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Limbic encephalitis

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Brain limbicsystem.jpg|
Limbic encephalitis
ICD-10 G131
ICD-9
OMIM [1]
DiseasesDB 30707
MedlinePlus [2]
eMedicine /
MeSH {{{MeshNumber}}}

Limbic encephalitis is a form of encephalitis, which is to say, it is a disease characterised by inflammation of the brain.[1] Limbic encephalitis is caused by auto-immunity: that is an abnormal state where the body produces antibodies against itself. Some cases are associated with cancer and some are not.[1] Although the disease is known as "limbic" encephalitis, the fact is that disease is seldom limited to the limbic system and post-mortem studies usually show involvement of other parts of the brain also.[2][3][4] The disease was first described by Brierley and others in 1960 as a series of three cases. The link to cancer was first noted in 1968[3] and confirmed by later investigators.[5]

The majority of cases of limbic encephalitis are paraneoplastic syndromes associated with a tumour (diagnosed or undiagnosed). In cases caused by tumour, cure is only achieved when the tumour is removed completely (this is not always possible). Limbic encephalitis is classified according to the auto-antibody that causes the disease.

The most common types are:

Symptoms and signsEdit

Symptoms develop over days or weeks. The subacute development of short-term memory deficits is considered the hallmark of this disease,[1] but this symptom is often overlooked, because it is overshadowed by other more obvious symptoms such as headache, irritability, sleep disturbance, delusions, hallucinations, agitation, seizures and psychosis, or because the other symptoms mean the patient has to be sedated, and it is not possible to test memory in a sedated patient. Examination of cerebrospinal fluid (CSF) shows elevated numbers of lymphocytes (but usually < 100 cells/µl); elevated CSF protein (but usually <1.5 g/l), normal glucose, elevated IgG index and oligoclonal bands. Patients with antibodies to voltage-gated potassium channels may have a completely normal CSF examination.[6][7][8]

DiagnosisEdit

The diagnosis of limbic encephalitis is extremely difficult and it is usual for the diagnosis to be delayed for weeks. The key diagnostic test (detection of specific auto-antibodies in cerebrospinal fluid) is not routinely available offered by most immunology laboratories. Some of the rarer auto-antibodies (e.g., NMDAR) have no commercially available assay and can only be measured by a very small number of research laboratories worldwide, further delaying diagnosis by weeks or months. Most patients with limbic encephalitis are initially diagnosed with herpes simplex encephalitis, because the two syndromes cannot be distinguished clinically.[1] HHV-6 (human herpes virus 6) encephalitis is also clinically indistinguishable from limbic encephalitis.[1]

There are two sets of diagnostic criteria used. The oldest are those proposed by Gultekin et al. in 2000.[9]

Gultekin criteria
EITHER, Pathological demonstration of limbic encephalitis
OR, All four of:
  • Short-term memory loss, seizures, or psychiatric symptoms suggestive of limbic system involvement
  • <4 years between onset of neurological symptoms and cancer diagnosis
  • Exclusion of metastases, infection, metabolic and nutritional deficits, stroke and side-effects of therapy that may cause limbic encephalopathy
  • At least one of
    • CSF with inflammatory findings
    • Hyperintensity of the temporal lobes bilaterally on MRI FLAIR or T2
    • EEG with epilepsy or slow activity involving the temporal lobes focally

A revised set of criteria were proposed by Graus and Saiz in 2005.[10]

Graus and Saiz criteria
All four of
  • Subacute onset (<12 weeks) of seizures, short-term memory loss, confusion, and psychiatric symptoms
  • Neuropathologic or radiologic evidence (MRI, SPECT, PET) of involvement of the limbic system
  • Exclusion of other possible aetiologies of limbic dysfunction
  • Demonstration of a cancer within 5 years of the diagnosis of neurologic symptoms, or the development of classic symptoms of limbic dysfunction in association with a well-characterized paraneoplastic antibody (Hu, Ma2, CV2, amphiphysin, Ri)

The main distinction between the two sets of criteria is whether or not the detection of a paraneoplastic antibody is needed for diagnosis.

