Propriospinal myoclonus: is it always psychogenic?

Propriospinal myoclonus (PSM) is a rare type of movement disorder in which the myoclonic jerks are present or intensified in supine position. Arising from a thoracic or abdominal generator, the movements propagate rostrocaudally as per the spinospinal conduction velocity. Routine neuroimaging and electrophysiology being normal in most instances, it is frequently interpreted as psychogenic.

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.

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.

Early treatment causes clinicoradiological reversal of myelopathy due to vitamin B12 deficiency.

Vitamin B12 deficiency has a wide spectrum of clinical presentation with a variety of neurological symptoms and signs. As a result, many patients lack classic features of advanced severe deficiency. Early diagnosis and treatment are crucial in order to prevent the irreversible damage to the nervous system.

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.

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.

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.

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.

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.