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Neuromuscular Disorders: A Comprehensive Review with Illustrative Cases Softcover Reprint of the Original 1st 2018 ed. [Mīkstie vāki]

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  • Formāts: Paperback / softback, 534 pages, height x width: 235x155 mm, weight: 1154 g, 99 Illustrations, color; 12 Illustrations, black and white; XXXII, 534 p. 111 illus., 99 illus. in color., 1 Paperback / softback
  • Izdošanas datums: 11-Feb-2019
  • Izdevniecība: Springer Verlag, Singapore
  • ISBN-10: 9811353778
  • ISBN-13: 9789811353772
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Neuromuscular Disorders: A Comprehensive Review with Illustrative Cases Softcover Reprint of the Original 1st 2018 ed.Palielināt
  • Formāts: Paperback / softback, 534 pages, height x width: 235x155 mm, weight: 1154 g, 99 Illustrations, color; 12 Illustrations, black and white; XXXII, 534 p. 111 illus., 99 illus. in color., 1 Paperback / softback
  • Izdošanas datums: 11-Feb-2019
  • Izdevniecība: Springer Verlag, Singapore
  • ISBN-10: 9811353778
  • ISBN-13: 9789811353772
Citas grāmatas par šo tēmu:
Permanent link: https://www.kriso.lv/db/9789811353772.html
This book provides a concise overview of the diagnosis and therapy of a wide variety of neuromuscular disorders, in tabulated form and with illustrative cases.

Treating neuromuscular disorders calls for a sound, step-by-step clinical approach based on differential diagnosis and laboratory investigations. Yet to date, there has been no single, compact book that offers all the relevant information related to the management of these disorders.





This book fills that gap, presenting the state of the art in the field and addressing practical problems together with their solutions. Each chapter covers disorder characteristics, clinical differentiating points, relevant investigations and their interpretation, available genetic testing, best management approaches and counselling. Illustrative cases provide valuable insights, while extensive tables and illustrations mean that information can be rapidly found.
1 Neuromuscular Disorders: A Clinical Approach 1(14)
1.1 History and Examination in Neuromuscular Disorders
1(8)
1.1.1 Family History
2(1)
1.1.2 Gait, Stance and Posture
2(1)
1.1.3 Weakness
3(4)
1.1.4 Cramps, Pains, Fatigue and Exertional Symptoms
7(1)
1.1.5 Hypertrophy and Atrophy
7(1)
1.1.6 Myotonia
8(1)
1.1.7 Sensory Features
8(1)
1.1.8 Tendon Reflexes
9(1)
1.2 Differentiating Anterior Horn Cell Diseases from Myopathies
9(1)
1.3 General Examination
10(1)
1.4 Concluding Remarks
11(1)
1.5 Organisation of This Book
11(4)
Part I: Asymmetric Motor Weakness
2 Motor Neuron Diseases (Amyotrophic Lateral Sclerosis)
15(18)
2.1 Introduction
15(1)
2.2 Epidemiology
15(1)
2.3 Clinical Features
16(6)
2.3.1 Symptoms of ALS
16(1)
2.3.2 Clinical Signs in ALS
17(1)
2.3.3 Regional Variants of ALS
18(1)
2.3.4 Clinical Spectrum of MND
18(3)
2.3.5 Diagnosis of ALS
21(1)
2.4 Pathophysiology
22(1)
2.5 Investigations
22(2)
2.5.1 Electrodiagnosis
22(1)
2.5.2 Pulmonary Function
23(1)
2.5.3 Secondary ALS
24(1)
2.6 Differential Diagnosis
24(2)
2.7 Management
26(2)
