ABSTRACT
The evaluation of patients with rapidly progressive dementia can be challenging, largely due to the rarity of the conditions responsible for such fulminant presentations of dementia. This article presents a framework for categorizing such disorders based on the underlying pathophysiology, including degenerative, inflammatory, vascular, toxic, metabolic, neoplastic, and infectious causes of rapidly progressive dementia. Diagnostic approaches, representative imaging abnormalities, and treatment options for these uncommon conditions are also presented.
KEYWORDS
Rapidly progressive dementia
REFERENCES
-
1
Glatzel M, Stoeck K, Seeger H, Luhrs T, Aguzzi A.
Human prion diseases: molecular and clinical aspects.
Arch Neurol.
2005;
62
545-552
-
2
Alperovitch A, Zerr I, Pocchiari M et al..
Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease [erratum appears in Lancet 2000;355:72].
Lancet.
1999;
353
1673-1674
-
3
Collie D A, Sellar R J, Zeidler M, Colchester A C, Knight R, Will R G.
MRI of Creutzfeldt-Jakob disease: imaging features and recommended MRI protocol.
Clin Radiol.
2001;
56
726-739
-
4
Zeidler M, Sellar R J, Collie D A et al..
The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease [erratum appears in Lancet 2000;356:170].
Lancet.
2000;
355
1412-1418
-
5
Aksamit Jr A J, Preissner C M, Homburger H A.
Quantitation of 14-3-3 and neuron-specific enolase proteins in CSF in Creutzfeldt-Jakob disease.
Neurology.
2001;
57
728-730
-
6
Gambetti P, Kong Q, Zou W, Parchi P, Chen S G.
Sporadic and familial CJD: classification and characterisation.
Br Med Bull.
2003;
66
213-239
-
7
Tschampa H J, Neumann M, Zerr I et al..
Patients with Alzheimer's disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease.
J Neurol Neurosurg Psychiatry.
2001;
71
33-39
-
8
Haik S, Brandel J P, Sazdovitch V et al..
Dementia with Lewy bodies in a neuropathologic series of suspected Creutzfeldt-Jakob disease.
Neurology.
2000;
55
1401-1404
-
9
Miller B L, Josephs K AJ, Parisi J E, Keegan B M.
A 34-year-old man with progressive behavioral and language disturbance.
Neurology.
2007;
68
68-74
-
10
Brain L, Jellinek E H, Ball K.
Hashimoto's disease and encephalopathy.
Lancet.
1966;
2
512-514
-
11
Caselli R J, Boeve B F, Scheithauer B W, O'Duffy J D, Hunder G G.
Nonvasculitic autoimmune inflammatory meningoencephalitis (NAIM): a reversible form of encephalopathy.
Neurology.
1999;
53
1579-1581
-
12
Caselli R J, Scheithauer B W, Bowles C A et al..
The treatable dementia of Sjogren's syndrome.
Ann Neurol.
1991;
30
98-101
-
13
Pittock S J, Yoshikawa H, Ahlskog J E et al..
Glutamic acid decarboxylase autoimmunity with brainstem, extrapyramidal, and spinal cord dysfunction.
Mayo Clin Proc.
2006;
81
1207-1214
-
14
Schielke E, Nolte C, Muller W, Bruck W.
Sarcoidosis presenting as rapidly progressive dementia: clinical and neuropathological evaluation.
J Neurol.
2001;
248
522-524
-
15
Castillo P, Woodruff B, Caselli R et al..
Steroid-responsive encephalopathy associated with autoimmune thyroiditis [see comment].
Arch Neurol.
2006;
63
197-202
-
16
Chong J Y, Rowland L P, Utiger R D.
Hashimoto encephalopathy: syndrome or myth?.
Arch Neurol.
2003;
60
164-171
-
17
Schauble B, Castillo P R, Boeve B F, Westmoreland B F.
EEG findings in steroid-responsive encephalopathy associated with autoimmune thyroiditis.
Clin Neurophysiol.
2003;
114
32-37
-
18
Bohnen N I, Parnell K J, Harper C M.
Reversible MRI findings in a patient with Hashimoto's encephalopathy.
Neurology.
