Heramba Narayan Praharaj

Acute transverse myelitis following Japanese encephalitis viral infection: an uncommon complication of a common disease.

Japanese encephalitis (JE) is an epidemic encephalitis characterised by altered sensorium, convulsions, headache, brainstem signs with pyramidal and extrapyramidal features. Immune-mediated manifestation as acute transverse myelitis (ATM) has not been previously reported in JE. We describe a 40-year-old man who presented with an acute onset quadriparesis with urinary retention, which was preceded by fever and headache 3 weeks prior. He had elevated IgM titres against JE virus in serum and cerebrospinal fluid. MRI of cervico-thoracic spine demonstrated signal intensity alterations extending from C1 to D10 spinal segments. The patient was treated with intravenous methyl prednisolone for 5 days. He regained normal power at 6 months follow-up and repeat MRI study demonstrated complete resolution of the lesion. We conclude that in a case of JE, one should be vigilant for early diagnosis of possible complication as ATM, in which an early institution of immunomodulator therapy prevents adverse consequences.

Study of micronutrients (copper, zinc and vitamin B12) in posterolateral myelopathies

BACKGROUND Vitamin B12 deficiency is a well recognized cause of posterolateral myelopathy. In Indian subcontinent, it may coexist with nutritional copper deficiency producing partial response of patients to B12 supplementation. Hence the study was planned to look for association of hypocupremia and B12 deficiency. METHODS Twenty-three patients with posterolateral myelopathy (Romberg sign positive) were enrolled and investigated for levels of vitamin B12, copper and zinc and followed up for six months. RESULT In three patients, copper deficiency alone was found to be the cause. In another three, both copper and vitamin B12 were deficient. In all these six patients, ceruloplasmin and 24h urinary copper were found to be low suggesting dietary copper deficiency. Hyperzincemia was found in four of these patients. Magnetic resonance imaging of spine was normal in lone Cu deficient patients but showed T2 hyperintensity of posterior column in lone B12 or combined B12 and copper deficiency. CONCLUSION In cases of B12 deficiency myelopathy not responding to supplementation, copper deficiency must be sought at the earliest to avoid and treat persistent neurological disability.

Hemichorea-hemiballism as the presenting manifestation of diabetes mellitus.

A 70-year-old woman presented with a history of acute onset abnormal movements of left half of the body for the previous 2 days. The movements were rapid, jerky, non-rhythmic present through out the day and were not associated with loss of consciousness, tongue bite, urinary incontinence. There was no history of fever, headache, nausea or vomiting associated with the movements. There was no previous history of diabetes mellitus, hypertension, tuberculosis, trauma or epilepsy in the past. General examination revealed no abnormality except for bilateral xanthelasma. Higher mental function and cranial nerve examinations were normal. There was no weakness of limbs. There was no sensory …

New onset refractory status epilepticus (NORSE) as the heralding manifestation of herpes simplex encephalitis.

New onset refractory status epilepticus (NORSE) is a relatively novel concept used to describe a cohort of previously healthy young adults mostly women presenting with denovo refractory status epilepticus which has a miserable impact on the outcome. Various infectious and non-infectious causes have been considered to be responsible for this dreaded syndrome; however, many a times the exact cause is not identified. As therapy with antiepileptic and anaesthetic drugs is not so successful, identifying and treating the exact cause could improve the outcome. Here the authors describe a woman who presented with NORSE. Investigations confirmed the diagnosis of herpes simplex encephalitis (HSE) and she responded drastically to acyclovir along with complete control of seizures. In this case, NORSE was the presenting feature of HSE and the refractoriness of her seizures was terminated only after treating the exact cause, that is, encephalitis.

Bilateral facial palsy as a manifestation of Japanese encephalitis.

Japanese encephalitis is a cause of substantial morbidity and mortality, prevalent mainly in South East nations. It is caused by group B arbovirus and transmitted with bite of infected culex mosquitoes. The clinical features described are: headache, vomiting, altered sensorium, convulsions and both hypokinetic and hyperkinetic movement disorders. In this submission, we described a 68-year-old man suffering from Japanese encephalitis, who presented with bilateral facial palsy with encephalitic features. The bilateral facial palsy has not been reported previously in association with Japanese encephalitis.