2.7.1 Molecules: Approved and in Development
26(1)
2.7.2 Gene Therapy Efforts
26(1)
2.7.3 Symptomatic Therapy
27(1)
2.7.4 Exercises in ALS and Adaptive Equipment
27(1)
2.7.5 Dealing with Patients and Families Having ALS
28(1)
2.7.6 Alternative Therapies and End of Life Issues
28(1)
2.8 Prognosis
28(1)
References
29(4)
3 Hirayama Disease and Other Focal Amyotrophies
33(14)
3.1 Hirayama Disease (HD)
33(8)
3.1.1 Introduction
33(1)
3.1.2 Epidemiology
33(1)
3.1.3 Clinical Features
34(1)
3.1.4 Pathophysiology
35(2)
3.1.5 Investigations
37(2)
3.1.6 Differential Diagnosis
39(1)
3.1.7 Management
40(1)
3.1.8 Prognosis
41(1)
3.2 Crural Amyotrophy (Wasted Leg Syndrome)
41(1)
3.3 Proximal Segmental Upper Limb Amyotrophy (Brachial Amyotrophic Diplegia)
42(1)
3.4 Case Study
42(2)
References
44(3)
4 Multifocal Motor Neuropathy
47(8)
4.1 Introduction
47(1)
4.2 Epidemiology
47(1)
4.3 Clinical Features
47(1)
4.4 Pathophysiology
48(1)
4.5 Investigations
49(1)
4.5.1 CSF Study
49(1)
4.5.2 Electrophysiology
49(1)
4.5.3 Imaging
50(1)
4.5.4 Antibodies
50(1)
4.6 Differential Diagnosis
50(1)
4.7 Management
50(1)
4.8 Prognosis
51(1)
4.9 Case Study
51(2)
References
53(2)
5 Acute Motor Axonal Neuropathy
55(6)
5.1 Introduction
55(1)
5.2 Epidemiology
55(1)
5.3 Clinical Features
56(1)
5.4 Pathophysiology
56(1)
5.5 Investigations
56(1)
5.5.1 Electrophysiology
56(1)
5.5.2 Antibodies
57(1)
5.6 Differential Diagnosis (Refer to GBS
Chapter)
57(1)
5.7 Management
57(1)
5.8 Prognosis
57(1)
References
58(3)
6 Facioscapulohumeral Dystrophy
61(10)
6.1 Introduction
61(1)
6.2 Epidemiology
61(1)
6.3 Clinical Features
61(2)
6.4 Pathophysiology
63(1)
6.5 Investigations
64(1)
6.6 Differential Diagnosis
64(1)
6.7 Management
65(1)
6.7.1 Physiotherapy
65(1)
6.7.2 Surgery
66(1)
6.7.3 Pharmacotherapy
66(1)
6.7.4 Management of Pain
66(1)
6.8 Prognosis
66(1)
6.9 Case Study
66(3)
References
69(2)
7 Progressive Muscular Atrophy
71(6)
7.1 Introduction
71(1)
7.2 Epidemiology
71(1)
7.3 Clinical Features
72(1)
7.4 Pathophysiology
72(1)
7.5 Investigations
72(1)
7.6 Differential Diagnosis
73(1)
7.7 Management
73(1)
7.8 Prognosis
73(2)
References
75(2)
8 Post-polio Syndrome
77(8)
8.1 Introduction
77(1)
8.2 Epidemiology
77(1)
8.3 Clinical Features
78(1)
8.4 Pathophysiology
78(1)
8.5 Investigations
79(1)
8.6 Differential Diagnosis
79(1)
8.7 Management
80(1)
8.7.1 Rehabilitation
80(1)
8.7.2 Pharmacological Measures
81(1)
8.8 Prognosis
81(1)
References
82(3)
Part II: Symmetric Proximal Weakness
9 Duchenne and Becker Muscular Dystrophies
85(14)
9.1 Introduction
85(1)
9.2 Epidemiology
85(1)
9.3 Clinical Features
86(2)
9.3.1 DMD
86(1)
9.3.2 Becker Muscular Dystrophy (BMD)
86(1)
9.3.3 Examination of the Mothers of Affected Boys
86(2)
9.3.4 Other Phenotypes Associated with Dystrophinopathies
88(1)
9.4 Pathophysiology
88(2)
9.5 Investigations
90(2)
9.5.1 Biochemistry
90(1)
9.5.2 Electrocardiography
90(1)
9.5.3 Genetic Evaluations
91(1)
9.5.4 Electrophysiology
91(1)
9.5.5 Imaging
91(1)
9.5.6 Muscle Biopsy
92(1)
9.6 Differential Diagnosis
92(1)
9.7 Management
93(2)
9.7.1 General Management and Surveillance
93(1)
9.7.2 Corticosteroid Therapy
93(1)
9.7.3 Other Drugs
94(1)
9.7.4 Gene Therapy
94(1)
9.8 Prognosis
95(1)
9.9 Case Study
