal recessive type LGMD.Conclusion: A large family with LGMD from Indonesia was presented with more than 14 years of care. Clinical diagnosis was made based on physical and additional examination, however molecular analysis for establishing definitive diagnosis is still limited. Further studies such as targeted or whole exome sequencing is warranted to elucidate the cause of disease. Long-term evaluation and supportive care, in addition to proper counseling may increase quality of life.

Atypical Presentation of Lambert Eaton Myasthenic Syndrome

SESSION TITLE: Critical Care 3 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare disorder in which antibodies against presynaptic voltage-gated calcium channels (VGCC) prevent the flux of calcium into the nerve terminal. This reduces the release of acetylcholine affecting neuromuscular transmission. Here we discuss an atypical presentation of LEMS. CASE PRESENTATION: A 64 year old male was admitted for dysphagia and difficulty ambulating. Chest x-ray showed a mediastinal mass. CT confirmed a 5 x 5 cm anterior mediastinal mass. He had 4/5 strength and 1+ deep tendon reflexes (DTRs) in lower extremities. Sodium was 119 mEq/L. On day 2, the patient had bradycardic arrest. Return of spontaneous circulation was achieved after 3 minutes of chest compressions and he was intubated. During his hospital course, he had tachybradyarrhythmias. Despite immediate recovery to his normal mental status, normal chest x-ray, and arterial blood gas, he continued to fail his spontaneous breathing trials. He had a negative inspiratory force of -6 and vital capacity of 150 mL. Paraneoplastic syndrome was suspected due to neuromuscular respiratory failure, hyponatremia, and mediastinal mass. Intravenous immunoglobulin (IVIG) was given for 5 days. On day 10 of intubation, supraclavicular lymph node biopsy showed small cell lung carcinoma and carboplatin and etoposide was initiated. The patient was extubated on the same day. Serology was positive for P/Q type VGCC antibodies. DISCUSSION: LEMS has a prevalence of 2.3 per million and an incidence of 0.5 per million. There have been 400 reported LEMS cases in the US, of which 17 cases had frank respiratory failure. Sixty percent of LEMS cases are associated with small cell lung cancer. Patients with LEMS have proximal muscle weakness that transiently improves after repetitive exertion, absent DTRs, bulbar dysfunction, and autonomic dysfunction. This patient had dysphagia, a sign of bulbar dysfunction. He ambulated while intubated, which may reflect improvement of weakness after repetitive exertion. If not, this level of weakness is uncommon in LEMS as diaphragmatic weakness often parallels the degree of proximal muscle weakness. In contrast to other cases in which DTRs are absent, this patient had hyporeflexia. Finally, he had dysautonomia manifesting as fluctuating episodes of bradycardia and tachycardia. EMG is used to confirm LEMS, however was not available. CONCLUSIONS: Neuromuscular respiratory failure should lead to evaluation of more common causes of weakness. In patients with malignancy, the possibility of LEMS should be entertained. The early use of IVIG in this patient allowed for earlier extubation. Reference #1: Laghi F, et al. Disorders of the respiratory muscles. Am J Respir Crit Care Med. 2003. 168(1): 10-48. Reference #2: Bekircan-Kurt C, et al. Voltage gated calcium channel antibody-related neurological diseases. World J of Clin Cases: 3.3 (2015): 293. Reference #3: Sanders DB. Lambert-eaton myasthenic syndrome: diagnosis and treatment. Ann NY Acad Sci. 2003. 998: 500-508. DISCLOSURE: The following authors have nothing to disclose: Gauri Behari, Raja Sinha, Vadym Rusnak, Andrea Espinoza, Michael Charlet, Ibrahim El-Abbassi No Product/Research Disclosure Information

Zika virus disease-associated Guillain-Barré syndrome—Barranquilla, Colombia 2015–2016

