Skip to main content
SpringerLink
Log in
Book cover

Neurology at the Bedside pp 137–269Cite as

Differential Diagnosis: “What Is the Lesion?”

  • Chapter
  • First Online:

  • 3189 Accesses

Abstract

Following the history and bedside examination, the anatomic localization of the lesion should have been identified, and the neurologist can proceed with a differential diagnosis. This is usually straightforward by taking into account information related to epidemiology, comorbidities, hereditary predispositions, and occupation, as well as the time course of the disorder. For instance, hyperacute onset of deficits is characteristic for cerebrovascular disease, subacute onset for infectious and inflammatory disorders, and prolonged onset over weeks or months for malignancy. A chronic progressive course over years is indicative of neurodegenerative diseases. However, occasionally, a slowly progressing illness comes rather suddenly to the attention of the patient because the declining level of function has been exposed by an unusual activity or event. If important data are unavailable, early fixation on a single differential diagnosis should be avoided due to the risk of overlooking important details. This chapter provides a simple yet comprehensive differential diagnosis of most of the disorders encountered in neurological practice. Further, it makes extensive use of mind maps in order to enable the reader to approach and visualize the differential diagnosis of each neurological field in a structured and intuitive manner.

This is a preview of subscription content, log in via an institution.

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   54.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

Notes

  1. 1.

    Thus, obvious cases of poisoning and trauma were not included in this series.

  2. 2.

    Measurement of the plasma levels of the brain biomarker S100B on admission (≤6 h after the injury) in patients with minor head injury is a promising screening tool that may support the clinician’s decision not to perform CT imaging in certain cases of low-risk head injury (Zongo et al. 2012). It has been suggested that integration of the brain biomarker S100B within existing management routines may reduce the need for CT scans by 30 % (Undén and Romner 2009).

  3. 3.

    Depression is defined as a cluster of symptoms that may include depressed mood; anhedonia; changes in eating, appetite, or weight; hypersomnia or insomnia; psychomotor agitation or retardation; impaired self-esteem and self-confidence; ideas of guilt and unworthiness; bleak and pessimistic view of the future; tiredness; impaired concentration and memory; and in some cases thoughts of death. For a diagnosis of depression at least two of the core symptoms (depressed mood, anhedonia, and reduced energy) must be present, plus two of the other symptoms mentioned above (ICD 10, WHO 2010).

  4. 4.

    Delirium is defined as an acute or subacute condition with reversible and fluctuating decreased consciousness due to organic brain disease or metabolic-toxic impairment. Common causes are polypharmacy, electrolyte derangements, hypo- and hyperthyroidism, kidney and liver failure, and hypo- and hyperthermia, as well as urinary tract and other infections. Patients are disoriented in time and space, incoherent, and perseverative, with agitation or apathy, and they may have visual hallucinations and asterixis.

  5. 5.

    Beware of the fact that coexistent epilepsy is found in up to 20 % of those with dissociative seizures. Also, many bizarre motor features such as pelvic thrusting and bicycling, while commonly seen in PNES, may occur in frontal lobe seizures. Further, it must be borne in mind that panic and other emotions may be part of a temporal lobe seizure.

  6. 6.

    In elderly patients with sudden falls and presumed drop attacks, the absence of a history of loss of consciousness is unreliable. More often than not, syncope is the correct diagnosis.

  7. 7.

    Most cases of cryptogenic ischemic stroke are probably due to undetected paroxysmal atrial fibrillation, hence the need for prolonged cardiac monitoring using telemetry/Holter ECG monitor.

  8. 8.

    The differential diagnosis of non-aneurysmal subarachnoidal bleeding includes “angiogram negative subarachnoidal bleeding” (e.g., perimesencephalic bleeding from the perimesencephalic venous plexus, which has excellent prognosis), cerebral arteriovenous malformations (AVM), vasculitis, carotid or vertebral artery dissection, cerebral venous thrombosis, spinal AVM, abuse of sympathomimetics and stimulants such as cocaine and amphetamine, sickle cell disease, pituitary apoplexy, tumors, and coagulation disorders. Atraumatic, non-aneurysmal subarachnoidal bleeding localized on the brain convexities is typically due to cerebral amyloid angiopathy (CAA) in the elderly (>60 years), whereas in younger patients it is often due to reversible cerebral vasoconstriction syndrome (RCVS) (Kumar et al. 2010).