95(2)
References
97(2)
10 Spinal Muscular Atrophy
99(14)
10.1 Introduction
99(1)
10.2 Epidemiology
99(1)
10.3 Clinical Features
100(2)
10.3.1 SMA
100(1)
10.3.2 X-linked Spinobulbar Muscular Atrophy (SBMA)
101(1)
10.4 Pathophysiology
102(1)
10.4.1 Genetics of SMA
102(1)
10.4.2 Genetics of SBMA
103(1)
10.4.3 Mutations in Genes Other than SMN 1
103(1)
10.5 Investigations
103(1)
10.5.1 SMA
103(1)
10.5.2 X-linked SBMA
103(1)
10.6 Differential Diagnosis
104(1)
10.6.1 Early-Onset SMA
104(1)
10.6.2 Adult-Onset SMA
105(1)
10.7 Management
105(2)
10.7.1 Antisense Oligonucleotides (ASO)
105(1)
10.7.2 Molecules that Increase SMN 2 Expression
106(1)
10.7.3 Neuroprotective Agents
106(1)
10.7.4 Gene Therapy
107(1)
10.7.5 Supportive Care
107(1)
10.7.6 Prenatal Screening
107(1)
10.8 Prognosis
107(1)
10.9 Case Study
107(3)
References
110(3)
11 Idiopathic Inflammatory Myopathies
113(18)
11.1 Introduction
113(1)
11.2 Epidemiology
114(1)
11.3 Clinical Features
114(2)
11.4 Pathophysiology
116(1)
11.5 Investigations
116(3)
11.5.1 Muscle Biopsy
116(2)
11.5.2 Antibody Studies
118(1)
11.6 Differential Diagnosis
119(1)
11.7 Management
120(4)
11.7.1 Corticosteroids
120(1)
11.7.2 Other Immunosuppressants
121(2)
11.7.3 Interstitial Lung Disease
123(1)
11.7.4 Inclusion Body Myositis
124(1)
11.7.5 Rehabilitation
124(1)
11.8 Prognosis
124(1)
11.9 Case Study
125(2)
References
127(4)
12 Limb-Girdle Muscular Dystrophies
131(18)
12.1 Introduction
131(2)
12.2 Epidemiology
133(1)
12.3 Commonly Reported LGMDs
134(9)
12.3.1 Clinical Features
134(1)
12.3.2 Pathophysiology
134(3)
12.3.3 Investigations
137(3)
12.3.4 Differential Diagnosis
140(2)
12.3.5 Management
142(1)
12.3.6 Prognosis
143(1)
12.4 Uncommonly Reported LGMDs
143(1)
12.5 Case Study 1
144(1)
12.6 Case Study 2
144(1)
References
145(4)
13 Congenital Myasthenic Syndromes
149(12)
13.1 Introduction
149(2)
13.2 Common Subtypes of CMS
151(6)
13.2.1 Epidemiology
151(1)
13.2.2 Clinical Features
151(2)
13.2.3 Pathophysiology
153(1)
13.2.4 Investigations
153(1)
13.2.5 Differential Diagnosis
154(1)
13.2.6 Management
155(1)
13.2.7 Prognosis
156(1)
13.3 Rare Forms of CMS
157(1)
13.4 Case Study
158(1)
References
159(2)
14 Congenital Muscular Dystrophies
161(12)
14.1 Introduction
161(1)
14.2 Epidemiology
161(2)
14.3 Clinical Features
163(2)
14.4 Pathophysiology
165(2)
14.5 Investigations
167(2)
14.6 Differential Diagnosis
169(1)
14.7 Management
170(1)
14.8 Prognosis
170(1)
References
171(2)
15 Congenital Myopathies
173(10)
15.1 Introduction
173(1)
15.2 Epidemiology
173(1)
15.3 Clinical Features
174(3)
15.4 Pathophysiology
177(1)
15.5 Investigations
177(1)
15.6 Differential Diagnosis
177(3)
15.7 Management
180(1)
15.8 Prognosis
180(1)
References
180(3)
16 Emery-Dreifuss Muscular Dystrophy
183(6)
16.1 Introduction
183(1)
16.2 Epidemiology
183(1)
16.3 Clinical Features
183(1)
16.4 Pathophysiology
184(1)
16.5 Diagnosis
184(1)
16.6 Differential Diagnosis
185(1)
16.7 Management
185(1)
16.8 Prognosis
186(1)
References
187(2)
17 Oculopharyngeal Muscular Dystrophy
189(8)
17.1 Introduction
189(1)
17.2 Epidemiology
189(1)
17.3 Clinical Features
190(1)
17.4 Pathophysiology
190(1)
17.5 Investigations
190(1)
17.6 Differential Diagnosis
191(1)