BackgroundAn outbreak of Guillain-Barré syndrome (GBS), a disorder characterized by acute, symmetric limb weakness with decreased or absent deep-tendon reflexes, was reported in Barranquilla, Colombia, after the introduction of Zika virus in 2015. We reviewed clinical data for GBS cases in Barranquilla and performed a case-control investigation to assess the association of suspect and probable Zika virus disease with GBS. MethodsWe used the Brighton Collaboration Criteria to confirm reported GBS patients in Barranquilla during October 2015–April 2016. In April 2016, two neighborhood and age range-matched controls were selected for each confirmed GBS case-patient. We obtained demographics and antecedent symptoms in the 2-month period before GBS onset for case-patients and the same period for controls. Sera were collected for Zika virus antibody testing. Suspected Zika virus disease was defined as a history of rash and ≥2 other Zika-related symptoms (fever, arthralgia, myalgia, or conjunctivitis). Probable Zika virus disease was defined as suspected Zika virus disease with laboratory evidence of a recent Zika virus or flavivirus infection. Conditional logistic regression adjusted for sex and race/ethnicity was used to calculate odds ratios (ORs) and 95% confidence intervals (CIs). ResultsWe confirmed 47 GBS cases. Incidence increased with age (10-fold higher in those ≥60years versus those <20years). We interviewed 40 case-patients and 79 controls. There was no significant difference in laboratory evidence of recent Zika virus or flavivirus infection between case-patients and controls (OR: 2.2; 95% CI: 0.9–5.1). GBS was associated with having suspected (OR: 3.0, 95% CI: 1.1–8.6) or probable Zika virus disease (OR: 4.6, CI: 1.1–19.0). ConclusionsOlder individuals and those with suspected and probable Zika virus disease had higher odds of developing GBS. Key pointsWe confirmed a Guillain-Barré syndrome (GBS) outbreak in Barranquilla, Colombia, during October 2015–April 2016. A case-control investigation using neighborhood controls showed an association of suspected and probable Zika virus disease with GBS.

ORAL FACTITIAL INJURY ASSOCIATED WITH FAMILIAL DYSAUTONOMIA

Familial dysautonomia (FD), known as Riley-Day syndrome or hereditary sensory and autonomic neuropathy type III (HSAN3), is a rare autosomal recessive disorder occurring almost exclusively in individuals of Ashkenazi Jewish (AJ) descent (estimated carrier incidence 1:27). Mutations in the IKBKAP gene have recently been shown to be responsible for FD. Signs and symptoms of FD include: reduced sensitivity to pain and thermal extremes, absence of fungiform papillae, the inability to produce tears upon emotional crying, absence of axon flare after injection of intradermal histamine, and absent deep tendon reflexes. There is a high incidence of self-inflicted or accidental traumas to the oral cavity, including crown fractures, luxation or avulsion injuries, and ulceration of the oral and perioral soft tissues.

Friedreich’s ataxia associated with marfanoid features &amp; alopecia areata-rare disease manifestations or chance association?

Abstract Friedreich’s ataxia is the commonest cause of inherited ataxia and has an autosomal recessive mode of inheritance. The disease manifestations usually start appearing before the end of puberty and in most of the patients before 20 years of age. Classical disease presentation consists of slowly progressive sensory and cerebellar ataxia, absent deep tendon reflexes in lower limbs with subsequent development of dysarthria and other features. Apart from neurological features it has cardiac, skeletal and endocrine manifestations but its association with marfanoid features or alopecia areata has not been described in literature.