1997;
49
246-247
-
19
Boers P M, Colebatch J G.
Hashimoto's encephalopathy responding to plasmapheresis.
J Neurol Neurosurg Psychiatry.
2001;
70
132
-
20
West S G.
Central nervous system vasculitis.
Curr Rheumatol Rep.
2003;
5
116-127
-
21
Calabrese L H.
Diagnostic strategies in vasculitis affecting the central nervous system.
Cleve Clin J Med.
2002;
69(suppl 2)
SII105-SII108
-
22
Vollmer T L, Guarnaccia J, Harrington W, Pacia S V, Petroff O A.
Idiopathic granulomatous angiitis of the central nervous system: diagnostic challenges.
Arch Neurol.
1993;
50
925-930
-
23
Finelli P F, Onyiuke H C, Uphoff D F.
Idiopathic granulomatous angiitis of the CNS manifesting as diffuse white matter disease [see comment].
Neurology.
1997;
49
1696-1699
-
24
Calabrese L H, Furlan A J, Gragg L A, Ropos T J.
Primary angiitis of the central nervous system: diagnostic criteria and clinical approach.
Cleve Clin J Med.
1992;
59
293-306
-
25
Younger D S.
Vasculitis of the nervous system.
Curr Opin Neurol.
2004;
17
317-336
-
26
Regan M, Haque U, Pomper M, Pardo C, Stone J.
Central nervous system vasculitis as a complication of refractory dermatomyositis.
J Rheumatol.
2001;
28
207-211
-
27
Buxton N, McConachie N S.
Amphetamine abuse and intracranial haemorrhage.
J R Soc Med.
2000;
93
472-477
-
28
Quintero M, Mirza N, Chang H, Perl A.
Antiphospholipid antibody syndrome associated with primary angiitis of the central nervous system: report of two biopsy proven cases.
Ann Rheum Dis.
2006;
65
408-409
-
29
Silbert P L, Bartleson J D, Miller G M, Parisi J E, Goldman M S, Meyer F B.
Cortical petechial hemorrhage, leukoencephalopathy, and subacute dementia associated with seizures due to cerebral amyloid angiopathy.
Mayo Clin Proc.
1995;
70
477-480
-
30
Petty G W, Engel A G, Younge B R et al..
Retinocochleocerebral vasculopathy.
Medicine.
1998;
77
12-40
-
31
Dichgans M, Mayer M, Uttner I et al..
The phenotypic spectrum of CADASIL: clinical findings in 102 cases.
Ann Neurol.
1998;
44
731-739
-
32
Krolak-Salmon P, Montavont A, Hermier M, Milliery M, Vighetto A.
Thalamic venous infarction as a cause of subacute dementia.
Neurology.
2002;
58
1689-1691
-
33
Spiers H J, Maguire E A, Burgess N.
Hippocampal amnesia.
Neurocase.
2001;
7
357-382
-
34
Velasco J, Head M, Farlin E, Lippmann S.
Unsuspected subdural hematoma as a differential diagnosis in elderly patients.
South Med J.
1995;
88
977-979
-
35
Isozumi K, Fukuuchi Y, Tanaka K, Nogawa S, Ishihara T, Sakuta R A.
MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) mtDNA mutation that induces subacute dementia which mimicks Creutzfeldt-Jakob disease.
Intern Med.
1994;
33
543-546
-
36
Constine L S, Konski A, Ekholm S, McDonald S, Rubin P.
Adverse effects of brain irradiation correlated with MR and CT imaging.
Int J Radiat Oncol Biol Phys.
1988;
15
319-330
-
37
Moretti R, Torre P, Antonello R M et al..
Neuropsychological evaluation of late-onset post-radiotherapy encephalopathy: a comparison with vascular dementia.
J Neurol Sci.
2005;
229-230
195-200
-
38
Abrey L E, Yahalom J, DeAngelis L M.
Treatment for primary CNS lymphoma: the next step.
J Clin Oncol.
2000;
18
3144-3150
-
39
Weigel R, Senn P, Weis J, Krauss J K.
Severe complications after intrathecal methotrexate (MTX) for treatment of primary central nervous system lymphoma (PCNSL).
