Dr. Nadeem Akhtar - NEUROLOGY EQUATIONS MADE SIMPLE: differential diagnosis and neuroemergencies
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Neurology Equations Made Simple Differential Diagnosis and Neuroemergencies Dr Nadeem Akhtar (MRCP UK, MRCP Neurology UK) AuthorHouse UK Ltd. 1663 Liberty Drive Bloomington, IN 47403 USA www.authorhouse.co.uk Phone: 0800.197.4150 2018 Dr Nadeem Akhtar. All rights reserved. No part of this book may be reproduced, stored in a retrieval system, or transmitted by any means without the written permission of the author. Published by AuthorHouse 07/10/2018 ISBN: 978-1-5246-6625-5 (sc) ISBN: 978-1-5246-6624-8 (e) Any people depicted in stock imagery provided by Thinkstock are models,
and such images are being used for illustrative purposes only. Certain stock imagery Thinkstock.
Because of the dynamic nature of the Internet, any web addresses or links contained in this book may have changed since publication and may no longer be valid. The views expressed in this work are solely those of the author and do not necessarily reflect the views of the publisher, and the publisher hereby disclaims any responsibility for them. 
CONTENTS Neurology Equations Made Si mple To my parents Sehba Akhtar, Saeeda Akhtar, my sister Rubina Akhtar and my family for their endless support which has left fingerprints on my h eart.
| Post Infectious Cerebellitis | Acute or subacute onset, following an infection usually viral; (can be GI or, pulmonary). Ataxia can develop over several hours, days or, 1-4 weeks after infection. burgdorferi) Mycoplasma Legionella can be involved * Neurological Equation Sudden onset of unsteadiness (truncal ataxia + Hx of diarrhoea/ chest infection + intact DTR (If lost think of MFS) = Post infectious Cerebellitis |
| Vascular Cerebellar Haemorrhage/ Infarction | Long-standing hypertension -most common cause Anticoagulant use Haemorrhage into a tumour * Neurological Equation Sudden abrupt onset headache + Nausea and vomiting + Inability to walk (truncal ataxia) + Loss or alteration of consciousness = Cerebellar haemorrhage, (infarction will generally be without headache) |
| Miller Fischer Syndrome | The Miller Fischer syndrome which is an acute self-limiting condition, is characterised by truncal ataxia, areflexia and ophthalmopleigia The anti-GQ1b IgG antibody titer is most commonly elevated in MFS Gullian Barre syndrome and Bickerstaff encephalitis. burgdorferi) Mycoplasma Legionella can be involved * Neurological Equation Sudden onset of unsteadiness (truncal ataxia + Hx of diarrhoea/ chest infection + intact DTR (If lost think of MFS) = Post infectious Cerebellitis |
| Vascular Cerebellar Haemorrhage/ Infarction | Long-standing hypertension -most common cause Anticoagulant use Haemorrhage into a tumour * Neurological Equation Sudden abrupt onset headache + Nausea and vomiting + Inability to walk (truncal ataxia) + Loss or alteration of consciousness = Cerebellar haemorrhage, (infarction will generally be without headache) |
| Miller Fischer Syndrome | The Miller Fischer syndrome which is an acute self-limiting condition, is characterised by truncal ataxia, areflexia and ophthalmopleigia The anti-GQ1b IgG antibody titer is most commonly elevated in MFS Gullian Barre syndrome and Bickerstaff encephalitis. The ataxia is similar to cerebellar disease, but it is not yet known whether it arises centrally or peripherally. * Neurological Equation Sudden onset of unsteadiness (truncal ataxia + Diplopia (complex ophthalmoplegia) + loss of DTR + anti-GQ1b IgG antibody = MFS) |
| Multiple Sclerosis | Multiple sclerosis can produce disorders of equilibrium of cerebellar, vestibular, or sensory origin. More common in women Gait and limb ataxia (gait involvement is more common than limb. |
| Paraneoplastic Subacute cerebellar degeneration | Severe dysarthria Common tumours which cause Paraneoplastic sub-acute cerebellar degeneration are lung carcinoma (small-cell) ovarian uterine lymphomas * Neurological Equation Known case of cancer (can precede the identification of the cancer) + Subacute onset of Ataxia/dysarthria + Anti Hu antibodies/Anti Yo = Paraneoplastic Subacute cerebellar degeneration |