Manifestaciones neurológicas de la infección por el virus zika

El virus zika es un flavivirus transmitido a través de la mordedura de mosquito, transfusión de sangre, la relación sexual o de madre a hijo durante la gestación. Aunque las complicaciones neurológicas de la infección por el virus zika son raras, el síndrome de Guillain-Barré (GBS) es la manifestación más común y normalmente se desarrolla poco después de las manifestaciones sistémicas iniciales de la infección por el virus. Este síndrome comienza en las extremidades distales con alteraciones sensoriales simétricas y progresa hasta involucrar la debilidad y la disminución o ausencia de reflejos tendinosos profundos. Los casos graves pueden implicar deterioro respiratorio y cardiovascular, requiriendo atención en una unidad de cuidados intensivos, así como ventilador o soporte circulatorio. Una revisión de 166 casos publicados de GBS asociados con el virus zika se caracteriza por una menor mortalidad que los observados con GBS esporádica, pero los datos son limitados con respecto a resultados a largo plazo. Cuando esté disponible, el tratamiento con IgG intravenoso (IVIg) o plasmaféresis puede reducir la gravedad y duración de los síntomas.

Childhood hereditary ataxias: experience from a tertiary referral university hospital in Turkey.

Hereditary ataxias are a group of genetic disorders that are progressive and heterogeneous. The purpose of this study was to develop a practical and time-efficient approach to diagnosing childhood hereditary ataxias by analyzing characteristics and final diagnosis at a tertiary referral clinic for pediatric neurology. 196 patients admitted to the pediatric neurology department were included. The medical records were examined for demographic features, neurological, laboratory, electrophysiological, cranial imaging, and pathological findings, and for genetic studies. Patients were divided into two groups based on whether a final diagnosis was made. The undiagnosed and diagnosed groups consisted of 157 (81.1%) and 39 (19.9%) patients, respectively. The two groups differed in terms of levels of history of consanguineous marriage and mental and motor development before diagnosis, absence of deep tendon reflexes, and the presence of polyneuropathic changes detected by electromyelography (EMG), abnormal visual evoked potentials (VEPs), electroretinography (ERG), and muscle biopsy. To the best of our knowledge, this is the first study involving a large spectrum of diseases related to autosomal recessive ataxias in childhood in Turkey. One out of five patients with hereditary childhood ataxias can be diagnosed with clinical and laboratory and electrodiagnostic examination, especially with the help of imaging facilities, while genetic analysis is not possible for every child. Cranial magnetic resonance imaging followed by EMG provides the most important clues for the diagnosis of hereditary childhood ataxias.

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) as a first presentation of systemic lupus erythematosus

Peripheral nervous system involvement frequently occurs in systemic lupus erythematosus (SLE) patients. However, chronic inflammatory demyelinating polyneuropathy (CIDP) is an unusual presentation that can develop before, after, or simultaneously with the onset of SLE. This paper reports the case of a 20-year-old man with diabetes mellitus (DM) and CIDP accompanied by SLE. The patient complained of progressive weakness in the bilateral upper and lower extremities that had begun 2 months prior to this visit. He was diagnosed with CIDP and treated with intravenous immunoglobulin (IVIG), but had little improvement. A plasma exchange was then scheduled, but it was not helpful either. The patient developed polyarthritis, oral ulcer, and a worsening of his muscle weakness two weeks later. A neurologic examination revealed 3/5 muscle strength in the upper and lower extremities, absent deep tendon reflex (DTR), and impaired position sense. The patient was diagnosed with SLE because of pancytopenia, lymphopenia, pleuropericardial effusion, proteinuria, high titer anti-nuclear antibody (ANA), and anti-dsDNA. A kidney biopsy revealed stage IV lupus nephritis. The patient received 3 pulses of methyl prednisolone, 6 months of cyclophosphamide, and a high daily dose of prednisolone. His proteinuria improved, and he regained the ability to ambulate with a normal gait after about 2.5 months. To the best of the authors’ knowledge, the concurrency of CIDP with SLE and DM has not been previously reported.

Prediction and Outcome of Intensive Care Unit-Acquired Paresis.