17.7 Management
192(1)
17.7.1 Surgical Correction of Ptosis
192(1)
17.7.2 Management of Dysphagia
192(1)
17.7.3 Newer Advances in Treatment
192(1)
17.8 Prognosis
192(1)
References
193(4)
Part III: Symmetric Distal Weakness
18 Myotonic Dystrophies
197(12)
18.1 Introduction
197(1)
18.2 Epidemiology
197(1)
18.3 Clinical Features
198(2)
18.3.1 DM 1
198(2)
18.3.2 DM 2
200(1)
18.4 Pathophysiology
200(1)
18.4.1 DM 1
200(1)
18.4.2 DM 2
200(1)
18.5 Investigations
201(1)
18.5.1 DM 1
201(1)
18.5.2 DM 2
202(1)
18.6 Differential Diagnosis
202(1)
18.7 Management
203(1)
18.7.1 Cardiac Arrhythmias
203(1)
18.7.2 Respiratory Compromise
203(1)
18.7.3 Myotonia
203(1)
18.7.4 Cataract
203(1)
18.7.5 Exercise
203(1)
18.7.6 Dehydroepiandrosterone
204(1)
18.7.7 Prenatal Genetic Testing
204(1)
18.8 Prognosis
204(1)
18.9 Case Study
204(2)
References
206(3)
19 Distal Myopathies
209(16)
19.1 Introduction
209(1)
19.2 Classic Distal Myopathies
210(7)
19.2.1 Epidemiology
210(1)
19.2.2 Clinical Features
211(2)
19.2.3 Pathophysiology
213(1)
19.2.4 Investigations
213(2)
19.2.5 Differential Diagnosis
215(1)
19.2.6 Management
215(2)
19.2.7 Prognosis
217(1)
19.3 Myofibrillar Myopathy (MFM)
217(1)
19.4 Other Uncommon Distal Myopathies
218(1)
19.5 Case 1
219(1)
19.6 Case 2
220(1)
References
221(4)
20 Distal Hereditary Motor Neuropathy
225(6)
20.1 Introduction
225(1)
20.2 Epidemiology
225(1)
20.3 Clinical Features
225(2)
20.4 Pathophysiology
227(1)
20.5 Investigations
227(1)
20.6 Differential Diagnosis
227(1)
20.7 Management
228(1)
20.8 Prognosis
228(1)
20.9 Case
228(2)
References
230(1)
21 Brown-Vialetto-Van Laere [ BVVI] Syndrome
231(8)
21.1 Introduction
231(1)
21.2 Epidemiology
231(1)
21.3 Clinical Features
231(1)
21.4 Pathophysiology
232(1)
21.5 Investigations
232(1)
21.6 Differential Diagnosis
232(1)
21.7 Management
232(1)
21.8 Prognosis
233(1)
21.9 Case Study
233(2)
References
235(4)
Part IV: Fluctuating Weakness
22 Myasthenia Gravis
239(22)
22.1 Introduction
239(1)
22.2 Epidemiology
240(1)
22.3 Clinical Features
240(5)
22.4 Pathophysiology
245(1)
22.4.1 Neuromuscular (NM) Junction in MG
245(1)
22.4.2 Role of Thymus in MG
246(1)
22.5 Investigations
246(2)
22.5.1 Ice-Pack Test
246(1)
22.5.2 Repetitive Nerve Stimulation (RNS)
247(1)
22.5.3 Tensilon or Neostigmine Test
247(1)
22.5.4 Antibody Tests
248(1)
22.5.5 SFEMG
248(1)
22.6 Differential Diagnosis
248(2)
22.7 Management
250(5)
22.8 Prognosis
255(1)
22.9 Case Study
255(2)
References
257(4)
23 Lambert-Eaton Myasthenic Syndrome
261(12)
23.1 Introduction
261(1)
23.2 Epidemiology
261(1)
23.3 Clinical Features
262(1)
23.4 Pathophysiology
263(1)
23.5 Investigations
264(2)
23.5.1 Electrophysiology
264(1)
23.5.2 Serological Markers
264(1)
23.5.3 Imaging
264(2)
23.6 Differential Diagnosis
266(1)
23.7 Management
266(1)
23.7.1 Symptomatic Treatment
266(1)
23.7.2 Immunosuppression
267(1)
23.7.3 Antitumour Therapy
267(1)
23.8 Prognosis
267(1)
23.9 Myasthenia Gravis Lambert-Eaton Overlap Syndrome (MLOS)
268(1)
23.10 Case Study
268(3)
References
271(2)
24 Periodic Paralysis
273(12)
24.1 Introduction
273(1)
24.2 Epidemiology
273(1)
24.3 Clinical Features
274(1)
24.4 Pathophysiology
275(1)
24.5 Investigations
275(1)
24.6 Differential Diagnosis
