ABSTRACT
Objectives: Demyelinating diseases of central nervous system (CNS) are a broad spectrum of conditions with autoimmune process against myelin. In a resource limited country like India, it is imperative to perform proper clinical evaluation, neuroimaging to differentiate among various categories of CNS demyelinating diseases to decide regarding further workup and treatment. The objective of our study was to determine clinical presentation, imaging findings, serology results, diagnosis, and treatment outcome of primary demyelinating disorders of CNS. Materials and Methods: In this prospective study, a total of 44 patients were enrolled over a period of 1 year. After proper evaluation, patients were categorized into different groups applying newer diagnostic criteria. Patients were treated with steroids, appropriate immunomodulatory therapy, and outcomes were analyzed. Results: The majority of cases were of neuromyelitis optica spectrum disorder (NMOSD) (45.5%) with an overall female-to-male ratio of 3.4:1 and mean age of presentation was 30.5 ± 11.15. Myelitis (52.3%) followed by optic neuritis (45.5%) was the most common initial presentation. The most common site of involvement on magnetic resonance imaging was the spinal cord (particularly the cervicodorsal cord). The majority showed good response to therapy (77.27%) and two patients did not survive. Conclusion: Higher disability observed among seropositive NMOSD patients warrants aggressive treatment during the first attack itself. It is important to suspect myelin oligodendrocyte glycoprotein antibody disease in patients with preceding viral infection. A good outcome in the majority is likely due to the availability of serological assays and aggressive immunomodulatory therapy.
ABSTRACT
We report about two young males who developed significant proximal weakness of all four limbs secondary to intracranial hypertension due to intracranial venous sinus thrombosis. Intracranial venous sinus thrombosis can manifest in a variety of ways which includes isolated intracranial hypertension, focal neurological symptoms or signs and acute or subacute encephalopathy. Various false localising signs have been reported to occur in patients with raised intracranial pressure including cranial nerve palsies and extensive radiculopathy. In a patient presenting with flaccid areflexic quadriparesis and papilledema, the possibility of a potentially reversible dysfunction of the cranial nerves and spinal nerve roots due to a marked rise in intracranial and intraspinal pressure must be recognised. Lumboperitoneal shunt to reduce the intraspinal pressure on the spinal nerve roots has been advocated to reverse the symptoms of extensive radiculopathy in such patients. Both of our patients showed remarkable improvement in symptoms and signs with medical treatment of CVT.
Subject(s)
Intracranial Hypertension , Papilledema , Polyradiculopathy , Sinus Thrombosis, Intracranial , Cranial Sinuses , Humans , Male , Sinus Thrombosis, Intracranial/complications , Sinus Thrombosis, Intracranial/diagnostic imagingSubject(s)
Central Nervous System Vascular Malformations/diagnostic imaging , Central Nervous System Vascular Malformations/etiology , Lateral Sinus Thrombosis/complications , Lateral Sinus Thrombosis/diagnostic imaging , Angiography, Digital Subtraction , Fatal Outcome , Humans , Magnetic Resonance Angiography , Middle AgedABSTRACT
Strokes involving posterior inferior cerebellar arteries (PICA) are rare and usually unilateral. They can involve either the lateral division or the medial division of PICA. Sudden simultaneous cerebellar infarctions in the medial PICA territories are extremely rare. We report one such patient who presented with acute bilateral cerebellar infarctions in the medial PICA territories. She was a diabetic and had features of diffuse atherosclerotic vertebrobasilar disease. We discuss the anatomical peculiarities of PICA and its blood supply along with the clinical features differentiating the medial and lateral PICA infarctions. We also list out the possible mechanisms of bilateral medial PICA territory infarction. Though bilateral they generally have a good prognosis considering the very small area of blood supply.
Subject(s)
Cerebellum/blood supply , Cerebral Arteries , Cerebral Infarction , Cerebral Infarction/diagnostic imaging , Female , Humans , Middle AgedSubject(s)
Acidosis, Renal Tubular/etiology , Hypokalemia/etiology , Paralysis/etiology , Sjogren's Syndrome/complications , Acetazolamide/therapeutic use , Acidosis, Renal Tubular/diagnosis , Acidosis, Renal Tubular/therapy , Adult , Female , Humans , Hypokalemia/diagnosis , Hypokalemia/therapy , Immunosuppressive Agents/therapeutic use , Potassium/therapeutic use , Prednisolone/therapeutic use , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/etiology , Sjogren's Syndrome/therapy , Treatment OutcomeSubject(s)
Anesthesia, General/adverse effects , Blindness/etiology , Compartment Syndromes/etiology , Decompression, Surgical/adverse effects , Magnetic Resonance Imaging , Oculomotor Muscles/diagnostic imaging , Orbital Diseases/etiology , Adult , Blindness/diagnosis , Compartment Syndromes/diagnosis , Compartment Syndromes/drug therapy , Humans , Lumbar Vertebrae/surgery , Male , Methylprednisolone/therapeutic use , Ophthalmoplegia/diagnosis , Ophthalmoplegia/drug therapy , Ophthalmoplegia/etiology , Orbital Diseases/diagnosis , Orbital Diseases/drug therapy , Postoperative Complications/diagnosis , Prone Position , Vision Disorders/diagnosis , Vision Disorders/drug therapy , Vision Disorders/etiologyABSTRACT
Parry-Romberg syndrome is a rare clinical entity characterised by progressive hemifacial atrophy with appearance of 'saber'. Various neurological and otorhinolaryngological disorders are associated with this syndrome. The association of Parry -Romberg syndrome with Spasmodic dysphonia has rarely been reported. A 37 year old female presented with progressive atrophy of tissues of left side of face for 10 years and change in voice for 1 year. On examination, wasting and atrophy of tissues including tongue was noted on left side of the face. ENT examination revealed adductor spasmodic dysphonia. We report the rare association of Parry -Romberg syndrome with spasmodic dysphonia.
Subject(s)
Dysphonia/etiology , Facial Hemiatrophy/complications , Facial Hemiatrophy/diagnosis , Adult , Dysphonia/diagnosis , Female , HumansSubject(s)
Bardet-Biedl Syndrome/complications , Epilepsy, Tonic-Clonic/complications , Adolescent , Humans , MaleSubject(s)
Abducens Nerve/abnormalities , Duane Retraction Syndrome/complications , Lacrimal Apparatus Diseases/complications , Lacrimal Apparatus/innervation , Tears/metabolism , Abducens Nerve/pathology , Child , Cranial Nerve Diseases/diagnosis , Eating , Humans , Lacrimal Apparatus Diseases/congenital , Magnetic Resonance Imaging , Male , Oculomotor Nerve/abnormalities , Oculomotor Nerve/physiopathology , Ophthalmoplegia/complicationsABSTRACT
The familial episodic ataxia type II is a rare, dominantly inherited disease characterized by episodes of ataxia of early onset, often with completely normal cerebellar function between attacks. We report a family with affected members who had features of episodic ataxia type II and cerebellar atrophy on MRI imaging. All the affected members were successfully treated with acetazolamide, a carbonic anhydrase inhibitor. They are asymptomatic at 2 year follow-up.