Intensive care unit-acquired paresis (ICUAP) is associated with poor outcomes. Our objective was to evaluate predictors for ICUAP and the short-term outcomes associated with this condition. A secondary analysis of a prospective study including 4157 mechanically ventilated adults in 494 intensive care units from 39 countries. After sedative interruption, patients were screened for ICUAP daily, which was defined as the presence of symmetric and flaccid quadriparesis associated with decreased or absent deep tendon reflexes. A multinomial logistic regression was used to create a predictive model for ICUAP. Propensity score matching was used to estimate the relationship between ICUAP and short-term outcomes (ie, weaning failure and intensive care unit [ICU] mortality). Overall, 114 (3%) patients had ICUAP. Variables associated with ICUAP were duration of mechanical ventilation (relative risk ratio [RRR] per day, 1.10; 95% confidence interval [CI] 1.08-1.12), steroid therapy (RRR 1.8; 95% CI, 1.2-2.8), insulin therapy (RRR 1.8; 95% CI 1.2-2.7), sepsis (RRR 1.9; 95% CI: 1.2 to 2.9), acute renal failure (RRR 2.2; 95% CI 1.5-3.3), and hematological failure (RRR 1.9; 95% CI: 1.2-2.9). Coefficients were used to generate a weighted scoring system to predict ICUAP. ICUAP was significantly associated with both weaning failure (paired rate difference of 22.1%; 95% CI 9.8-31.6%) and ICU mortality (paired rate difference 10.5%; 95% CI 0.1-24.0%). Intensive care unit-acquired paresis is relatively uncommon but is significantly associated with weaning failure and ICU mortality. We constructed a weighted scoring system, with good discrimination, to predict ICUAP in mechanically ventilated patients at the time of awakening.

Severe thoracic fracture dislocation

A 48-year-old man had severe thoracic fracture and paraplegia after falling 7 m 3 weeks earlier. Physical examination showed hypoesthesia below the inguina, grade 0 weakness of the lower limbs, absent deep tendon reflexes, and a negative Babinski sign. Radiographs (Fig. 1A), computed tomography reconstruction images (Fig. 1B and C), and magnetic resonance imaging (Fig. 1D) revealed T10 and T11 vertebrae fracture, with T10 dislocating anterior of T11 totally.

A Case of Ataxia with Isolated Vitamin E Deficiency Initially Diagnosed as Friedreich's Ataxia.

Ataxia with isolated vitamin E deficiency (AVED) is a rare autosomal recessive condition that is caused by a mutation in the alpha tocopherol transfer protein gene. It is almost indistinguishable clinically from Friedreich's ataxia but with appropriate treatment its devastating neurological features can be prevented. Patients can present with a progressive cerebellar ataxia, pyramidal spasticity, and evidence of a neuropathy with absent deep tendon reflexes. It is important to screen for this condition on initial evaluation of a young patient presenting with progressive ataxia and it should be considered in patients with a long standing ataxia without any diagnosis in view of the potential therapeutics and genetic counselling. In this case report we present a patient who was initially diagnosed with Friedreich's ataxia but was later found to have AVED.

Gullian- Barre Syndrome in Pregnancy – A Case Report and Review of the Literature

The immune-mediated Gullian- Barre syndrome (GBS) is an acute demyelinating polyradiculopathy (AIDP) which typically presents as progressive, fairly symmetric muscle weakness accompanied by absent or depressed deep tendon reflexes. It has been linked to various infectious agents, such as Campilobacter jejuni and typically presents 2-4 weeks following a respiratory or gastrointestinal illness. With an estimated incidence in the general population of 0.75-2:100,000, its occurrence in pregnancy does not differ. Diagnostic criteria consist of clinical, laboratory and electrophysiological tests. Treatment of pregnant and non-pregnant patients with GBS usually does not differ and it is mainly composed of supportive care and monitoring of respiratory, cardiac and hemodynamic functions. Disease modifying treatments such as plasmapheresis and intravenous immunoglobulin (IVIG) are relatively safe in pregnancy. Timing and mode of delivery are based on obstetric indications and depend on maternal and fetal status. As such, if a pre-term delivery is indicated a course of antenatal corticosteroids should be considered. Therefore, GBS in pregnancy should be handled by a multidisciplinary team involving neurologists, obstetricians and anesthesiologists. We present a case report of an otherwise healthy woman diagnosed with GBS in pregnancy. Patient presentation, diagnosis, treatment and outcome as well of review of the literature will be discussed.