275(2)
24.7 Management
277(2)
24.8 Prognosis
279(1)
24.9 Case Study
279(2)
References
281(4)
Part V: Exercise Intolerance, Muscle Stiffness, Cramps and Contractures
25 Metabolic Myopathies
285(14)
25.1 Introduction
285(1)
25.2 Epidemiology
285(2)
25.3 Clinical Features
287(2)
25.3.1 Glycogen and Lipid Storage Disease
287(1)
25.3.2 Mitochondrial Diseases
288(1)
25.4 Pathophysiology
289(1)
25.5 Investigations
290(2)
25.6 Differential Diagnosis
292(1)
25.7 Management
292(1)
25.8 Prognosis
293(1)
25.9 Case Study
293(3)
References
296(3)
26 Peripheral Nerve Hyperexcitability Syndromes
299(12)
26.1 Introduction
299(1)
26.2 Epidemiology
299(1)
26.3 Clinical Features
300(2)
26.4 Pathophysiology
302(1)
26.5 Investigations
302(1)
26.6 Differential Diagnosis
303(1)
26.7 Management
304(1)
26.7.1 Symptomatic Treatment
304(1)
26.7.2 Immunotherapy
305(1)
26.7.3 Aetiological Treatment
305(1)
26.8 Prognosis
305(1)
26.9 Satoyoshi Syndrome
305(1)
26.10 Ocular Neuromyotonia
306(1)
26.11 Case Study
306(2)
References
308(3)
27 Nondystrophic Myotonic Disorders
311(14)
27.1 Introduction
311(1)
27.2 Myotonia Congenita (MC)
311(4)
27.2.1 Epidemiology
311(1)
27.2.2 Clinical Features
312(1)
27.2.3 Pathophysiology
313(1)
27.2.4 Investigations
313(1)
27.2.5 Differential Diagnosis
314(1)
27.2.6 Management
314(1)
27.2.7 Prognosis
315(1)
27.3 Paramyotonia Congenita (PMC)
315(1)
27.4 Hyperkalaemic Periodic Paralysis with Myotonia
316(1)
27.5 Potassium-Aggravated Myotonias (PAM)
317(1)
27.6 Myotonia Levior
317(1)
27.7 Brody's Disease
317(1)
27.8 Case Study
318(1)
27.9 Schwartz-Jampel Syndrome (SJS)
319(1)
References
320(5)
Part VI: Asymmetric Sensory Motor Weakness
28 Individual Mononeuropathies
325(12)
28.1 Introduction
325(1)
28.2 Management
325(7)
28.2.1 General Management
325(7)
28.2.2 Specific Therapies
332(1)
28.3 Case Study
332(3)
References
335(2)
29 Vasculitic Neuropathy
337(14)
29.1 Introduction
337(1)
29.2 Epidemiology
337(1)
29.3 Clinical Features
337(2)
29.4 Pathophysiology
339(1)
29.5 Investigations
340(2)
29.5.1 Significance of Blood Tests
340(1)
29.5.2 Electrodiagnosis
340(1)
29.5.3 CSF Study
341(1)
29.5.4 Histopathology
341(1)
29.6 Differential Diagnosis
342(1)
29.7 Management
343(3)
29.7.1 Remission Induction Therapy
343(3)
29.7.2 Remission Maintenance Therapy
346(1)
29.7.3 Newer Drugs
346(1)
29.8 Prognosis
346(1)
29.9 Case Study
347(2)
References
349(2)
30 Multifocal Acquired Demyelinating Sensorimotor Neuropathy
351(4)
30.1 Introduction
351(1)
30.2 Clinical Features
351(1)
30.3 Pathophysiology
351(1)
30.4 Investigations
352(1)
30.5 Differential Diagnosis
352(1)
30.6 Management
352(1)
30.7 Prognosis
353(1)
References
353(2)
31 Brachial Plexopathy
355(12)
31.1 Introduction
355(1)
31.2 Epidemiology
356(1)
31.3 Clinical Features
356(2)
31.3.1 Supraclavicular Plexopathy
356(1)
31.3.2 Neuralgic Amyotrophy
357(1)
31.3.3 Hereditary Neuralgic Amyotrophy (HNA)
357(1)
31.3.4 Rucksack Paralysis (Pack Palsy or Cadet Palsy)
357(1)
31.3.5 Neoplastic Plexopathy
358(1)
31.3.6 Radiation Plexopathy
358(1)
31.4 Pathophysiology and Anatomy
358(1)
31.5 Investigations
359(3)
31.5.1 Electrodiagnosis
359(2)
31.5.2 Radio Diagnosis
361(1)
31.6 Differential Diagnosis
362(1)