  9. 9.

    Susac’s syndrome involves microangiopathy of the brain, retina, and cochlea; it usually affects young women (female/male ratio 3:1) and leads to encephalopathy, retinopathy, and hearing loss.

  10. 10.

    However, limb shaking because of a lacunar TIA or hemodynamic carotid artery stenosis may be misdiagnosed as an epileptic seizure.

  11. 11.

    If the patient has a brainstem or spinal cord syndrome, the symptomatic lesions are excluded from the criteria and do not contribute to the lesion count.

  12. 12.

    Brain abscess is most commonly due to hematogenous spread. Less frequently, it occurs as a complication of sinusitis, otitis, mastoiditis, or penetrating trauma. Brain abscess may be caused by a single agent but can also be polymicrobial. Headache, fever, and focal neurologic signs are the classic triad of a brain abscess. However, most patients do not present with the complete triad, and the presentation is typically that of an intracerebral mass lesion with subacute onset. Intraventricular rupture of the abscess leads to rapid deterioration.

  13. 13.

    Moritz Romberg (1795–1873, German neurologist) invented the eponymous test for the screening of sensory ataxia in large numbers of recruits to detect those unsuited for the army because of tabes dorsalis.

  14. 14.

    In the Third Reich, German pathologists Julius Hallervorden (1882–1965) and Hugo Spatz (1888–1969) collaborated with the Nazi regime and collected the brains of several hundred NS-euthanasia victims. Based on the material from “euthanized” patients, Hallervorden published 12 papers after World War II (several of which are listed in MEDLINE) (Kondziella 2009).

  15. 15.

    Anticipation is a phenomenon characterized by increasingly severe symptoms and earlier outbreak in the subsequent generation due to accumulation of CAG repeats. In Huntington’s disease, this is mainly the case if the disease is transmitted by the father.

  16. 16.

    With acute rupture of an AComA aneurysm, the paraparesis will be flaccid; only later does spasticity develop.

  17. 17.

    Important variant forms of GBS include pharyngeal-cervical-brachial paresis, oculopharyngeal weakness, pure sensory GBS, pure autonomic failure, ataxic GBS, and the Miller-Fisher syndrome.

  18. 18.

    To perform a tensilon test, start with 1 mL normal saline IV (placebo), and then use edrophonium IV (10 mg/mL; give 0.2 mL, if no adverse reactions give another 0.8 mL). Atropine 0.5 mg IV (antidote) must be available at the bedside. The tensilon test is used to:

    • Verify the diagnosis of MG

    • Evaluate treatment (increase of pyridostigmine dosage possible?), performed 2 h after last pyridostigmine dose

    • Differentiate between myasthenic and cholinergic crisis (rarely necessary)

  19. 19.

    To perform the ice-on-eyes test, fill a rubber glove with crushed ice and place it over the patient’s eyes for 2 min or less. If this is too cold, put a paper towel between the eyes and the glove. Coldness increases synaptic transmission and ameliorates the ptosis. This test is believed to have even higher specificity and sensitivity than the tensilon test and is not associated with cardiovascular complications but can only be performed in patients with ptosis.

References and Suggested Reading

  • Albert MS, DeKosky ST, Dickson D, Dubois B, Feldman HH, Fox NC, Gamst A, Holtzman DM, Jagust DW, Petersen RC, Snyder PJ, Carrillo MC, Thies B, Phelpset CH. The diagnosis of mild cognitive impairment due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease. Alzheimers Dement. 2011;7:270–9.

    Article  PubMed Central  PubMed  Google Scholar 

  • American Psychiatric Association. Diagnostic and statistical manual of mental disorders. 5th ed. Arlington: American Psychiatric Association; 2013.

    Google Scholar 

  • Barkhof F, Fox NC, Bastos-Leite AJ, Scheltens P. Neuroimaging in dementia. Berlin/Heidelberg: Springer; 2011.

    Book  Google Scholar 

  • Callaghan B, McCammon R, Kerber K, et al. Tests and expenditures in the initial evaluation of peripheral neuropathy. Arch Intern Med. 2012;172:127.

