Intractable vomiting and hiccups as the presenting symptom of neuromyelitis optica.

Vomiting and hiccups can be due to peripheral or central causes. Neurological diseases causing vomiting and hiccups are due to lesions of medulla involving area postrema and nucleus tractus solitarius. Neuromyelitis optica (NMO) is one such disease which involves these structures. However refractory vomiting and hiccups as the presenting symptom of NMO is unusual. Here we report a patient with NMO in whom refractory vomiting and hiccups were the sole manifestation of the first attack. Diagnosis can be missed at this stage leading to delay in treatment and further complications. This case demonstrates the importance of considering NMO in any patient presenting with refractory vomiting and hiccups and with local and metabolic causes ruled out and linear medullary lesion on magnetic resonance imaging may indicate the diagnosis even when the classical clinical criteria are not met. Anti NMO antibody testing should be done and if positive appropriate treatment should be initiated to prevent further neurological damage.

Cystic cochleovestibular anomaly presenting with congenital deafness and recurrent bacterial meningitis in childhood.

Recurrent bacterial meningitis (RBM) in many instances is associated with identifiable anatomical defects. Presence of congenital deafness with recurrent meningitis should alert clinician for presence of middle and inner ear malformation. These defects can be demonstrated by various neuro imaging techniques and can be surgically corrected. In this case report we describe a child seen at our institute with congenital deafness and recurrent meningitis, discuss the approach to RBM and briefly describe inner ear malformation associated with the same and how to differentiate them.

Clinicopathological profile of polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome.

Patients with polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome caused by underlying plasma cell dyscrasias, although rare, may present with peripheral neuropathy. In this retrospective study we describe the clinical, electrophysiological and pathological profile of 29 patients (27 males and two females, with a mean age of 45.9±7.9 years) seen between 1983 to 2009 who satisfied the Dispenzieri criteria. Polyneuropathy with weakness and paresthesias of the limbs evolving over 8.3±9.1 months, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes were seen in all patients. Other features included pedal edema (25 patients), papilledema (24), weight loss (23), clubbing (20) and Castleman disease (three patients). We report the pathological features seen in nerve biopsies, osteosclerotic lesions, skin biopsies, lymph node biopsies and bone marrow aspirates. We report the method of a diagnosis confirmation and compare the clinicopathological features of our patients with other published series. Our findings highlight the importance of a meticulous systemic examination and systematic laboratory investigations in the diagnosis of POEMS syndrome.

Familial amyotrophic lateral sclerosis: first report from India.

We report two patients diagnosed to have familial amyotrophic lateral sclerosis (FALS). A 40 year old lady had progressive weakness and atrophy of the limbs and bulbar palsy from the age of 39 years and with electrophysiological evaluation was confirmed as definite ALS. Her mother had presented in 1978 at the age of 42 years with symptoms and signs of ALS. The other patient was a 43 year old male with rapidly progressive weakness, wasting and spasticity of the limbs and bulbar palsy of 4 months duration and with electrophysiological evidence of diffuse anterior horn cell involvement. His father also had onset of illness at 43 years of age with gradually progressive spasticity and atrophy of the extremities followed by bulbar palsy. In the first instance the mother had a duration of illness of 8 years while in the second the father lived for 15 years after the onset of illness.

Myasthenia gravis in children: a longitudinal study.

BACKGROUND: Juvenile myasthenia gravis (JMG) is an uncommon disease. Unlike adults, clinical characteristics and outcomes of myasthenia gravis (MG) are not well studied in children. PATIENTS AND METHODS: Case records of 77 patients with MG who were 15 years of age or less at disease onset, evaluated over a period of 34 years at the National Institute of Mental Health and Neurosciences, Bangalore, India, were reviewed. Their clinical characteristics and response to therapy was compared with 290 patients with MG onset after 15 years of age. RESULTS: Median age at onset was 8 years and mean period of follow-up was 6.2 years (range 6 months to 25 years). At presentation, 30% of patients had ocular myasthenia and the rest had generalized disease. Twenty-one patients (27%) had disease confined to ocular muscles throughout the course and three had limb girdle myasthenia. Familial myasthenia was more common than adult onset disease, 10 patients had positive family history. Unlike adults, none of the patients had associated autoimmune disease. Fifty-two patients (67%) received corticosteroids, and azathioprine was added in five patients. Thymectomy was performed in 11 patients, six below the age of 15 years. Thymic histology was normal in one and showed hyperplasia in eight and thymoma in one. Four patients had crisis. At the end of follow-up, 25 patients were asymptomatic, 28 had partial improvement, and nine remained unchanged or worsened and two died. Ten patients achieved complete stable remission. CONCLUSIONS: This study shows some distinctive characteristics of JMG, such as higher frequency of ocular myasthenia, benign course, better long-term outcome and lack of association of thymoma and other autoimmune disorders.

Early Disappearance of Cord Signal Changes Following Endovascular Treatment of a Spinal Dural AV Fistula.

SUMMARY: Spinal dural arterio venous fistulas (SDAVFs) are the commonest type of spinal vascular malformations and are encountered mostly among adult patients. Magnetic Resonance Imaging (MRI) in patients with SDAVFs demonstrates features like spinal cord swelling and signal changes. Cord signal changes are believed to be due to venous stasis.Treatment of a spinal vascular malformation may result in complete or partial resolution of MR findings with associated clinical improvement. The earliest documentation of reduction or disappearance of cord signal changes on post treatment MR images, was one month later in one series in the literature.We present and discuss disappearance of cord signal changes on MRI within 72 hours following endovascular treatment by liquid glue injection in a patient with SDAVF.

Ulnar nerve palsy due to axillary crutch.

A young lady with residual polio, using axillary crutch since early childhood, presented with tingling, numbness and weakness in ulnar nerve distribution of five months duration. Ulnar motor conduction study revealed proximal conduction block near the axilla, at the point of pressure by the crutch while walking. Distal ulnar sensory conduction studies were normal but proximal ulnar sensory conduction studies showed absence of Erb's point potential. These findings suggested the presence of conduction block in sensory fibers as well. Proper use and change of axillary crutch resulted in clinical recovery and resolution of motor and sensory conduction block.

Puerperal cerebral venous thrombosis: therapeutic benefit of low dose heparin.

Advances in imageology have improved the diagnostic yield of cerebral venous/sinus thrombosis (CVT). However, its management remains a challenge. The present study was carried out to study the role of heparin in CVT. Therapeutic outcome of 150 patients of puerperal CVT manifesting within one month of delivery or abortion, was analyzed. The diagnosis was supported by cranial computed tomography and/or angiography whenever required. Seventy three patients, 46 with non-haemorrhagic infarction and 27 with haemorrhagic infarction, received 2500 units of subcutaneous heparin, three times a day within 24 hours of hospitalization till 30th post partum day or symptomatic relief. Seventy seven patients during the same period, 50 with non-haemorrhagic infarction and 27 with haemorrhagic infarction, who did not receive heparin formed the control group. Repeat CT scans were done when indicated. Among the heparin group, 34 patients made full recovery. There were eight deaths, all among the patients with haemorrhagic lesion. In control group, only 14 patients recovered completely (P=<0.001) and 18 died (P=<0.001). There were no adverse effects of heparin. Low dose heparin is safe and efficacious in puerperal CVT, even in patients with haemorrhagic infarction.

Heparin in haemorrhagic infarction in cerebral venous sinus thrombosis.

Morbidity and mortality in puerperal cerebral venous thrombosis (CVT) can be reduced by arresting the progression of thrombosis using heparin. However, conventional dose of heparin requires monitoring of coagulation parameters and carries a risk of haemorrhage. The present study involved 56 patients of puerperal CVT with CT evidence of haemorrhagic infarction. Twenty nine of these patients received low dose heparin till 30th post-partum day or symptomatic relief. Their clinical features and severity were similar to 27 patients who did not receive heparin. The mortality and morbidity at discharge was significantly less (P < 0.001) in heparin treated group. There were no haemorrhagic complications. Low dose heparin is safe and effective in cerebral venous thrombosis, even with haemorrhagic infarction.

Sensory dysfunction in GB syndrome: a clinical and electrophysiological study of 100 patients.

Studies of sensory system involvement in Guillain Barre' (GB) Syndrome are sparse in the literature. This communication presents the clinical and electrophysiological data of 100 patients of GB Syndrome evaluated over 5 years at NIMHANS, Bangalore, India. Sensory symptoms or signs were present in 45% and 59% of patients in upper and lower limbs respectively and were distal and symmetrical. Impairment of joint position and vibration sense was the commonest finding and was associated with a greater need for ventilatory support and autonomic disturbances. Sensory nerve conduction studies involved median, ulnar and sural nerves and electrophysiological evidence of abnormality was present in at least one sensory nerve in 80% of cases. These were: absent sensory nerve action potential (SNAP) in 19%-41%, reduced SNAP amplitude in 28-34% and reduced conduction velocity in 8%-15%. "Abnormal Median and normal Sural response", the pattern characteristic of demyelinating neuropathy, was noted in 29% of the cases. While sensory symptoms and signs were more frequent in lower limbs, electrophysiological abnormalities were more frequent in median and ulnar nerves. There was good association between motor and sensory conduction parameters in median and ulnar nerves. The time of performance of nerve conduction studies did not influence the results. Electro-clinical study of sensory system adds to our understanding of GB Syndrome.

Basal ganglionic angioleiomyoma.

A rare case of basal ganglionic angioleiomyoma in a 12-year-old girl is reported. She presented with features of raised intracranial tension, multifocal seizures, left hemidystonia and apraxia of eyelid closure. Cranial CT scan showed a large hypodense lesion with an enhancing mural nodule in the region of head of the caudate nucleus on the right side. The lesion was extending to the posterior limb of internal capsule postero-medially and to globus pallidus laterally. Another small, hyperdense, enhancing lesion was seen in the region of globus pallidus on the left side. The right basal ganglionic mass was excised through frontal craniotomy. Histopathological, Immunocytochemical and Ultrastructural examination of the right basal ganglionic mass showed features characteristic of angioleiomyoma. Following surgery the patient improved initially with respect to dystonia but worsened later on. Repeat cranial CT, 20 months after surgery, showed bilateral frontotemporal atrophy without any tumor recurrence while the lesion on the left side remained quiescent.

Paradoxical masking of epileptic focus during hyperventilation.

Hyperventilation (HV) has traditionally been used to activate epileptiform discharges in EEG, especially in generalized epilepsies. A young girl, with complex partial seizures secondarily generalised had frequent spike discharges (1spike/4.2 sec.) in left anterior temporal region in the resting record. During hyperventilation the spike discharges became less frequent (1 spike/15 sec.) and later disappeared. The spike discharges reappeared gradually in post-hyperventilation period and 2 minutes after stopping hyperventilation the spike frequency increased to 1/3.2 sec. The decrease in spike discharges may be due to hyperventilation induced arousal response or decrease in spike discharges may be due to hyperventilation induced arousal response or decrease in Cerebral blood flow resulting in suppression of spike focus. It is also possible that HV induced slow waves may obscure the spike discharges. Such paradoxical reduction of spike discharge during HV warrants cautions interpretation of the results of EEG when HV is used as a routine activation procedure.

Sarcoidosis with primary neurological involvement.

Neurosarcoidosis is rare, accounting for less than 5 percent of all cases of sarcoidosis. We report a case of neurosarcoidosis presenting with neuropsychiatric features and meningitis, confirmed by meningeal biopsy. The difficulties encountered in establishing the diagnosis and treatment are highlighted.

Disseminated Burkitt's lymphoma presenting as multiple cranial nerve palsies.

A case of disseminated Burkitt's lymphoma with nervous system involvement in a HIV negative 35 year old lady is described. She primarily presented with multiple cranial nerve palsies. At autopsy, diffuse involvement of parenchymatous organs and lymphomatous meningitis with conspicuous sparing of gastrointestinal system was observed. In addition, there was an unusual feature of paraneoplastic demyelinating peripheral neuropathy. Incidentally, a large hydatid cyst was also seen in the left lobe in addition to the lymphomatous involvement of the liver.