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Mollaret's Meningitis

Benign Recurrent Aseptic Meningitis

Mollaret's meningitis also referred to as recurrent benign lymphocytic meningitis, is characterized by a transient and recurrent onset of neurological symptoms such as headaches, fever, meningismus and various neurological deficits that last for a few days in a self-limiting fashion. Herpes simplex type 2 virus is the causative agent in virtually all cases. CSF examination and polymerase chain reaction (PCR) testing to detect viral DNA are recommended steps in the diagnostic workup.


Presentation

Although it was initially described the 1940s, the cause of Mollaret's meningitis was revealed later to be an infection due to the reactivation of herpes simplex viruses (HSV-2), much more commonly HSV-2 [1] [2] [3]. Due to still undisclosed reasons, Mollaret's meningitis is more commonly seen in females and predominantly affects adults around 35 years of age, but the infection is described across all age groups [1] [2] [3] [4]. The clinical presentation is distinguished by a recurring and sudden onset of headaches that may be quite severe, fever, photophobia, radiculopathy, paresthesias, arthralgia and in about 50% of cases, neurological symptoms - hallucinations, cranial nerve deficits, diplopia, seizures, or even altered consciousness [1] [2] [3] [4]. Patients report maximal discomfort and pain in the first few hours of symptom appearance, after which a gradual decline in severity is observed [1]. Because of a relatively short duration of symptoms (about 1-7 days in most cases), their recurrence (weeks, months or sometimes even years can pass between attacks), as well as their self-limiting nature that does not pose any harm to the patient, the term benign recurrent lymphocytic meningitis (BRLM) is often used to describe Mollaret's meningitis in the literature [1] [5] [6]. The recurrent appearance of symptoms usually lasts over a period of 3-5 years, followed by a spontaneous resolution in virtually all cases [1] [3] [4].

Fever
  • On admission, the vital signs were stable, there was no fever, and a complete physical examination was normal except for the...[dx.doi.org]
  • In many ways it resembles recurrent hereditary polyserositis (familial Mediterranean fever) in its natural history, pattern of attacks, and response to colchicine. Association of the two conditions has been reported, so far, in two patients only.[ncbi.nlm.nih.gov]
  • Mollaret's meningitis also referred to as recurrent benign lymphocytic meningitis, is characterized by a transient and recurrent onset of neurological symptoms such as headaches, fever, meningismus and various neurological deficits that last for a few[symptoma.com]
  • He also described benign, recurrent endothelio-leukocytic meningitis in three patients who had short-lived recurrent attacks of fever, headache and vomiting caused by sterile meningitis, with 'fantomes cellulaires' (cell ghosts) in the cerebrospinal fluid[ncbi.nlm.nih.gov]
  • Our patient’s presentation fulfilled each of Bruyns requirements with the exception of recurrent fever criteria. Our patient did not present with a fever, nor did she report having a fever before coming to the hospital.[ojhas.org]
Back Pain
  • Her past medical history was significant for depression on sertraline, back pain, and previous episodes of meningitis found to be aseptic in nature. Also noted was a history of lupus erythematosus controlled with no ongoing therapy.[dx.doi.org]
Diplopia
  • […] presentation is distinguished by a recurring and sudden onset of headaches that may be quite severe, fever, photophobia, radiculopathy, paresthesias, arthralgia and in about 50% of cases, neurological symptoms - hallucinations, cranial nerve deficits, diplopia[symptoma.com]
  • More severe cases can occur with neurological abnormalities, including seizures, diplopia, abnormal reflexes, cranial nerve palsy, hallucinations and coma.[patient.info]
  • Transient neurological abnormalities (seizures, diplopia, pathologic reflexes, cranial nerve paresis, hallucinations, and aphasia) have all been reported 2 , 3 . However persistent neurological deficits should call the diagnosis into question.[ispub.com]
  • Approximately one-half of patients have transient neurological manifestations, including seizures, hallucinations, diplopia, cranial nerve palsies, or altered levels of consciousness.[dx.doi.org]
Meningism
  • INTRODUCTION: Mollaret's meningitis is an unusual and under-appreciated syndrome of benign, recurrent aseptic meningitis.[ncbi.nlm.nih.gov]
  • Abstract It is well known that herpes simplex virus (HSV) type 2 produces acute meningitis, while HSV type 2 rarely causes recurrent meningitis (Mollaret's meningitis).[ncbi.nlm.nih.gov]
  • Since Mollaret's meningitis is a recurrent, benign (non-cancerous), aseptic meningitis, it is also referred to as benign recurrent lymphocytic meningitis.[en.wikipedia.org]
  • (BRLM) is often used to describe Mollaret's meningitis in the literature.[symptoma.com]
  • Keywords Meningitis Cerebral Spinal Fluid Aseptic Meningitis Lymphocytic Pleocytosis Recurrent Meningitis These keywords were added by machine and not by the authors.[dx.doi.org]
Headache
  • A 57-year-old woman presented to the emergency department (ED) for headache and nausea lasting 2 weeks. She had near-syncope, vomiting, chills, neck stiffness and dizziness, but denied any fever. The headache was progressive and bi-frontal.[dx.doi.org]
  • