Annals of Indian Academy of Neurology
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   2008| April-June  | Volume 11 | Issue 2  
    Online since July 21, 2008

 
 
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REVIEW NEUROPATH
An insight into the biochemistry of inborn errors of metabolism for a clinical neurologist
Rita Christopher, Bindu P Sankaran
April-June 2008, 11(2):68-81
DOI:10.4103/0972-2327.41873  PMID:19893643
Neurological dysfunction is an important manifestation of inherited metabolic disorders. Although these are more common in childhood, adult onset forms with a different clinical presentation are often encountered. Recent advances in the diagnosis and treatment of these conditions have substantially improved the outcome in many of these conditions. This makes it essential that the practicing physician be familiar with the clinical presentation and diagnosis of these disorders. For the evaluation of a patient with a possible inborn error of metabolism, simple screening tests may aid in the diagnosis and provide direction for more comprehensive laboratory analysis. In this review, we present a practical approach to diagnosis of neurometabolic disorders. Establishing a specific diagnosis in these disorders will enable the clinician in offering a definitive long-term treatment, prognosis and genetic counselling.
  13,359 895 2
REVIEW ARTICLE
Diagnostic approach to peripheral neuropathy
Usha Kant Misra, Jayantee Kalita, Pradeep P Nair
April-June 2008, 11(2):89-97
DOI:10.4103/0972-2327.41875  PMID:19893645
Peripheral neuropathy refers to disorders of the peripheral nervous system. They have numerous causes and diverse presentations; hence, a systematic and logical approach is needed for cost-effective diagnosis, especially of treatable neuropathies. A detailed history of symptoms, family and occupational history should be obtained. General and systemic examinations provide valuable clues. Neurological examinations investigating sensory, motor and autonomic signs help to define the topography and nature of neuropathy. Large fiber neuropathy manifests with the loss of joint position and vibration sense and sensory ataxia, whereas small fiber neuropathy manifests with the impairment of pain, temperature and autonomic functions. Electrodiagnostic (EDx) tests include sensory, motor nerve conduction, F response, H reflex and needle electromyography (EMG). EDx helps in documenting the extent of sensory motor deficits, categorizing demyelinating (prolonged terminal latency, slowing of nerve conduction velocity, dispersion and conduction block) and axonal (marginal slowing of nerve conduction and small compound muscle or sensory action potential and dennervation on EMG). Uniform demyelinating features are suggestive of hereditary demyelination, whereas difference between nerves and segments of the same nerve favor acquired demyelination. Finally, neuropathy is classified into mononeuropathy commonly due to entrapment or trauma; mononeuropathy multiplex commonly due to leprosy and vasculitis; and polyneuropathy due to systemic, metabolic or toxic etiology. Laboratory investigations are carried out as indicated and specialized tests such as biochemical, immunological, genetic studies, cerebrospinal fluid (CSF) examination and nerve biopsy are carried out in selected patients. Approximately 20% patients with neuropathy remain undiagnosed but the prognosis is not bad in them.
  12,787 1,466 6
ORIGINAL ARTICLES
Neuroretinitis, a great mimicker
Sunil K Narayan, Subashini Kaliaperumal, Renuka Srinivasan
April-June 2008, 11(2):109-113
DOI:10.4103/0972-2327.41879  PMID:19893649
Neuroretinitis is a less-known clinical entity, which can be funduscopically confused with papillitis or papilledema and with hypertensive, renal and infiltrative retinopathies as well as with retinal vein occlusion or anterior ischemic optic neuropathy. Report: Two young patients presented with sudden onset of blurring of vision. Ophthalmic evaluation revealed a characteristic picture of neuroretinitis. Detailed study of the cases failed to indicate any specific etiology, thereby suggesting the diagnosis of idiopathic neuroretinitis. Although funduscopy showed marked inflammatory changes in the retina and optic nerve head, the recovery following medical treatment was remarkable. Comment: Familiarity with the fundus picture and awareness of the specific causes of neuroretinitis among neurologists and physicians may enable a prompt clinical diagnosis, avoidance of expensive brain imaging studies and early referral for appropriate and effective therapy. A brief review of the literature is presented suggesting a need for further studies to establish specific environmentally determined etiological factors such as infections and the effectiveness of the current modalities of treatment.
  9,518 344 4
TECHNICAL NOTES
Technical notes for digital polysomnography recording in sleep medicine practice
Manjari Tripathi
April-June 2008, 11(2):129-138
DOI:10.4103/0972-2327.41887  PMID:19893657
  6,506 318 -
CASE REPORTS
An unusual case of rapidly progressive contractures: Case report and brief review
R Subasree, Samhita Panda, Pramod Kumar Pal, S Ravishankar
April-June 2008, 11(2):119-122
DOI:10.4103/0972-2327.41882  PMID:19893652
An 8-year-old boy, diagnosed as cervical dystonia, was referred to our tertiary center. After a trivial trauma he had developed painful lumps in the axial region, which was followed by restricted movements of neck, shoulder, and abdominal muscles over 4 months. He had kyphoscoliosis, torticollis, rigid abdomen, and multiple muscle contractures. He also had short great toes. A detailed skeletal survey showed calcification in the soft tissues surrounding the shoulder anterior chest wall, thorax, and paraspinal muscles; there was also beaking of vertebrae, which was confirmed by CT thorax. This report showcases the diagnostic challenge posed by myositis ossificans progressiva, which can rarely cause rapidly progressing muscle contractures. A brief review of literature is also presented.
  5,694 172 1
Intraluminal dural venous sinus cyst simulating as aerocele in computerized tomography brain
Rajul Rastogi
April-June 2008, 11(2):116-118
DOI:10.4103/0972-2327.41881  PMID:19893651
Intradural venous sinuses are commonly visualized structures in the CT brain, even in noncontrast images. Rarely, hypoattenuating focal lesions may be observed within their lumen as a coincidental finding, which may remain undiagnosed. However, when such lesions appear in the patients who are scanned for head injury, they might pose diagnostic difficulties
  4,737 91 4
ORIGINAL ARTICLES
Unexplained neurological events during bathing in young people: Possible association with the use of gas geysers
Prabhjeet Singh, Anuraag Lamba, Rajinder Bansal, Gagandeep Singh
April-June 2008, 11(2):103-105
DOI:10.4103/0972-2327.41877  PMID:19893647
Here, we report sudden, unexplained neurological collapse in 14 young people while bathing with hot water associated with the use of liquefied petroleum gas (LPG)-based water heaters (gas geysers) in ill-ventilated bathrooms. None of the patients reported any circumstantial evidence of seizures or prior epilepsy. One patient developed cortical blindness and demonstrated posterior leucoencephalopathy on imaging studies. The remaining patients made rapid and excellent recovery without any residual neurological sequelae. In these cases, the results of all routine investigations, i.e., serum chemistry, brain imaging (computed tomography in 2 and magnetic resonance imaging in 10) and electroencephalography were normal. The clinical clustering of these cases in winter months with similar presentations of reversible encephalopathy probably indicates an inhalational toxin exposure. Therefore, we postulate a hypothesis that harmful emissions consisting of carbon monoxide (CO), hydrocarbon gases (HC) and nitrogen oxides (NOx), produced by incomplete combustion of LPG might be responsible for the cellular injury and subsequent transient neurological deficits. Physicians should be aware of this entity in order to avoid misdiagnosis of this condition as seizures, and a public awareness should also be created regarding the proper use of these devices.
  4,369 175 2
Migraine pain location in adult patients from eastern India
Ambar Chakravarty, Angshuman Mukherjee, Debasish Roy
April-June 2008, 11(2):98-102
DOI:10.4103/0972-2327.41876  PMID:19893646
Background: Sparse literature documenting the location of pain at the onset of migraine attacks and during established headaches is available. Objectives: A prospective study (2003-05) on 800 adult migraine patients (International Classifications of Headache Disorders (ICHD), 2:1.1, 1.2.1 and 1.6.1) was conducted to document (a) sites of onset of pain and (b) location of pain during established attacks (in >50% occasions) through semistructured interviews. Results: Demography: N = 800; M:F = 144:656 (1:4.56); age, 16-42 years (mean, 26 years); duration of migraine, 1-18 years (mean, 6.8 years). 87% of the subjects were ethnic Bengalis from the eastern Indian state of West Bengal, Calcutta being the capital city. Migraine types (on the basis of >50% headache spells): N = 800; 1.1:668 (83.5%); 1.2.1:18 (2.25%); 1.6.1:114 (14.25%). Location of pain at onset: Unilateral onset was present in 41.38% of the patients; of these, 53.17% had eye pain; 8.16%, frontal pain and 38.67%, temporal pain. In 32.25% of the patients, bilateral/central location of pain, mostly bitemporal or at vertex was noted. Cervico-occipital pain onset was noted in 26.43% patients (predominantly occipital, 14.68%; predominantly cervical, 11.75%). Location of established headaches: In 47.4% of the patients, with unilateral ocular or temporal onset, pain remained at the same site. Pain became hemicranial in 32.9%. In most patients, unilateral frontal onset pain (55.5%) became bilateral or holocranial. Most bilateral ocular (69.4%) and temporal onset (69.7%) pains remained at the same location. However, most bifrontal (55.6%) and vertex onset (56.9%) pains subsequently became holocranial. Most occipital pains at onset became holocranial (45.3%), but cervical pains subsequently became either hemicranial (38.3%) or holocranial (36.2%). Conclusions: This study documents location of pain at the onset and during established headaches in migraine patients largely from a specific ethnic group. Migraine with aura appears to be rare among ethnic Bengalis in eastern India. More than half had onset pain bilaterally/centrally and in the cervico-occipital regions. Only 40.5% experienced only unilateral pain. Cervico-occipital migraine pain appears to be common in ethnic Bengalis.