Clin Neurol Neurosurg.
2004;
106
82-87
-
40
Lai R, Abrey L E, Rosenblum M K, DeAngelis L M.
Treatment-induced leukoencephalopathy in primary CNS lymphoma: a clinical and autopsy study [see comment].
Neurology.
2004;
62
451-456
-
41
Franco D A, Greenberg H S.
5-FU multifocal inflammatory leukoencephalopathy and dihydropyrimidine dehydrogenase deficiency.
Neurology.
2001;
56
110-112
-
42
Steeghs N, de Jongh F E, Sillevis Smitt P A, van den Bent M J.
Cisplatin-induced encephalopathy and seizures.
Anticancer Drugs.
2003;
14
443-446
-
43
Marchiori P E, Mies S, Scaff M.
Cyclosporine A-induced ocular opsoclonus and reversible leukoencephalopathy after orthotopic liver transplantation: brief report.
Clin Neuropharmacol.
2004;
27
195-197
-
44
Schuuring J, Wesseling P, Verrips A.
Severe tacrolimus leukoencephalopathy after liver transplantation.
AJNR Am J Neuroradiol.
2003;
24
2085-2088
-
45
Wu V C, Huang J W, Lien H C et al..
Levamisole-induced multifocal inflammatory leukoencephalopathy: clinical characteristics, outcome, and impact of treatment in 31 patients.
Medicine.
2006;
85
203-213
-
46
Stott V L, Hurrell M A, Anderson T J.
Reversible posterior leukoencephalopathy syndrome: a misnomer reviewed.
Intern Med J.
2005;
35
83-90
-
47
Coker S B.
The diagnosis of childhood neurodegenerative disorders presenting as dementia in adults [see comment].
Neurology.
1991;
41
794-798
-
48
Racette B A, Perry A, D'Avossa G, Perlmutter J S.
Late-onset neurodegeneration with brain iron accumulation type 1: expanding the clinical spectrum.
Mov Disord.
2001;
16
1148-1152
-
49
Thomson A D, Marshall E J.
The natural history and pathophysiology of Wernicke's encephalopathy and Korsakoff's psychosis.
Alcohol Alcohol.
2006;
41
151-158
-
50
Nierenberg D W, Nordgren R E, Chang M B et al..
Delayed cerebellar disease and death after accidental exposure to dimethylmercury [see comment].
N Engl J Med.
1998;
338
1672-1676
-
51
Gordon J N, Taylor A, Bennett P N.
Lead poisoning: case studies.
Br J Clin Pharmacol.
2002;
53
451-458
-
52
Ratnaike R N.
Acute and chronic arsenic toxicity.
Postgrad Med J.
2003;
79
391-396
-
53
Josephs K A, Ahlskog J E, Klos K J et al..
Neurologic manifestations in welders with pallidal MRI T1 hyperintensity [see comment].
Neurology.
2005;
64
2033-2039
-
54
Slee M, Pretorius P, Ansorge O, Stacey R, Butterworth R.
Parkinsonism and dementia due to gliomatosis cerebri mimicking sporadic Creutzfeldt-Jakob disease (CJD).
J Neurol Neurosurg Psychiatry.
2006;
77
283-284
-
55
Lozsadi D A, Wieshmann U, Enevoldson T P.
Neurological presentation of intravascular lymphoma: report of two cases and discussion of diagnostic challenges.
Eur J Neurol.
2005;
12
710-714
-
56
Beristain X, Azzarelli B.
The neurological masquerade of intravascular lymphomatosis.
Arch Neurol.
2002;
59
439-443
-
57
Baehring J M, Longtine J, Hochberg F H.
A new approach to the diagnosis and treatment of intravascular lymphoma.
J Neurooncol.
2003;
61
237-248
-
58
Koizumi M, Nishimura M, Yokota A, Munekata S, Kobayashi T, Saito Y.
Successful treatment of intravascular malignant lymphomatosis with high-dose chemotherapy and autologous peripheral blood stem cell transplantation.
Bone Marrow Transplant.
2001;
27
1101-1103
-
59
Lawn N D, Westmoreland B F, Kiely M J, Lennon V A, Vernino S.
Clinical, magnetic resonance imaging, and electroencephalographic findings in paraneoplastic limbic encephalitis.
Mayo Clin Proc.
2003;
78
1363-1368
-
60
Practice parameter for diagnosis and evaluation of dementia (summary statement): report of the Quality Standards Subcommittee of the American Academy of Neurology.
Neurology.
1994;
44
2203-2206
-
61
Knopman D S, DeKosky S T, Cummings J L et al..
Practice parameter. Diagnosis of dementia (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology [see comment].
Neurology.
2001;
56
1143-1153
-
62
Lessig S, Tecoma E.
Perils of the prozone reaction: neurosyphilis presenting as an RPR-negative subacute dementia.
Neurology.
2006;
66
777
-
63
Brooks B R, Walker D L.
Progressive multifocal leukoencephalopathy.
Neurol Clin.
1984;
2
299-313
-
64 Berger J R, Major E O, Sabath B. Clinical Neurovirology. New York, NY; Informa Healthcare 2003
-
65
Kleinschmidt-DeMasters B K, Tyler K L.
Progressive multifocal leukoencephalopathy complicating treatment with natalizumab and interferon beta-1a for multiple sclerosis [see comment].
N Engl J Med.
2005;
353
369-374
-
66
Langer-Gould A, Atlas S W, Green A J, Bollen A W, Pelletier D.
Progressive multifocal leukoencephalopathy in a patient treated with natalizumab [see comment].
N Engl J Med.
2005;
353
375-381
-
67
Bartt R E.
Multiple sclerosis, natalizumab therapy, and progressive multifocal leukoencephalopathy [see comment].
Curr Opin Neurol.
2006;
19
341-349
-
68
Snyder C H.
Coccidioidal meningitis presenting as memory loss.
J Am Acad Nurse Pract.
2005;
17
181-186
-
69
Boukobza M, Tamer I, Guichard J P et al..
[Tuberculosis of the central nervous system: MRI features and clinical course in 12 cases].
J Neuroradiol.
1999;
26
172-181
-
70
Kennedy P G, Chaudhuri A.
Herpes simplex encephalitis [see comment].
J Neurol Neurosurg Psychiatry.
2002;
73
237-238
-
71 Venkataramana A, McArthur J C. HIV Dementia. In: Roos KL Principles of Neurologic Infectious Diseases. New York, NY; McGraw-Hill 2005: 113-136
-
72
Navia B A, Jordan B D, Price R W.
The AIDS dementia complex: I. Clinical features.
Ann Neurol.
1986;
19
517-524
-
73
Wendel K A, McArthur J C.
Acute meningoencephalitis in chronic human immunodeficiency virus (HIV) infection: putative central nervous system escape of HIV replication.
Clin Infect Dis.
2003;
37
1107-1111
-
74
Sacktor N, Schifitto G, McDermott M P, Marder K, McArthur J C, Kieburtz K.
Transdermal selegiline in HIV-associated cognitive impairment: pilot, placebo-controlled study.
Neurology.
2000;
54
233-235
-
75
Anderson E R, Gendelman H E, Xiong H.
Memantine protects hippocampal neuronal function in murine human immunodeficiency virus type 1 encephalitis.
J Neurosci.
2004;
24
7194-7198
-
76
Prashanth L K, Taly A B, Ravi V, Sinha S, Arunodaya G R.
Adult onset subacute sclerosing panencephalitis: clinical profile of 39 patients from a tertiary care centre.
J Neurol Neurosurg Psychiatry.
2006;
77
630-633
-
77
Rossi T, Haghighipour R, Haghighi M, Paolini S, Scarpino O.
Cerebral Whipple's disease as a cause of reversible dementia.
Clin Neurol Neurosurg.
2005;
107
258-261
-
78
Waniek C, Prohovnik I, Kaufman M A, Dwork A J.
Rapidly progressive frontal-type dementia associated with Lyme disease.
J Neuropsychiatry Clin Neurosci.
1995;
7
345-347
Bryan Keith WoodruffM.D.
Mayo Clinic Arizona, 13400 East Shea Boulevard
Scottsdale, AZ 85259
