Acute haemorrhagic leukoencephalitis (AHLE) - our experience and a short review.

BACKGROUND: Acute haemorrhagic leukoencephalitis (AHLE), a rare variant of acute disseminated encephalomyelitis (ADEM), often presents differently from classical ADEM, thereby posing a diagnostic challenge to the clinician. AIM: To report AHLE, its clinic-radiological manifestations, process of diagnosis and prognosis. METHOD AND RESULTS: Eight patients presented with altered sensorium, acute focal deficits with or without seizures. Initial workup showed evidence of haemorrhagic lobar or thalamic lesions in seven patients. All patients underwent extensive evaluation for collagen vascular disease and vasculitis profile, autoimmune encephalitis panel and aquaporin-4 antibody, which were found to be normal. Cerebrospinal fluid (CSF) biochemistry and microscopy was non-contributory and CSF viral PCRs, toxoplasma antibodies, cryptococcal antigen were also negative. All patients had progressively worsening sensorium and neurological deficits. Repeat MRIs showed increase in oedema in the lesions and appearance/expansion of haemorrhage in the thalamic/hemispherical lesions. All patients received intravenous methylprednisolone (IVMP) without any benefit. Four patients underwent plasmapheresis (PLEX), one received intravenous immunoglobulin (IVIG) and one received both second line immunotherapies, without significant improvement. Brain biopsy (performed in three patients) showed inflammatory demyelination and areas of haemorrhage, thus confirming the diagnosis. Six patients succumbed in 7-30 days of the illness, despite aggressive treatment and only two survived, albeit with a significant disability. CONCLUSION: AHLE is a rare, yet very severe variant of ADEM. MRI shows lesions with haemorrhages, oedema and mass effect and histology findings reveal inflammatory infiltrates, haemorrhagic foci and fibrinoid necrosis of vessel walls. Prognosis is worse as compared to the classic ADEM, with a high mortality rate. To the best of our knowledge, this is one of the largest series of AHLE to have been reported anywhere in the world. KEYMESSAGE: Acute encephalopathy, multifocal deficits accompanied by haemorrhagic CNS demyelinating lesions with oedema and mass effect are the key features of AHLE. It is a rare, yet very severe form of ADEM with very high morbidity and mortality.

Neurology as career option among postgraduate medical students.

BACKGROUND: In the context of inadequacy of neurology workforce in India, it is important to understand factors that post-graduate medical students consider for and against choosing neurology as their career option. Understanding these factors will help in planning strategies to encourage students to pursue a career in neurology. At present, there is a paucity of studies addressing this issue in India. AIMS AND OBJECTIVES: (1) To analyze factors, which post-graduate students consider for and against choosing neurology as a career specialty. (2) To access the level and quality of neurology exposure in the current MBBS and MD curricula. MATERIALS AND METHODS: Statewide questionnaire based study was conducted in the state of Maharashtra for students eligible to take DM neurology entrance examination (MD Medicine and MD Pediatrics). RESULTS: In this survey, 243 students were enrolled. Factors bringing students to neurology were - intellectual challenge and logical reasoning (72%), inspired by role model teachers (63%), better quality-of-life (51%) and scope for independent practice without expensive infrastructure (48%). Factors preventing students from taking neurology were - perception that most neurological diseases are degenerative (78%), neurology is mainly an academic specialty (40%), neurophobia (43%) and lack of procedures (57%). Inadequate exposure and resultant lack of self-confidence were common (31%, 70-80%). 84% of the students felt the need for a short term certification course in neurology after MD. CONCLUSIONS: To attract more students to neurology, "role model" teachers of neurology could interact and teach students extensively. Neurologists' efforts to shed their diagnostician's image and to shift their focus to therapeutics will help change the image of neurology. Out-patient neurology clinics should be incorporated early in the student's career. Procedures attract students; hence, they should be made conversant with procedures and interventions. Increasing the level of neurological exposure in our current MBBS and MD curriculum is necessary. A case could be made for consideration of short certification course in neurology for physicians.

Clinical profile of Guillain Barre syndrome.

OBJECTIVES: To study clinical presentation, hospital care and outcome of patients of Guillain Barre Syndrome (GBS) and number of patients of respiratory failure and need for ventilators. To study efficacy of IVIg in patients of GBS. MATERIAL AND METHODS: 40 patients of GBS studied in detail including history, clinical examination and investigations (Nerve conduction velocity and C.S.F. examination). All patients were watched for respiratory insufficiency and those who developed respiratory paralysis were given assisted mechanical ventilation. Patients were treated with IVIG and outcome was observed. Outcome of 2 groups of patients one treated with IVIg and other not treated with IVIg (supportive line of treatment) were compared. RESULTS: Commonest age group affected was 13-40 yrs. The male:female ratio was 1.5:1. Antecedent infection in form of fever, cough [11 patients], loose motions [10 patients] were present in 21 out of 40 patients. Quadriparesis was present in 39 patients and paraparesis in 1 patient. Cranial nerve involvement was seen in 25 out of 40 patients. Facial nerve was involved in 12 [30%] patients and Glossopharyengeal, vagus nerves were involved in 12 [30%] patients. Areflexia was found in all 40 patients. In CSF examination, albuminocytologic dissociation was present in 17 out of 26 patients. NCV findings show conduction velocity slowing, delayed f latencies in 90% patients. Out of 40 patients, 13 [30%] required mechanical ventilation. Out of 40 patients, 14 were treated with IVIg, 4 patients treated with plasmapheresis and 22 patients received only supportive treatment. Out of 40 patients 30 [75%] patients recovered completely, 8 [20%] patients died and 2 [5%] patients developed severe neurologic deficit. CONCLUSION: GBS is more common in 13-40 yrs age group with male:female ratio of 1.5:1. Antecedent infection is seen in 55% patients. Commonest presentation was paresthesia in legs and ascending paralysis. One third [32.5%] patients developed respiratory paralysis and needed ventilatory support. Patients who received IVIg early in the course of disease had faster recovery as compared to patients who received only supportive line of treatment.