  4,263 192 3
VIEW POINT
Issues in evaluation of cognition in the elderly in developing countries
R Mathew, PS Mathuranath
April-June 2008, 11(2):82-88
DOI:10.4103/0972-2327.41874  PMID:19893644
Background: Developing regions of the world host the majority of elderly subjects who are at risk for dementia. Reliable epidemiological data from these countries is invaluable in tackling this global problem. Scarcity of such data in literature is largely attributable to problems that are unique to developing communities worldwide. Objective: To classify and describe the problems that interfere with the collection of reliable epidemiological data on cognitive impairment in the elderly in developing communities, and to suggest practical solutions for some of them. Methods: Inferring from the experiences of a large, ongoing, population-based study on the cognitive impairments in the elderly in South India and from the review of literature. Conclusion: A fatalistic attitude regarding aging in the communities, significant heterogeneity in educational abilities and activities of daily living, high illiteracy among rural subjects, and lack of an organized health care system and updated demographic figures are some of the major factors that contribute to technical, namely, methodology-related problems and practical, namely, subject-related problems in such epidemiological studies.
  4,145 248 5
ORIGINAL ARTICLES
Prevalence of dementia among Kashmiri migrants
Sunil Raina, Sushil Razdan, Kamal K Pandita, Sujeet Raina
April-June 2008, 11(2):106-108
DOI:10.4103/0972-2327.41878  PMID:19893648
Background: Neurological diseases are common disorders resulting in the loss of productive life and disability. Dementia is becoming a major public health problem in the developing world also. Aim: To ascertain the prevalence of dementia among Kashmiri Pandit population aged 60 years and above. Materials and Methods: A cross-sectional survey was conducted among the elderly population of the Kashmiris living in a migrant camp. We developed and used a Kashmiri version of the Mini-Mental State Examination as the test instrument, and a score below 24 was considered indicative of dementia. A functional ability questionnaire was also administered to the subjects. A neurologist carried out the examinations. Results: A sample comprising 200 subjects (95 males and 105 females) were evaluated. The prevalence of dementia is 6.5% among the Kashmiri Pandit population aged 60 years and above, which is higher than that reported from other parts of India.
  4,075 162 5
IMAGES IN NEUROLOGY
Transient and reversible focal lesion involving the splenium of the corpus callosum in a person with epilepsy
Nitin C Parikh, Makarand Kulkarni
April-June 2008, 11(2):123-124
DOI:10.4103/0972-2327.41883  PMID:19893653
  3,770 148 1
CASE REPORTS
Magnetic resonance imaging may simulate progressive multifocal leucoencephalopathy in a patient with chronic lymphocytic leukemia after fludarabine therapy
J Kalita, NS Patel, UK Misra
April-June 2008, 11(2):114-115
DOI:10.4103/0972-2327.41880  PMID:19893650
A 60-year-old male with chronic lymphatic leukemia (CLL) after 6 months of fludarabine therapy was admitted with status epilepticus and developed left hemiplegia. His magnetic resonance imaging revealed multiple T2 hyperintense lesions in the right frontal and left parieto-occipital lesion, simulating progressive multifocal leucoencephalopathy (PML). Cerebrospinal fluid Polymerase Chain Reaction (PCR) for JC virus was negative. We suggest the possible role of fludarabine in producing PML-like lesions in patients with Chronic Lymphocytic Leukemia (CLL).
  3,463 125 -
IMAGES IN NEUROLOGY
Cockayne syndrome
Firosh Khan, Thomas Chemmanam, PS Mathuranath
April-June 2008, 11(2):125-126
DOI:10.4103/0972-2327.41884  PMID:19893654
  3,026 205 1
LETTER TO EDITOR
Immediate switching from a chrono to an extended-release formulation of divalproex sodium: A two-part study in patients with controlled epilepsy
Rakesh Shukla, Sanjay Prakash, Asad Abbas
April-June 2008, 11(2):127-128
DOI:10.4103/0972-2327.41886  PMID:19893656
  2,322 146 -
EDITORIAL
Climate, health and disease
Sanjeev V Thomas
April-June 2008, 11(2):67-67
DOI:10.4103/0972-2327.41872  PMID:19893642
  2,153 179 -
LIGHTER MOMENTS
Individual reflections
Sreedhar K Krishna
April-June 2008, 11(2):126-126
PMID:19893655
  1,546 90 -
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