Rakesh Lalla

DTI correlates of cognition in term children with spastic diplegic cerebral palsy

BACKGROUND AND AIMS Presently, there is no published study that shows association between cognition and white matter injury in spastic cerebral palsy. We aimed to correlate cognitive functions with diffusion tensor imaging derived metrics in spastic diplegic children. METHODS Twenty-two term children with spastic diplegia and 22 healthy controls were recruited. All patients were graded on the basis of gross motor function. The Indian children intelligence Test was used to quantify cognition and diffusion tensor imaging was used to quantify microstructural changes in various white matter regions. Diffusion tensor imaging metrics were quantified by placing regions of interests in different white matter regions like corona radiata, anterior limb of internal capsule, posterior limb of internal capsule, mid brain, pons, medulla, genu, splenium, temporal white matter, parietal white matter, frontal white matter and occipital white matter. RESULTS Spastic diplegic children showed significantly lower neuropsychological test scores as compared to controls. A significantly decreased fractional anisotropy values were observed in corona radiata, anterior limb of internal capsule, posterior limb of internal capsule, mid brain, pons, medulla, genu, splenium and occipital white matter; however significantly increased mean diffusivity values were observed in corona radiata, anterior limb of internal capsule, posterior limb of internal capsule, mid brain, pons and genu in spastic diplegic as compared to controls. A significant positive correlation in fractional anisotropy and negative correlation in mean diffusivity was observed with neuropsychological test scores. CONCLUSION These results suggest that these imaging metrics may be used as a biomarker of cognitive functions in term children with spastic diplegia.

A rare presentation of cerebral venous sinus thrombosis associated with tubercular meningitis.

Central nervous system (CNS) tuberculosis may manifest as meningitis, meningoencephalitis, tuberculoma, tubercular abscess, stroke due to tuberculous vasculitis and tuberculous encephalopathy. Occasionally, tubercular meningitis (TBM) can predispose to cerebral venous sinus thrombosis (CVST). We report a young man, who developed CVST as a complication of TBM. Worsening of pre-existing headache, impairment of consciousness and seizures should raise suspicion of CVST in any patient with CNS infection. Early diagnosis and appropriate clinical management are important for good outcome.

Mirror movements in progressive hemifacial atrophy.

Mirror movements are simultaneous, involuntary, identical movements occurring during contralateral voluntary movements. These movements are considered as soft neurologic signs seen uncommonly in clinical practice. The mirror movements are described in various neurological disorders which include parkinsonism, cranio veretebral junction anamolies, and hemiplegic cerebral palsy. These movements are intriguing and can pose significant disability. However, no such observation regarding mirror movements in progressive hemifacial atrophy have been reported previously. We are reporting a teenage girl suffering from progressive hemifacial atrophy and epilepsy with demonstrable mirror movements in hand.

Pituitary apoplexy syndrome as the manifestation of intracranial tuberculoma

Pituitary apoplexy syndrome is characterised by acute neuro-ophthalmological features and usually occurs due to bleeding in a pituitary adenoma. It is an unusual presentation of tuberculoma, as only few similar cases have been reported previously. A 17-year-old girl presented with headache, vomiting, altered sensorium and vision loss. MRI of the brain revealed ring enhancing sellar lesions with other enhancing lesions and leptomeningeal enhancement. Cerebrospinal fluid microscopy, biochemistry and PCR for tuberculosis confirmed tubercular meningitis. The patient was treated with antituberculous therapy and was asymptomatic at the end of treatment.

Spastic foot-drop as an isolated manifestation of neurocysticercosis.

Foot-drop is a rare but important manifestation of intracranial pathologies ranging from space-occupying lesions to cerebrovascular accidents. Being most commonly associated with peripheral nerve lesions or radicular compressions, it remains an underappreciated feature of central-structural abnormalities. We describe an interesting case of a 14-year-old boy who had presented with acute onset right-sided foot-drop due to a left-sided parasagittal neurocysticercus lesion, without seizures and discuss the location of the lesion in the precentral area in reference to Penfields motor homunculus.

Hirayama disease: a frequently undiagnosed condition with simple inexpensive treatment

Hirayama disease is a non-progressive asymmetric juvenile muscular atrophy involving C7-T1 myotomes leading to weakness and atrophy of intrinsic muscles of hand and forearm with relative sparing of the brachioradialis muscle. This benign focal cervical poliopathy is believed to be caused by forward displacement of the posterior cervical dural sac. In this case report we highlight the importance of dynamic MRI of cervical spine for diagnosis of the above condition and the use of hard cervical collar to prevent further flexion injury.

Extensive acute disseminated encephalomyelitis in a young girl responding to intravenous methylprednisolone

Acute disseminated encephalomyelitis (ADEM) is a monophasic clinical syndrome, characterised by immune-mediated demyelination of the central nervous system. Differentiating ADEM from acute viral encephalomyelitis may pose a difficult clinical challenge. We describe a 3-year-old girl who presented with fever, weakness in all four limbs, urinary retention, respiratory distress and altered sensorium. MRI of the brain showed multiple cerebral T2-hyperintense signals with bilateral thalamic and basal ganglia involvement. MRI of the spine showed extensive T2-hyperintensities from cervical to lumbar spinal cord. Cerebrospinal fluid examination was normal. The patient was diagnosed as ADEM and treated with intravenous methylprednisolone. She showed complete clinical and radiological improvement at the end of 1-month follow-up.

Acute myeloneuropathy: An uncommon presentation of Sjögren's syndrome.

Sjögrens syndrome is associated with central and peripheral nervous system involvement. The peripheral neuropathy is usually a sensory predominant neuropathy or a cranial neuropathy. Myelopathy is usually of focal, subacute, chronic or relapsing type. Acute myeloneuropathy as the predominant manifestation has not been described in the literature. We describe a middle aged woman who presented with an acute onset motor quadriparesis and bladder dysfunction. She had dryness of eyes and mouth for 8 months. Nerve conduction studies revealed motor axonal neuropathy and magnetic resonance imaging of spinal cord showed T2 hyperintensities involving entire cord. Mild perineural fibrosis, focal foamy changes in endoneurium and lymphocytic infiltration were seen in sural nerve biopsy specimen. Patient improved clinically after intravenous methylprednisolone therapy.

Extensive extracranial and intracranial Varicella zoster vasculopathy.

Varicella zoster virus (VZV)-induced vasculopathy is an uncommon cause of stroke in a young immunocompetent host. Owing to scarcity of data of VZV-induced vasculopathy and lack of awareness about this condition and its diagnostic test, these cases may be easily missed. In this case, we report an immunocompetent host presenting right-side hemiplegia with motor aphasia and complete loss of vision in the left eye due to complete occlusion of the left common carotid artery without any history of skin rash preceding stroke. Cerebrospinal fluid analysis for varicella antibody revealed very high titres and CT aortogram demonstrated aortoarteritis with occlusion of left common carotid artery. To our knowledge, varicella zoster vasculopathy-associated aortoarteritis has not been described in the literature.

Purple glove syndrome: a dreadful complication of intravenous phenytoin administration

Purple glove syndrome is an uncommon but dreaded complication of intravenous phenytoin administration characterised by pain, oedema and purple-blue discolouration of the limb distal to the site of injection. We describe a 37-year-old gentleman having the characteristic purple glove appearance after phenytoin loading, and discuss the salient features of this syndrome highlighting the pathophysiological and preventive aspects.