    Article  PubMed Central  PubMed  Google Scholar 

  • Cramfield CS, Cramfield PR. Juvenile myoclonic epilepsy 25 years after seizure onset: a population-based study. Neurology. 2009;73:1041–5.

    Article  Google Scholar 

  • Crutch SJ, Schott JM, Rabinovici GD, Boeve BF, Cappa SF, Dickerson BC, Dubois B, Graff-Radford NR, Krolak-Salmon P, Lehmann M, Mendez MF, Pijnenburg Y, Ryan NS, Scheltens P, Shakespeare T, Tang-Wai DF, van der Flier WM, Bain L, Carrillo MC, Fox NC. Shining a light on posterior cortical atrophy. Alzheimers Dement. 2013;9(4):463–5.

    Article  PubMed  Google Scholar 

  • Dubois B, Feldman HH, Jacova C, Dekosky ST, Barberger-Gateau P, Cummings J, Delacourte A, Galasko D, Gauthier S, Jicha G, Meguro K, O’Brien J, Pasquier F, Robert P, Rossor M, Salloway S, Stern Y, Visser PJ, Scheltens P. Research criteria for the diagnosis of Alzheimer’s disease: revising the NINCDS–ADRDA criteria. Lancet Neurol. 2007;6:734–46.

    Article  PubMed  Google Scholar 

  • Dubois B, Feldman HH, Jacova C, Cummings JL, Dekosky ST, Barberger-Gateau P, Delacourte A, Frisoni G, Fox NC, Galasko D, Gauthier S, Hampel H, Jicha GA, Meguro K, O’Brien J, Pasquier F, Robert P, Rossor M, Salloway S, Sarazin M, de Souza LC, Stern Y, Visser PJ, Scheltens P. Revising the definition of Alzheimer’s disease: a new lexicon. Lancet Neurol. 2010;9:1118–27.

    Article  PubMed  Google Scholar 

  • Easton JD, Saver JL, Albers GW, Alberts MJ, Chaturvedi S, Feldmann E, Hatsukami TS, Higashida RT, Johnston SC, Kidwell CS, Lutsep HL, Miller E, Sacco RL, American Heart Association; American Stroke Association Stroke Council; Council on Cardiovascular Surgery and Anesthesia; Council on Cardiovascular Radiology and Intervention; Council on Cardiovascular Nursing; Interdisciplinary Council on Peripheral Vascular Disease. Definition and evaluation of transient ischemic attack: a scientific statement for healthcare professionals from the American Heart Association/American Stroke Association Stroke Council; Council on Cardiovascular Surgery and Anesthesia; Council on Cardiovascular Radiology and Intervention; Council on Cardiovascular Nursing; and the Interdisciplinary Council on Peripheral Vascular Disease. Stroke. 2009;40:2276–93.

    Article  PubMed  Google Scholar 

  • Emre M, Aarsland D, Brown R, et al. Clinical diagnostic criteria for dementia associated with Parkinson’s disease. Mov Disord. 2007;22(12):1689–707.

    Article  PubMed  Google Scholar 

  • Geschwind MD, Su H, Haman A, Sejvar JJ, Miller BL. Rapidly progressive dementia. Ann Neurol. 2008;64:97–108.

    Article  PubMed Central  PubMed  Google Scholar 

  • Giles MF, Rothwell PM. Risk of stroke early after transient ischaemic attack: a systematic review and meta-analysis. Lancet Neurol. 2007;6:1063–72.

    Article  PubMed  Google Scholar 

  • Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76(11):1006–14.

    Article  PubMed Central  PubMed  Google Scholar 

  • Harding AE. Friedreich’s ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features. Brain. 1981;104:589–620.

    Article  CAS  PubMed  Google Scholar 

  • Hodges JR, editor. Early-Onset Dementia. A multidisciplinary approach. Oxford: Oxford University Press; 2001.

    Google Scholar 

  • Hort J, O’Brien JT, Gainotti G, Pirttila T, Popescu BO, Rektorova I, Sorbi S, Scheltens P, EFNS Scientist Panel on Dementia. EFNS guidelines for the diagnosis and management of Alzheimer’s disease. Eur J Neurol. 2010;17:1236–48.

