Approach to a case of myeloneuropathy

Myeloneuropathy is a frequently encountered condition and often poses a diagnostic challenge. A variety of nutritional, toxic, metabolic, infective, inflammatory, and paraneoplastic disorders can present with myeloneuropathy. Deficiencies of vitamin B12, folic acid, copper, and vitamin E may lead to myeloneuropathy with a clinical picture of subacute combined degeneration of the spinal cord. Among infective causes, chikungunya virus has been shown to produce a syndrome similar to myeloneuropathy. Vacuolar myelopathy seen in human immunodeficiency virus (HIV) infection is clinically very similar to subacute combined degeneration. A paraneoplastic myeloneuropathy, an immune-mediated disorder associated with an underlying malignancy, may rarely be seen with breast cancer. Tropical myeloneuropathies are classified into two overlapping clinical entities — tropical ataxic neuropathy and tropical spastic paraparesis. Tropical spastic paraparesis, a chronic noncompressive myelopathy, has frequently been reported from South India. Establishing the correct diagnosis of myeloneuropathy is important because compressive myelopathies may pose diagnostic confusion. Magnetic resonance imaging (MRI) in subacute combined degeneration of the spinal cord typically reveals characteristic signal changes on T2-weighted images of the cervical spinal cord. Once the presence of myeloneuropathy is established, all these patients should be subjected to a battery of tests. Blood levels of vitamin B12, folic acid, vitamins A, D, E, and K, along with levels of iron, methylmalonic acid, homocysteine, and calcium should be assessed. The pattern of neurologic involvement and results obtained from a battery of biochemical tests often help in establishing the correct diagnosis

Abnormal head movement in a patient with tuberculous meningitis

The bobble-head doll syndrome is characterised by abnormal head movements. These head movements are usually ‘yes–yes’ (up and down) type; rarely, head movements are ‘no–no’ (side-to-side) type. Commonly described causes of the bobble-head doll syndrome include third ventricular tumours, suprasellar arachnoid cysts, aqueductal stenosis and other lesions in the region of the third ventricle of the brain. We report a case of tuberculous meningitis with hydrocephalus; in this patient bobble-head doll syndrome developed following external ventricular drainage. In our patient, placement of intraventricular drain led to massive dilatation of the frontal horn of the left lateral ventricle because of blocked foramina of Monro on the left side. The bobble-head doll syndrome, presumably, developed because of the pressure effect of the dilated third ventricle on the dorsomedial nucleus of the thalamus, red nucleus and dentatorubrothalamic pathways. We think that distortion of the third ventricle was responsible for the impairment of the functions of all these structures

Seizures in patients with cerebral hemiatrophy: A prognostic evaluation

Purpose: Cerebral hemiatrophy is a common childhood disease. It clinically manifests with seizures, hemiparesis and mental retardation. Materials and Methods: In this prospective study, previously untreated patients with seizures and cerebral hemiatrophy were recruited. Cerebral hemiatrophy was diagnosed on the basis of hemispheric ratio. Patients with acquired hemiconvulsion, hemiplegia, and epilepsy (HHE) syndrome were included in group A. Group B included patients with congenital HHE syndrome. Patients were followed up for 6 months for seizure recurrence. Results: Out of 42 patients 26 were in group A and 16 were in group B. After 6 months, there was significant reduction in seizure frequency (P < 0.0001) in both the groups. At least 50% reduction in seizure frequency was noted in all the patients. Complete seizure freedom was observed in 15 (35.7%) patients. Seizure recurrences were significantly higher (P = 0.008) in group A. On univariate analysis, predictors of seizure recurrences were history of febrile seizures (P = 0.013), hippocampal sclerosis (P = 0.001), thalamic atrophy (P = 0.001), basal ganglia atrophy (P = 0.001), cerebellar atrophy (P = 0.01), ventricular dilatation (P = 0.001), epileptiform discharges at presentation (P = 0.023), complex partial seizures (P = 0.006) and status epilepticus (P = 0.02). On multivariate analysis, hemispheric ratio was the only significant factor for seizure recurrence. Conclusion: Patients with congenital hemiatrophy had better seizure control than that in patients with HHE syndrome

Focal neuromyotonia as a presenting feature of lumbosacral radiculopathy

Neuromyotonia is characterized by motor, sensory, and autonomic features along with characteristic electrophysiologic findings, resulting from hyperexcitability of the peripheral nerves. We describe the case of a 36-year-old man, who presented with the disabling symptoms suggestive of focal neuromyotonia involving both the lower limbs. His neurological examination revealed continuous rippling of both the calf muscles with normal power, reflexes, and sensory examination. Electrophysiology revealed spontaneous activity in the form of doublets, triplets, and neuromyotonic discharges along with the neurogenic motor unit potentials in bilateral L5, S1 innervated muscles. Magnetic resonance imaging lumbosacral spine revealed lumbar intervertebral disc protrusion with severe foraminal and spinal canal stenosis. Patient had good response to steroids and carbamazepine. The disabling focal neuromyotonia, occurring as a result of chronic active radiculopathy, brought the patient to medical attention. Patient responded to medical management

Simultaneous occurrence of axonal Guillain–Barré syndrome in two siblings following dengue infection

Guillain–Barré syndrome (GBS) is an infrequent neurological complication of dengue viral infection. It is broadly divided into either acute inflammatory demyelinating variety or an axonal variety (acute motor axonal and acute sensory and motor axonal variants). Axonal variants are distinctly infrequent. Two brothers (18 years and 15 years) were hospitalized on the same day with complaints of acute symmetric upper- and lower-limb weakness of 7 days' duration. They did not have overt manifestations of dengue fever which preceded the neurological presentation. They had flaccid areflexic pure motor quadriparesis. Dengue IgM serology was positive in both. Nerve conduction study showed predominant axonopathic changes in both the patients. Both the patients showed significant improvement with conservative treatment during hospital stay of 7 days. This is unusual report of dengue-associated axonal GBS in two brothers. Whether simultaneous occurrence of GBS in two siblings is a coincidence or genetically determined is still a question