Fifty Neurologic Cases From Mayo Clinic
Fifty Neurologic Cases From Mayo Clinic
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Abstract
This informative and entertaining compilation of 50 short neurological cases demonstrates important principles in clinical localization and differential diagnosis. Each case presents the key elements without revealing the diagnosis at first. A vivid clinical scenario provides enough information for the student to localize the site of the lesion and for the experienced neurological physician to reach a differential diagnosis. Each case description is followed by one or two illustrations, the diagnosis, and then a commentary by a Mayo Clinic consultant. The commentary highlights the issues in the differential diagnosis and provides an update on what is currently known about the specific diagnostic entity. The book will be of interest to physicians and surgeons caring for neurological patients at each stage of their career. It will be of particular help to medical students and to residents and fellows in neurology and neurosurgery. Internists, pediatricians, geriatricians, and psychiatrists will also find it useful.
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Front Matter
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Diagnosis Case 1
Asymmetric cortical degeneration syndrome: semantic dementia
Dr. Bradley F Boeve
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Diagnosis Case 2
GM2 gangliosidosis (adult-onset Tay-Sachs disease)
Dr. James H Bower
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Diagnosis Case 3
Occipital condyle syndrome due to metastatic cancer
Dr. Paul W Brazis
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Diagnosis Case 4
Intractable partial epilepsy of left frontal lobe origin related to focal cortical dysplasia
Dr. Gregory D Cascino
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Diagnosis Case 5
Fascioscapulohumeral (FSH) muscular dystrophy
Dr. Brian A Crum
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Diagnosis Case 6
Morvan’s syndrome as a paraneoplastic phenomenon (thymoma)
Dr. Keith A Josephs
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Diagnosis Case 7
Central pain disorder: thoracic cavernous angioma
Dr. Paola Sandroni
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Diagnosis Case 8
Spinal dural arteriovenous fistula (SDAVF)
Drs. Guillermo A Suarez andWilliam E Krauss
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Diagnosis Case 9
Paraneoplastic autonomic neuropathy and limbic encephalitis
Dr. Steven Vernino
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Diagnosis Case 10
Central nervous system Whipple’s disease
Dr. David W Kimmel
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Diagnosis Case 11
Adult Alexander’s disease
Dr. John H Noseworthy
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Diagnosis Case 12
Adult polyglucosan body disease (APBD)
Dr. Christopher J Boes
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Diagnosis Case 13
Orthostatic tremor (“shaky legs syndrome”)
Dr. Ryan J Uitti
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Diagnosis Case 14
Chronic paroxysmal hemicrania (CPH)
Dr. David W Dodick
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Diagnosis Case 15
Angelman’s syndrome
Dr. Kenneth J Mack
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Diagnosis Case 16
Opercular syndrome
Dr. Frank A Rubino
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Diagnosis Case 17
Amyotrophic lateral sclerosis (ALS)-dementia complex
Dr. Richard J Caselli
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Diagnosis Case 18
Steroid-responsive Hashimoto’s encephalopathy presenting as status epilepticus
Drs. Joseph I Sirven andJoseph F Drazkowski
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Diagnosis Case 19
Neuromyelitis optica (Devic’s disease)
Dr. Moses Rodriguez
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Diagnosis Case 20
Intracranial dural arteriovenous fistula (DAVF) presenting after cerebral venous sinus thrombosis
Dr. Robert D Brown
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Diagnosis Case 21
REM sleep behavior disorder (RBD) associated with dementia with Lewy bodies
Dr. Michael H Silber
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Diagnosis Case 22
Facial pain secondary to an intrathoracic mass lesion
Drs. David J Capobianco andWilliam P Cheshire
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Diagnosis Case 23
Anterior communicating artery aneurysm paraparesis syndrome
Dr. Eelco F M Wijdicks
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Diagnosis Case 24
Hereditary neuropathy with a liability to pressure palsy (HNPP or tomaculous neuropathy)
Dr. Jasper R Daube
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Diagnosis Case 25
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
Dr. Kelly D Flemming
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Diagnosis Case 26
α-Lipoprotein deficiency (Tangier disease)
Dr. Peter J Dyck
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Diagnosis Case 27
Dural arteriovenous fistula (DAVF) of the left medulla
Dr. Brian A Crum
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Diagnosis Case 28
AL amyloidosis (λ type) with autonomic neuropathy
Dr. Christopher J Klein
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Diagnosis Case 29
Chronic infantile neurologic cutaneous and articular/neonatal-onset multisystem inflammatory disease (CINCA/NOMID)
Dr. Nancy L Kuntz
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Diagnosis Case 30
Retinocochleocerebral vasculopathy (Susac’s syndrome)
Dr. George W Petty
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Diagnosis Case 31
Chronic immune sensory polyradiculopathy
Drs. Michael Sinnreich andP James B Dyck
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Diagnosis Case 32
Cardioskeletal myopathy associated with lysosome-associated membrane protein-2 (LAMP-2) deficiency
Dr. Duygu Selcen
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Diagnosis Case 33
Myeloneuropathy due to copper deficiency (caused by chronic excessive oral intake of zinc)
Dr. Neeraj Kumar
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Diagnosis Case 34
Squamous carcinoma involving the perineural sheath of the facial nerve
Dr. Raymond G Auger
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Diagnosis Case 35
Dopamine β-hydroxylase (DBH) deficiency
Dr. William P Cheshire
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Diagnosis Case 36
Polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome with Castleman’s disease
Dr. Paul W Brazis
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Diagnosis Case 37
Diffuse B-cell lymphoma (primary central nervous system lymphoma[PCNSL])
Dr. Irene Meissner
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Diagnosis Case 38
Intermittent angle-closure glaucoma
Dr. Jerry W Swanson
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Diagnosis Case 39
Hereditary generalized dystonia and tremor secondary to DYT1 gene mutation
Drs. Charles H Adler andManfred D Muenter
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Diagnosis Case 40
Familial hemiplegic migraine (FHM) in a patient with Erdheim-Chester disease (ECD)
Commentary by Dr. David. F Black
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Diagnosis Case 41
Presynaptic congenital myasthenic syndrome caused by choline acetyltransferase (ChAT) deficiency
Dr. Andrew G Engel
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Diagnosis Case 42
Leber’s hereditary optic neuropathy
Dr. Deborah L Renaud
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Diagnosis Case 43
Adult acid maltase deficiency
Dr. E Peter Bosch
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Diagnosis Case 44
Posterior sacral meningocele
Dr. Bahram Mokri
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Diagnosis Case 45
Lambert-Eaton myasthenic syndrome (LEMS) due to small cell lung carcinoma (SCLC)
Dr. C Michel Harper
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Diagnosis Case 46
Paraneoplastic optic neuritis, polyradiculopathy, and ataxia for which collapsin response mediator protein-5 (CRMP-5) autoantibody serves as a serologic marker
Dr. Shelley A Cross
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Diagnosis Case 47
Sarcoidosis with lung, bone, and nervous system involvement
Dr. Claudia F Lucchinetti
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Diagnosis Case 48
Schwannoma originating from cranial nerve X or XII, suggesting a high-grade glioma or metastasis
Dr. Joon H Uhm
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Diagnosis Case 49
Segmental paresis secondary to presumed herpes zoster infection
Dr. Allen J Aksamit
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Diagnosis Case 50
Mild cognitive impairment (MCI), amnestic type
Dr. Ronald C Petersen
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