    Article  CAS  PubMed  Google Scholar 

  • International Statistical Classification of Diseases and Related Health Problems, 10th. revision version for 2007. http://apps.who.int/classifications/apps/icd/icd10online/

  • Johnston SC, Rothwell PM, Nguyen-Huynh MN, Giles MF, Elkins JS, Bernstein AL, Sidney S. Validation and refinement of scores to predict very early stroke risk after transient ischaemic attack. Lancet. 2007;369:283–92.

    Article  PubMed  Google Scholar 

  • Kondziella D. Thirty neurological eponyms associated with the Nazi era. Eur Neurol. 2009;62:56–64.

    Article  PubMed  Google Scholar 

  • Kondziella D, Skagervik I. Malignant external otitis with extensive cranial neuropathy but no facial paralysis. J Neurol. 2007;254:1298–9.

    Article  PubMed  Google Scholar 

  • Kondziella D, Hansen K, Gonzalez T, Gideon P, Christiansen I, Sellebjerg F. Anti-thyroid antibodies in two patients with subacute dementia, ataxia and myoclonus [Danish]. Ugeskr Læg. 2012;174:577–9.

    PubMed  Google Scholar 

  • Kondziella D, Cortsen M, Eskesen V, Hansen K, Holtmannspötter M, Højgaard J, Stavngaard T, Søndergaard H, Wagner A, Welling KL. Update on acute endovascular and surgical stroke treatment. Acta Neurol Scand. 2013;127:1–9.

    Article  CAS  PubMed  Google Scholar 

  • Kumar S, Goddeau Jr RP, Selim MH, Thomas A, Schlaug G, Alhazzani A, Searls DE, Caplan LR. Atraumatic convexal subarachnoid hemorrhage: clinical presentation, imaging patterns, and etiologies. Neurology. 2010;74:893–9.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology. 1983;33:1444–52.

    Article  CAS  PubMed  Google Scholar 

  • Lavallée PC, Meseguer E, Abboud H, Cabrejo L, Olivot JM, Simon O, Mazighi M, Nifle C, Niclot P, Lapergue B, Klein IF, Brochet E, Steg PG, Lesèche G, Labreuche J, Touboul PJ. Amarenco P.A transient ischaemic attack clinic with round-the-clock access (SOS-TIA): feasibility and effects. Lancet Neurol. 2007;6:953–60.

    Article  PubMed  Google Scholar 

  • Lipton RB, Bigal ME. Ten Lessons on the epidemiology of migraine. Headache. 2007;47:S2–9.

    Article  PubMed  Google Scholar 

  • McKeith IG, Dickson DW, Lowe J, et al. Diagnosis and management of dementia with Lewy bodies: third report of the DLB consortium. Neurology. 2005;65:1863.

    Article  CAS  PubMed  Google Scholar 

  • McKhann GM, Knopman DS, Chertkow H, Hyman BT, Jack Jr CR, Kawas CH, Klunk WE, Koroshetz WJ, Manly JJ, Mayeux R, Mohs RC, Morris JC, Rossor MN, Scheltens P, Carrillo MC, Thies B, Weintraub S, Phelps CH. The diagnosis of dementia due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease. Alzheimers Dement. 2011;7:263–9.

    Article  PubMed Central  PubMed  Google Scholar 

  • Moore AP, Blumhardt LD. A prospective survey of the causes of non-traumatic spastic paraparesis and tetraparesis in 585 patients. Spinal Cord. 1997;35:361.

    Article  CAS  PubMed  Google Scholar 

  • Owen AM, Coleman MR, Boly M, Davis MH, Laureys S, Pickard JD. Detecting awareness in the vegetative state. Science. 2006;313:1402.

    Article  CAS  PubMed  Google Scholar 

  • Polman CH, Reingold SC, Banwell B, Clanet M, Cohen JA, Filippi M, Fujihara K, et al. Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol. 2011;69:292–302.

    Article  PubMed Central  PubMed  Google Scholar 

  • Posner JB, Saper CB, Schiff N, Plum F. Plum and Posner’s diagnosis of stupor and coma, Contemporary neurology, vol. 71. 4th ed. New York: Oxford University Press, USA; 2007.

    Google Scholar 

  • Petersen RC, Smith G, Waring S, Ivnik R, Tangalos E, Kokmen E, et al. MD mild cognitive impairment: clinical characterization and outcome. Arch Neurol. 1999;56:303–8.