Antibodies against intracellular neuronal antigensEdit

The main antibodies within this group are those against Hu, Ma2, CV2, amphiphysin and Ri. The syndrome of anti-Ma2 encephalitis may be clinically mistaken for Whipple's disease.[11]

Antibodies against cell membrane antigensEdit

The main antibodies within this group are those again voltage-gated potassium channels (VGKC) and anti-N-methyl-D-aspartate receptors (NMDAR). They are associated with tumours of the thymus and ovary. Anti-NDMAR encephalitis is strongly associated with benign tumours of the ovary (usually teratomas or dermoid cysts).

Patients with NMDAR encephalitis are frequently young women who present with fever, headache and fatigue. This is often misdiagnosed as influenza, but progresses to severe behavioural and personality disturbance, delusions, paranoia and hallucinations.[12] Patients may therefore initially be admitted to a psychiatric ward for acute psychosis or schizophrenia. The disease then progresses to catatonia, seizures and loss of consciousness. The next stage is hypoventilation requiring intubation, orofacial dyskinesia and autonomic instability (dramatic fluctuations in blood pressure, temperature and heart rate).[13] Treatment is removal of the associated ovarian tumour.

TreatmentEdit

Limbic encephalitis is a rare condition with no randomised-controlled trials to guide treatment. Treatments that have been tried include intravenous immunoglobulin, plasma exchange, corticosteroids, cyclophosphamide and rituximab.[1] None of these treatments have been proven to work. If an associated tumour is found, then recovery is not possible until the tumour is removed. Unfortunately, this is not always possible, especially if the tumour is malignant and advanced.

ReferencesEdit

  1. 1.0 1.1 1.2 1.3 1.4 1.5 Tüzün E, Dalmau J (2007). Limbic encephalitis and variants: classification, diagnosis and treatment. The Neurologist 13 (5).
  2. Brierley JB, Corsellis JAN, Hierons R, et al. (1960). Subacute encephalitis of later adult life. Mainly affecting the limbic areas. Brain 83 (3): 357–368.
  3. 3.0 3.1 Corsellis JA, Goldberg GJ, Norton AR (1968). "Limbic encephalitis" and its association with carcinoma. Brain 91 (3): 481–496.
  4. Bakheit AM, Kennedy PG, Behan PO (1990). Paraneoplastic limbic encephalitis: clinico-pathological correlations. J Neurol Neurosurg Psychiatry 53 (12): 1084–1088.
  5. Henson RA, Hoffman HL, Urich H (1965). Encephalomyelitis with carcinoma. Brain 88 (3): 449–464.
  6. Buckley C, Oger J, Clover L, et al. (2001). Potassium channel antibodies in two patients with reversible limbic encephalitis. Ann Neurol 50: 73–78.
  7. Vincent A, Buckley C, Schott JM, et al. (2004). Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain 127 (3): 701–712.
  8. Thieben MJ, Lennon VA, Boeve BF, et al. (2004). Potentially reversible auto-immune limbic encephalitis with neuronal potassium channel antibody. Neurology 62 (7): 1177–1182.
  9. Gultekin SH, Rosenfeld MR, Voltz R, et al. (2000). Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain 123 (7): 1481–1494.
  10. Graus F, Saiz A (2005). Limbic encephalitis: a probably under-recognized syndrome. Neurologia 20: 24–30.
  11. Castle J, Sakonju A, Dalmau J, et al. (2006). Anti-Ma2-associated encephalitis with normal FDG-PET: a case of pseudo-Whipple's disease. Nat Clin Pract Neurol 2 (10): 566–572.
  12. Koide R, Shimizu T, Koike K, et al. (2007). EFA6A-like antibodies in paraneoplastic encephalitis associated with immature ovarian teratoma: a case report. J Neurooncol 81: 71–74.
  13. Dalmau J, Tuzun E, Wu HY, et al. (2007). Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 61: 25–36.

External linksEdit

  • PNSEURONET: the PNSEURONET group provides information on paraneoplastic neurological syndromes for medical specialists worldwide.
  • [3]: "Diagnosis: 'Forgetting Everything'", New York Times, November 11, 2007.

Template:Paraneoplastic syndromes

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