Neonatal spinal cord injury after an uncomplicated caesarean section

Abstract Introduction: Spinal cord injury of the newborn is a rare and critical condition which is typically reported after difficult deliveries. The few cases of spinal cord injury after caesarean section reported in literature are usually associated with intrauterine hyperextension of the head. However, in exceptional cases the authors described intrauterine vascular insults as a possible cause for spinal cord lesions during pregnancy. Presentation of the case: A term infant was born after an uncomplicated caesarean section following cephalic presentation. Pregnancy was reported as uneventful. Foetal ultrasounds were normal and showed no evidence of hyperextension of the head. Although the boy was in good general conditions after birth, he showed a flaccid paralysis of both upper extremities with absent deep tendon reflexes and missing pain response. Other neurologic findings like general tonus and reactivity as well as spontaneous movements of both lower extremities were adequate. A spinal MRI performed at 48 h of life showed intact brachial plexuses with evidence of spinal cord lesion compatible with an ischemic injury. Accurate anamnesis revealed that the mother had perceived reduced foetal movements in the last 2 days before birth, suggesting a possible intrauterine origin of the ischaemic event. Conclusion: Our case reinforces the hypothesis that small ischaemic spinal cord lesions may occur during uncomplicated pregnancy. Therefore, such lesions may not always be related to a difficult delivery. Irrespective of the cause, treatment and parental counselling remain challenging due to the rarity of reported cases.

Isolated facial diplegia in Guillain–Barré syndrome: Bifacial weakness with paresthesias

Bifacial weakness with paresthesias (BFP) is a subtype of Guillain-Barré syndrome defined by rapidly progressive bilateral facial weakness in the absence of other cranial neuropathies, ataxia, or limb weakness. Many patients also complain of distal limb paresthesias and display diminished or absent deep tendon reflexes. BFP is a localized form of Guillain-Barré syndrome and is thought to be caused exclusively by demyelinating- rather than axonal-type neuropathy. Patients with BFP do not display anti-ganglioside IgG antibodies. Since it is rare, many physicians are unfamiliar with BFP, as bilateral facial weakness is more commonly associated with sarcoidosis, Lyme disease, or meningeal pathology. Many patients diagnosed with bilateral Bell palsy may instead have BFP. In this review, we highlight the clinical features of BFP and outline diagnostic criteria.

Hereditary motor and sensory neuropathy lom type in 6-year-old boy: Case report

Six year old boy had slow psychomotor development from an early age. Sitting independently at the age of 11 months and walking at the age of 17 months. He was always clumsy, often stumbling and falling. Neurological examination on admission to the Institute at the age of 6.6 years: wasting of leg muscles, moderate weakness of proximal and distal muscles of legs, absent deep tendon reflexes; eqinovarus. Upper limbs, cranial nerves and sensory exam including hearing were found normal. Magnetic resonance of the brain was normal, and developmental quotient was 78. Electromyoneurographic examination (EMG) showed signs of sensitive and motor demyelinating neuropathy of a severe degree. Data on ethnicity of Bulgarian Gypsy directed us to the molecular – genetic testing, which was conducted at the Faculty of Biology in Belgrade. It was found that the boy has a homozygous mutation c.442>T in the NDRG1 gene. EMG examination is of great importance in the diagnosis of hereditary neuropathy, and in accordance with clinical signs directing us to the molecular genetic studies, which are important for establishing the diagnosis and providing appropriate genetic advice to the patients and their families.