| Pearls | * Other known Paraneoplastic Syndromes Lambert-Eaton myasthenia syndrome (LEMS) Opsoclonus/myoclonus Sensory neuronopathy |
| Creutzfeldt-Jakob disease | In CJD, cerebellar signs are present in about 60% and some patients present with isolated ataxia in about 10% of cases. |
| Other causes | Infectious Cerebellar abscess. 25% can have a typical age of presentation which could be after 25 years of age. 25% can have a typical age of presentation which could be after 25 years of age. Associated features include weakness in pyramidal distribution or extensors planters. Peripheral neuropathy (axonal) Optic atrophy (25%) Skeletal deformities: pes cavus (50%) and scoliosis (75 %) Hypertrophic Cardiomyopathy 25% Diabetes or glucose intolerance (20%) * Neurological Equation Patient under 25 years of age + Cerebellar signs + Pyramidal signs (up going plantars) + peripheral neuropathy (absent ankle jerks) + Optic atrophy = Friedreich ataxia |
| Pearls | * D/D of extensor plantar with absent ankle jerks includes FA, MND Tabes Dorsalis |
| SCA | There are more than 20 types of SCA These types are given numbers (1 to 22, excluding the number 9). Spinocerebellar ataxias (SCAs) is characterised by progressive cerebellar ataxia in association with Ophthalmoplegia Pyramidal signs Pigmentry retinopathy Peripheral neuropathy dementia |
| Ataxia with Isolated vitamin E deficiency | Examples of treatable ataxias include those due to deficiencies of vitamin E or coenzyme Q10 and Episodic Ataxia Type 2 (EA2) Clinical phenotype similar to Friedreich ataxia Onset is between 2-52 years and generally < than 20 yrs. Slowly progressive. Skin signs can be xanthelasma and tendon xanthomas. * Neurological Equation Ataxia + hint of malabsorption (Hx of diarrhoea/anaemia/low calcium) + skin xanthomas = Ataxia with isolated vitamin E deficiency * Causes of deficiency include Cystic fibrosis, Abetalipoproteinemia Short bowel syndrome Malabsorption syndrome |
| Alcohol | Degenerative changes in the cerebellum are largely restricted to the superior vermis probably as a result of nutritional deficiency. Affected patients typically have a history of daily or binge drinking with associated dietary inadequacy * Neurological Equation Hx of Alcohol overuse + progressive gait disturbance (predominant involvement of vermis) + minimal limb ataxia (relative sparing of cerebellar hemispheres) + MRI showing topical vermial atrophy = Alcohol-related cerebellar degeneration |
| Ataxia telangiectasia | Slowly progressive ataxia and dysarthria associated Choreoathetosis Look for, cutaneous, conjunctiva and bulbar telangiectasia Bulbar conjunctivae are typically affected first, followed by sun-exposed areas of the skin, including the ears, nose, face, and antecubital and popliteal fossae Increased susceptibility to infections due (selectiveIg A and IgG deficiency) * Neurological Equation Ataxia + recurrent infections (respiratory due to immuno deficiency) + Telangiectasia (one has to look for in conjucntival and bulbar areas) + progressive choreoathetosis + Ataxia (uncommon) = Telangiectasia Ataxia |
| Adrenloeukodystrophy | Adrnleukodystrophy is an X-liked hereditary disorder caused by genetic problem in Xq28 affecting the peroxisomal membrane. The genetic defect would result in peroxisomal dysfunction resulting in very long chain fatty acids (VLCFA) The nervous system, adrenal cortex, and testis are involved Acanthocytes in peripheral blood smears Decreased serum cholesterol level Increased high density lipoprotein cholesterol levels * Neurological Equation Boy / man (x linked disorder) + Ataxia + cognitive compromise + progressive loss of vision + hypotension and hyper pigmentation (due to adrenal insufficiency)+ increased VLCFA = Adrenoleukodystrophy |
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