31.7 Management
362(1)
31.8 Prognosis
363(1)
31.9 Case Study
363(2)
References
365(2)
32 Lumbosacral Plexopathy
367(10)
32.1 Introduction
367(1)
32.2 Epidemiology
368(1)
32.3 Clinical Features
368(1)
32.4 Relevant Anatomy and Pathophysiology
369(2)
32.5 Investigations
371(1)
32.6 Differential Diagnosis
372(1)
32.7 Management
372(1)
32.8 Prognosis
373(1)
32.9 Case Study
373(3)
References
376(1)
33 Compressive Radiculopathies
377(6)
33.1 Introduction
377(1)
33.2 Clinical Features
377(2)
33.3 Investigations
379(1)
33.4 Differential Diagnosis
380(1)
33.5 Management
380(1)
33.6 Prognosis
380(1)
References
381(2)
34 Leprosy
383(12)
34.1 Introduction
383(1)
34.2 Epidemiology
383(1)
34.3 Clinical Features
384(3)
34.4 Pathophysiology
387(1)
34.5 Investigations
387(1)
34.6 Differential Diagnosis
388(1)
34.7 Management
389(1)
34.7.1 Medications
389(1)
34.7.2 Management of Disabilities
390(1)
34.8 Prognosis
390(1)
34.9 Case Study
390(3)
References
393(2)
35 Hereditary Liability to Pressure Palsies
395(8)
35.1 Introduction
395(1)
35.2 Epidemiology
395(1)
35.3 Clinical Features
395(1)
35.4 Pathophysiology
396(1)
35.5 Investigations
396(1)
35.5.1 Electrodiagnostic Tests
396(1)
35.5.2 Nerve Biopsy
396(1)
35.5.3 MRI
397(1)
35.5.4 Genetic Study
397(1)
35.6 Differential Diagnosis
397(1)
35.7 Management
398(1)
35.8 Prognosis
398(1)
35.9 Case Study
398(2)
References
400(3)
Part VII: Symmetric Sensory Motor Weakness
36 Guillain-Barre Syndrome
403(18)
36.1 Introduction
403(1)
36.2 Epidemiology
403(1)
36.3 Clinical Features
403(3)
36.3.1 Antecedent Events
403(1)
36.3.2 Classical GBS
404(1)
36.3.3 GBS Subtypes
405(1)
36.3.4 Red Flags for Diagnosis
406(1)
36.4 Pathophysiology
406(1)
36.5 Investigations
406(5)
36.5.1 Electrophysiological Study
406(2)
36.5.2 CSF Study
408(1)
36.5.3 Antibodies
408(3)
36.6 Differential Diagnosis
411(1)
36.7 Management
412(2)
36.7.1 General Measures
412(1)
36.7.2 Immunological Therapy
412(1)
36.7.3 Tracheostomy and Ventilation
413(1)
36.7.4 Autonomic Dysfunction
413(1)
36.8 Prognosis
414(1)
36.9 Case Study
415(2)
References
417(4)
37 Charcot-Marie-Tooth Disease
421(20)
37.1 Introduction
421(1)
37.2 Classification
421(4)
37.3 Epidemiology
425(1)
37.4 Clinical Features
425(2)
37.5 Investigations
427(3)
37.5.1 Electrodiagnostic Studies (EDx)
427(1)
37.5.2 Cerebrospinal Fluid (CSF)
427(1)
37.5.3 Neuromuscular Ultrasound
427(1)
37.5.4 Neuromuscular MRI
428(1)
37.5.5 Nerve Biopsy
428(1)
37.5.6 Genetic Testing
429(1)
37.6 Differential Diagnosis
430(1)
37.7 Management
430(2)
37.7.1 Physical Therapy and Rehabilitation
430(1)
37.7.2 Exercise
431(1)
37.7.3 Role of Surgery
431(1)
37.7.4 Putative Therapies
431(1)
37.8 Prognosis
432(1)
37.9 Case Study 1
432(2)
37.10 Case Study 2
434(2)
References
436(5)
38 Chronic Inflammatory Demyelinating Polyradiculoneuropathy
441(12)
38.1 Introduction
441(1)
38.2 Epidemiology
441(1)
38.3 Clinical Features
442(3)
38.3.1 Sensory CIDP
443(1)
38.3.2 Motor CIDP
443(1)
38.3.3 Focal CIDP
443(1)
38.3.4 MADSAM
444(1)
38.3.5 DADS
444(1)
38.3.6 CISP, CIMP and CISMP
444(1)
38.3.7 CIDP in Association with Diabetes and Myeloma
445(1)
38.4 Pathophysiology
445(1)
38.5 Investigations
445(1)
38.5.1 Diagnostic Criteria
445(1)
38.5.2 Electrophysiological Study
446(1)
38.5.3 Ultrasound
446(1)
38.5.4 CSF Study
446(1)
38.5.5 MRI Neurography
446(1)
38.6 Differential Diagnosis
446(1)
38.7 Management
447(2)
38.8 Prognosis
449(1)
38.9 Case Study
449(1)
References
450(3)
39 X-Linked Charcot-Marie-Tooth Disease
453(8)
39.1 Introduction
453(1)
39.2 Epidemiology
454(1)
39.3 Clinical Features
454(1)
39.4 Pathophysiology
454(1)
39.5 Investigations
455(1)
39.5.1 Electrophysiological Study
455(1)
39.5.2 Nerve Biopsy
456(1)
39.5.3 MRI Brain
456(1)
39.6 Differential Diagnosis
456(1)
39.7 Management
456(1)
39.8 Prognosis
456(1)
39.9 Case Study
457(1)
References
458(3)
40 Other Inherited Neuropathies I
461(12)
40.1 Introduction
461(1)
40.2 Epidemiology
461(3)
40.3 Neuropathies Secondary to Inherited Metabolic Disorders
464(1)
40.3.1 Familial Amyloid Polyneuropathy (FAP)
464(1)
40.3.2 Other Inherited Metabolic Disorders
464(1)
40.4 Neuropathies Associated with Leukodystrophies
464(2)
40.5 Neuropathies Associated with Hereditary Ataxias
466(1)
40.6 Investigations
467(3)
40.7 Differential Diagnosis
470(1)
40.8 Management
470(1)
40.8.1 Supportive Management
470(1)
40.8.2 Surgical Treatment
470(1)
40.8.3 Specific Treatment
470(1)
40.9 Prognosis
470(2)
References
472(1)
41 Other Inherited Neuropathies II
473(10)
41.1 Mitochondrial Diseases with Peripheral Neuropathy
473(1)
41.1.1 Pathogenesis
473(1)
41.2 Hereditary Sensory Autonomic Neuropathy (HSAN)
473(1)
41.3 Uncommon Inherited Neuropathies
473(4)
41.4 Investigations
477(1)
41.5 Management
477(1)
41.6 Prognosis
478(1)
41.7 Case Study
478(3)
References
481(2)
42 Neuropathies Secondary to Systemic Diseases
483(10)
42.1 Introduction
483(1)
42.2 Epidemiology
483(2)
42.3 Clinical Features and Investigations
485(3)
42.3.1 Neuropathies Secondary to Metabolic Disorders
485(1)
42.3.2 Toxic Neuropathies
485(2)
42.3.3 Neuropathies Secondary to Infections
487(1)
42.3.4 Neuropathies Secondary to Nutritional Deficiency
487(1)
42.4 Management and Prognosis
488(1)
42.5 Case Study
489(2)
References
491(2)
43 Porphyrias
493(12)
43.1 Introduction
493(1)
43.2 Epidemiology
494(1)
43.3 Clinical Features
494(1)
43.4 Pathophysiology
495(1)
43.5 Investigations
496(1)
43.6 Differential Diagnosis
497(2)
43.7 Management
499(1)
43.8 Prognosis
500(1)
References
501(4)
Part VIII: Predominant Sensory Syndromes
44 Sensory Neuronopathies
505(10)
44.1 Introduction
505(1)
44.2 Epidemiology
506(1)
44.3 Clinical Features
506(1)
44.4 Pathophysiology
507(1)
44.5 Investigations
508(1)
44.5.1 Electrophysiological Studies
508(1)
44.5.2 Laboratory Parameters
508(1)
44.5.3 CSF Study
508(1)
44.5.4 Imaging Studies
508(1)
44.5.5 Tissue Biopsy
509(1)
44.6 Differential Diagnosis
509(1)
44.7 Management
510(1)
44.8 Prognosis
511(1)
44.9 Case Study
511(1)
References
512(3)
45 Distal Acquired Demyelinating Symmetric Neuropathy
515(4)
45.1 Introduction
515(1)
45.2 Clinical Features
515(1)
45.2.1 DADS-M
515(1)
45.2.2 DADS-I
516(1)
45.2.3 Associations
516(1)
45.3 Investigations
516(1)
45.3.1 Antibodies
516(1)
45.3.2 Electrophysiological Study
516(1)
45.4 Differential Diagnosis
516(1)
45.5 Management
517(1)
45.6 Prognosis
517(1)
References
518(1)
46 Paraproteinaemic Neuropathy
519(12)