Axonal involvement with white matter abnormalities in merosin-positive congenital muscular dystrophy: A new association

An 18-month-old infant who presented with delayed motor development, hypotonia and absent deep tendon reflexes and normal cognitive development was diagnosed to have merosin-positive congenital muscular dystrophy (MP-CMD) on the basis of raised serum levels of creatine kinase, features suggestive of myopathy on electrophysiological studies, dystrophic muscle pathology and normal immunohistochemistry for merosin (laminin-α2). Neuroimaging studies demonstrated white matter hyperintensities on T2-weighted images similar to that seen in patients with merosin negative (MN-CMD). Electrophysiological studies also demonstrated features of axonal involvement. This finding has not been previously described in association with MP-CMD. Although we have not been able to exclude abnormal glycosylation of α-dystroglycan, this case expands the clinical phenotype of MP-CMD and suggests that neuroimaging of children with CMD may be useful in the identification of variants of MP-CMD.

Idiopathic pes cavus in adults is not associated with neurophysiological impairment in the lower limbs.

The nerve conduction characteristics of adults with idiopathic pes cavus/hammer toes have not been studied extensively. Among 2048 out-patients (59.5 ± 13.9 years) referring to a laboratory of Neurophysiology in Rome, we recruited 18 patients with idiopathic pes cavus (61.3 ± 12.5 years). Fifty-four age/sex-matched controls were also studied. No nerve conduction differences were observed between patients with and without cavus foot (p > 0.05). The absence of deep tendon reflexes and slight muscle weakness and hypotrophy in the lower limbs were more common in subjects with cavus foot deformity than in controls (p < 0.001). Adult patients with idiopathic pes cavus/hammer toes do not differ from healthy controls from a neurophysiological standpoint, but they could show minor signs of clinical impairment, such as lower limb weakness, hypotrophy and areflexia.

Understanding Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is a rapidly progressive peripheral neuropathy that most commonly presents with ascending symmetrical weakness and diminished or absent deep tendon reflexes. Because weakness may affect the diaphragm and cause respiratory distress, 10% to 30% of patients require mechanical ventilation. Symptoms progress and peak about 4 weeks after onset. Patients generally require hospitalization for respiratory and cardiac monitoring, as well as supportive care and treatment. The treatments of choice are IV immunoglobulin and plasmapheresis. Even after treatment, as many as 20% of patients have persistent neurologic symptoms, and up to 3% of patients die of autonomic issues.

Idiopathic hypokalemic periodic paralysis: A series of cases clustered in a part of Eastern India

Background: Most of the studies on hypokalemic periodic paralysis (HOPP) in India and abroad had described secondary causes as the origin of the disease. We had observed a series of HOPP cases clustered within 40 kilometers around Bankura, a district town of West Bengal, Eastern India. The aim of our study was to probe into the clinical, biochemical and electrophysiological details of the cases, which were found to be idiopathic. Material and Methods: 32 idiopathic HOPP cases, attending the neurology OPD or admitted in Bankura Sammilani Medical College &amp; Hospital (BSMCH), were selected from October 2006 to May 2008. All the secondary causes of HOPP were excluded. The patients were evaluated and the data was analyzed. Results: Cases were clustered within a 40-kilometer radius from Bankura town, location of BSMCH. Male: female = 4.3:1. Median age of onset was 23.5 years (4-50 years). Median number of attacks was 2.5, (1 to 70); duration 3 hours - 6 days, median 3 days. Strenous physical activity was most common precipitating factor. 25% had bulbar symptoms, 9.4% had bladder dysfunction and 46.9% had diminished to absent deep tendon reflexes. Mean serum potassium = 2.45 mEq/L (1.4 – 3.1 mEq/L). Conclusion: We had observed a group of idiopathic HOPP cases within a relatively small area in a short span of time in contrast to most other Indian studies, where the share of idiopathic aetiology is comparatively smaller. This underscores the importance of a larger study with genetic analysis on the aetiology of the idiopathic HOPP in this area.DOI: http://dx.doi.org/10.3126/ajms.v6i6.12482 Asian Journal of Medical Sciences Vol.6(6) 2015 83-87