    Article  CAS  PubMed  Google Scholar 

  • Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. 2011;134:2456–77.

    Article  PubMed Central  PubMed  Google Scholar 

  • Román GC, Tatemichi TK, Erkinjuntti T, Cummings JL, Masdeu JC, Garcia JH, Amaducci L, Orgogozo JM, Brun A, Hofman A, et al. Vascular dementia: diagnostic criteria for research studies. Report of the NINDS-AIREN International Workshop. Neurology. 1993;43:250–60.

    Article  PubMed  Google Scholar 

  • Rothwell PM, Giles MF, Chandratheva A, Marquardt L, Geraghty O, Redgrave JN, Lovelock CE, Binney LE, Bull LM, Cuthbertson FC, Welch SJ, Bosch S, Alexander FC, Silver LE, Gutnikov SA, Mehta Z, Early use of Existing Preventive Strategies for Stroke (EXPRESS) study. Effect of urgent treatment of transient ischaemic attack and minor stroke on early recurrent stroke (EXPRESS study): a prospective population-based sequential comparison. Lancet. 2007;370(9596):1432–42.

    Article  PubMed  Google Scholar 

  • Sorbi S, Hort J, Erkinjuntti T, Fladby T, Gainotti G, Gurvit H, Nacmias B, Pasquier F, Popescu BO, Rektorova I, Religa D, Rusina R, Rossor M, Schmidt R, Stefanova E, Warren JD, Scheltens P, EFNS Scientist Panel on Dementia and Cognitive Neurology. EFNS-ENS Guidelines on the diagnosis and management of disorders associated with dementia. Eur J Neurol. 2012;19:1159–79.

    Article  CAS  PubMed  Google Scholar 

  • Undén J, Romner B. A new objective method for CT triage after minor head injury-serum S100B. Scand J Clin Lab Invest. 2009;69:13–7.

    Article  PubMed  Google Scholar 

  • Vrancken AF, Kalmijn S, Buskens E, Franssen H, Vermeulen M, Wokke JH, Notermans NC. Feasibility and cost efficiency of a diagnostic guideline for chronic polyneuropathy: a prospective implementation study. J Neurol Neurosurg Psychiatry. 2006;77:397–401.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  • Waldemar G, Burns A, editors. Alzheimer’s disease. London: Oxford University Press; 2009.

    Google Scholar 

  • Waubant E, Weinshenker B, Wolinsky JS. Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol. 2011;69:292–302.

    Article  PubMed Central  PubMed  Google Scholar 

  • Wegener M, la Cour M, Milea D. Loss of sight and hearing and cognitive dysfunction – Susac’s syndrome? Ugeskr Laeger. 2009;171:618–20.

    PubMed  Google Scholar 

  • World Health Organization. International Statistical Classification of Diseases and Related Health Problems, 10th revision, vol. 2. 2010 ed. www.who.int/classifications/icd/

  • Wu CM, McLaughlin K, Lorenzetti DL, Hill MD, Manns BJ, Ghali WA. Early risk of stroke after transient ischemic attack: a systematic review and meta-analysis. Arch Intern Med. 2007;167(22):2417–22.

    Article  PubMed  Google Scholar 

  • Zongo D, Ribéreau-Gayon R, Masson F, Laborey M, Contrand B, Salmi LR, Montaudon D, Beaudeux JL, Meurin A, Dousset V, Loiseau H, Lagarde E. S100-B protein as a screening tool for the easy assessment of minor head injury. Ann Emerg Med. 2012;59:209–18.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Rigshospitalet Copenhagen University Hospital, Copenhagen, Denmark

    Daniel Kondziella MD, PhD, FEBN & Gunhild Waldemar MD, DMSC

Authors
  1. Daniel Kondziella MD, PhD, FEBN
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Gunhild Waldemar MD, DMSC
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag London

About this chapter

Cite this chapter

Kondziella, D., Waldemar, G. (2014). Differential Diagnosis: “What Is the Lesion?”. In: Neurology at the Bedside. Springer, London. https://doi.org/10.1007/978-1-4471-5251-4_4

Download citation

  • DOI: https://doi.org/10.1007/978-1-4471-5251-4_4

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-5250-7

  • Online ISBN: 978-1-4471-5251-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation