D Nagaraja

Cerebrovascular disease in children.

Stroke although rare in children, is an important cause of morbidity in the paediatric age group. Over a period of 8 years, 43 children (17 boys and 26 girls) in the age groups of 1–16 years (mean 8.02 yrs) presented with stroke which constituted 10% of all strokes in the young and 0.7% of all paediatric admissions. The chief clinical features were hemiplegia (86%), convulsions (27%), fever (23%). dysphasia (23%), headache (11%) and altered level of consciousness (11%). Routine laboratory tests were non‐contributory. Cranial computerized tomography (CCT) on 21 patients was abnormal in 95% and was useful in revealing the extent of infarction. Infarction was confined to middle cerebral artery territory, often involving basal ganglionic structures and was associated with focal or diffuse atrophy. Angiograms were abnormal in 78% of the patients (18/23) and were complimentary to the CCT. Etiological factors identified were: Moya‐moya disease 6, arteritis 5, fibromuscular dysplasia 2, scorpion sting 2, and venous sinus thrombosis and small vessel occlusion one each. Though 23% of the patients had fever at onset, no obvious evidence of systemic or CNS infection was noticed. Stroke in children continues to pose a diagnostic challenge.

Sleep-related disorders among a healthy population in South India

INTRODUCTION Sleep-related disorders (SRDs) though frequent, are under-reported and their implications are often neglected. OBJECTIVE To estimate SRDs in an apparently healthy South Indian population. MATERIALS AND METHODS Data was collected by administering a questionnaire including Sleep Disorders Proforma, Epworth Sleepiness Scale, and Pittsburgh Sleep Quality Index (PSQI) to 1050 apparently healthy attendants/relatives of patients attending a tertiary healthcare institution. RESULTS The mean age of the respondents was 35.1±8.7 years with even gender distribution (male: female; 29:21), work hours were 7.8±1.33 h and had regional representation from the southern Indian states. The majority of the respondents did not report any significant medical/psychiatric co-morbidities, hypertension was noted in 42.6%, in one-fourth, the body mass index (BMI) was >25, and in 7.7% the neck size was >40 cm. Daily tea (70.3%) and coffee (17.9%) consumption was common and 22.2% used tobacco. Average time-to-fall-asleep was 22 min (range: 5-90 min), average duration-of-actual-sleep was 7 h (range: 3.5-9.1 h) with the majority (93.8%) reporting good-quality sleep (global PSQI ≤5). The reported rates of SRDs varied between 20.0% and 34.2% depending on the instrument used in the questionnaire. Insomnia, sleep-related breathing disorders (SRBD), narcolepsy, and restless legs syndrome (RLS) were reported by 18.6%, 18.4%, 1.04% and 2.9%, respectively. Obesity was not strongly associated with SRBD. in 51.8% of subjects with SRBD BMI was <25 kg/m 2 . Of the respondents with insomnia, 18% had difficulty in initiating sleep, 18% in maintaining sleep and 7.9% had early morning awakening. Respondents attributed insomnia to depression (11.7%) or anxiety (2.5%). Insomnia was marginally high in females when compared to males (10.3% vs. 8.3%) and depression was the major reason. RLS, which was maximal at night, was responsible for delayed sleep onset (74.2%). Other SRDs included night terrors (0.6%), nightmares (1.5%), somnambulism (0.6%), and sleep-talking (2.6%). Family history of SRDs was present in 31.4% respondents. While, only 2.2% of the respondents self-reported and acknowledged having SRD, health-seeking was extremely low (0.3%). CONCLUSION SRDs are widely prevalent in India. Considering the health implications and poor awareness, there is a need to sensitize physicians and increase awareness among the public.

Pure word deafness in two patients with subcortical lesions

We report two patients with pure word deafness (PWD) with tumour in the III ventricle region with obstructive hydrocephalus. A diagnosis of PWD was made in these two patients in view of impaired verbal comprehension in the presence of adequate hearing, intact acoustic stapedius reflex and well preserved environmental sound perception. Return of verbal comprehension following the radiation therapy observed is probably due to the reduction of the tumour mass and the release of thalamocortical auditory pathways from its compressive effect. Our findings support the hypothesis of the presence of discrete auditory pathways for mediation of verbal and non-verbal stimuli independently.

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.

Acute ataxic neuropathy: a clinical, electrophysiological and morphological study

ABSTRACT Sensory ataxia as the chief manifestation of acute neuropathy is rather rare. Of the 224 cases of acute polyneuropathy seen over 6 years (1984‐1990) only 10 patients (M:F 3:7) had disabling ataxia as the presenting feature. Their ages ranged from 14‐61 years. Antecedent febrile illness was present in 6 patients and the peak deficit evolved over 2‐25 days. Severe ataxia, paresthesia, distal areflexia and predominant joint sense loss were common to all, motor weakness was either absent or insignificant. CSF was acellular and revealed elevated protein in 3 subjects. All patients had electrophysiological evidence of severe sensory neuropathy with mild or no motor neuropathy. Sural nerve biopsy in one patient showed loss of large, as well as small, diameter myelinated fibres, secondary demyelination, but no evidence of inflammation. At follow up marginal to moderate improvement in ataxia was noted in only 5 patients. Absence of ophthalmoplegia and motor weakness, poor prognosis and characteristic electrophysiological and histopathological observations suggest that acute ataxic neuropathy may be a distinct entity.

Lower motor neuron facial palsy in cerebral venous sinus thrombosis

With advances in the neuro-imaging modalities, diverse manifestations of the cerebral venous sinus thrombosis (CVT) are being recognized. There are very few reports of isolated cranial nerve palsies in CVT. In this case report, we describe a patient of lower motor neuron facial palsy with CVT who was successfully treated with anticoagulation, highlighting the atypical manifestation of the disease.

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.

Chronic progressive encephalopathy, intractable seizures, and neuropathy: A triad of neurological features in insulinoma

Sir, A 29-year-old lady presented with headache and refractory complex partial seizures with secondary generalization of 8 months duration. Patient also had behavioral, personality changes, severe depression, and fl uctuating altered sensorium. There was no history of fever, weight loss, hypotension, skin pigmentation, or substance abuse. Patient was irritable, bed bound, obeying simple commands with spontaneous eye opening, and uttering incomprehensible sounds. She had spastic dysarthria and dysphagia. Patient could withdraw limbs to pain, had spasticity with contractures in all the limbs, brisk deep tendon reflexes, and bilateral extensor plantar response. She was extensively investigated and the relevant investigations included fasting blood sugar which was very low (36 mg/dl). Further evaluation for hypoglycemia found elevated serum fasting insulin level (7.54 mcU/ml), normal serum fasting cortisol (18.19 mcg/dl), C-peptide (1.8 mg/ ml), and thyroid hormone profi le. Magnetic resonance imaging (MRI) of brain revealed only mild generalized cortical atrophy. Electroencephalogram showed frontal intermittent rhythmic delta activity (FIRDA) and background slowing. Nerve conduction studies revealed evidence of sensory motor axonopathy with evidence of active denervation on needle electromyography. Computerized tomography and endoscopic ultrasound of abdomen revealed contrast-enhancing lesion in the body of pancreas, suggestive of insulinoma [Figure 1]. The patient underwent distal pancreato-splenectomy. Following surgery, she showed signifi cant improvement in cognition and is seizure free. She had postoperative extrapyramidal features in the form of hand tremors, cervical dystonia which gradually resolved with treatment, and she also developed iatrogenic diabetes mellitus.

Headache patterns in cerebral venous sinus thrombosis

Objectives: The objective of this study was to assess the clinical characteristics, patterns, and factors associated with headache in patients with cerebral venous sinus thrombosis (CVT). Methods: In this prospective cohort study, we recruited conscious CVT patients who were able to give reliable history after consent. Institutional ethics approval was obtained. The diagnosis of CVT was based on the clinical and imaging parameters. Data regarding headache characteristic, severity (visual analog scale [VAS]), imaging findings and outcome was recorded. Results: Forty-seven patients (19 males and 28 females) with mean age 29.7 ± 8.7 years were recruited. The mean duration of headache was 12.6 ± 26.8 days, and VAS was 79.38 ± 13.41. Headache onset was acute in 51.1%, subacute in 42.6%, thunderclap in 4.3%, and chronic in 2.1%; location was holocranial in 36.2%, frontal in 27.7% patients; description was throbbing in 44.7% and aching in 25.5% patients. Superior sagittal sinus and transverse sinus were involved in 63.8% cases each. The prothrombotic factors were anemia in 55.3%, puerperal in 38.3%, hyperhomocysteinemia in 29.8%, and polycythemia in 19.1%. Conclusion: Holocranial and bifrontal headache of increasing severity may be a marker of CVT. This may be useful in clinical judgment in identifying conscious patients with CVT.