46.1 Introduction
519(1)
46.2 Epidemiology
520(1)
46.3 Clinical Features
520(2)
46.4 Pathophysiology
522(1)
46.5 Investigations
522(2)
46.6 Differential Diagnosis
524(1)
46.7 Management
525(2)
46.7.1 Immunosuppressive Agents
525(1)
46.7.2 Pain and Dysautonomia
525(1)
46.7.3 Systemic Complications
526(1)
46.8 Prognosis
527(1)
46.9 Case Study
527(3)
References
530(1)
47 Miller Fisher Syndrome
531
47.1 Introduction
531(1)
47.2 Clinical Features
531(1)
47.2.1 Classical MFS
531(1)
47.2.2 Incomplete Form of MFS
531(1)
47.2.3 Bickerstaff Brainstem Encephalitis (BBE)
532(1)
47.2.4 MFS and GBS Overlap
532(1)
47.3 Pathophysiology
532(1)
47.4 Investigations
533(1)
47.4.1 Anti-GQ1b Antibody
533(1)
47.4.2 Magnetic Resonance Imaging (MRI) and Electrophysiological Studies
533(1)
47.5 Differential Diagnosis
533(1)
47.6 Management
533(1)
47.7 Prognosis
533(1)
References
534
Prof. Dr. Satish V. Khadilkar is one of the first few Indian neurologists to have pursued the subspecialty of neuromuscular disorders, a field in which he has made a mark in India and beyond. He was the first to describe a new clinical sign, the hip abduction sign, which has since been included in international books. He contributed the first Indian accounts of sarcoglycanopathies and dysferlinopathies and was instrumental in finding the founder gene in calpainopahties in Agarwal community and the founder mutation in GNE myopathy in inhabitants of Rajasthan. He presently represents India at the Asian Oceanian Myology Center.

He has twice been honored with the best teacher award by Grant Medical College. His lecture series for MD students has been very popular and has been converted into digital teaching material, which is distributed free of charge. He has produced over 150 publications, and has written four and edited one neurology book. He has also been active in social work, serving as the trustee and secretary of the muscular dystrophy society and as the national Vice President of the multiple sclerosis society of India. He has received prestigious fellowships from the Indian Academy of Neurology, Indian College of Physicians and National Academy of Medical Sciences.

Dr Rakhil Yadav received his DM in Neurology from the prestigious Grant Medical College and Si J.J. Group of Hospital, going on to work as a lecturer in the same department. Subsequently, he trained in neuromuscular disorders at Paris. He is presently working as a consultant neurologist in Ahmedabad. He has contributed to and participated in various book chapters and peer-reviewed indexed publications, and has served as an ICMR project guide.





Dr Bhagyadhan Patel is currently a lecturer at the Neurology department of T.N.M.C and B.Y.L Nair Charitable Hospitals, Mumbai. Dr Patel has received a number of distinctions, including the National Young Achiever Award (2015), the Indian Academy of neurology (IAN) Summer school quiz (2015 and 2016) and the IAN-Bar-B-Quiz (2015). He has presented many papers, posters and cases at various national level conferences, and has published in international journals. Further, he is actively involved in teaching programs for postgraduate students in neurology and internal medicine.