Unusual association of Arnold-Chiari malformation and vitamin B12 deficiency.

Arnold-Chiari malformations are a group of congenital or acquired defects associated with the displacement of cerebellar tonsils into the spinal canal. First described by Chiari (1891), this has various grades of severity and involves various parts of neuraxis, for example, cerebellum and its outputs, neuro-otological system, lower cranial nerves, spinal sensory and motor pathways. The symptomatology of Arnold-Chiari malformations may mimic multiple sclerosis, primary headache syndromes, spinal tumours and benign intracranial hypertension. We highlighted a case of Chiari type I malformation, who presented with posterolateral ataxia associated with significant vitamin B12 deficiency. The patient was supplemented with vitamin B12 injections and showed remarkable improvement at follow-up after 3 months.

Reflex gelastic-dacrystic seizures following hypoxic-ischaemic encephalopathy.

Reflex or stimulus-sensitive epilepsies are uncommon epileptic syndromes triggered by exogenous-specific sensory stimulus or endogenous various mental activities. Gelastic–dacrystic seizures are rare epileptic manifestations characterised by ictal laughter and crying. Gelastic–dacrystic seizures are commonly caused by hypothalamic hamartoma but rarely described due to cortical dysplasia, lesions of frontal and temporal lobes, tumours and vascular malformations. We report a young woman who presented with somatosensory-evoked gelastic–dacrystic seizures. This patient had a positive history of perinatal insult substantiated by MRI findings. Hypoxic–ischaemic encephalopathy as the cause of gelastic–dacrystic seizures has not been reported so far in the literature.

A rare case of extensive leptomeningitis in neurosarcoidosis

Sarcoidosis is a systemic disease characterised by non-caseating granulomas affecting mainly the lung and lymphatics. Literature reveals that 5% of the patients of sarcoidosis have involvement of the nervous system. Various neurological complications of sarcoidosis commonly reported are cranial nerve palsies, aseptic meningitis, myelopathy and intracranial masses. We report unusual case of neurosarcoidosis who presented with combination of right-sided vision loss and complete ophthalmoplegia with extensive leptomeningitis. This patient was suffering from resistant diabetes which leads to therapeutic problems.

Unusual association of eclamptic encephalopathy and Japanese encephalitis.

Eclampsia is one of the hypertensive disorders of pregnancy causing considerable maternal morbidity and mortality. It is more common in developing nations, where access to adequate obstetric care is limited to a small population. Eclampsia is clinically characterised by encephalopathy, seizures, headache and cortical blindness and occurs in the setting of pre-eclampsia that comprises hypertension, proteinuria and peripheral oedema. Japanese encephalitis has emerged as a serious neurological disorder in South East Asian countries. In this case report, we highlight the unusual association of eclamptic encephalopathy and Japanese encephalitis in a patient belonging to northern India. This combination of illnesses caused diagnostic difficulty and delayed hospitalisation. A high index of suspicion is required in the endemic zone of Japanese encephalitis if the expected results are lacking in patients with eclampsia.

Intracranial venous sinus thrombosis mimicking Lhermitte Duclos disease

A middle-aged man presented with 1-month history of headache, dizziness, staggering of gait and progressive deafness. Examination revealed decreased visual acuity, papilloedema, gaze evoked nystagmus, bilateral sensorineural deafness and gait ataxia. MRI showed TI iso and T2 hyperintensity of cerebellum, prominence of folia with gyral enhancement in a tigroid pattern making impression of the rare entity, Lhermitte Duclos disease (LDD) or dysplastic gangliocytoma of cerebellum. Conservative management for the raised intra cranial pressure did not have any impact on patients general condition for which an MR venogram was planned and it surprisingly showed non-visualisation of left transverse sinus and internal jugular vein, suggestive of thrombosis. Patient was treated with anticoagulants but he succumbed to his illness soon after. We present this case to convey that a high index of suspicion should always be there in mind for this commoner condition (venous sinus thrombosis) before diagnosing the rarer ones (here LDD disease) to prevent the disastrous consequences of the former.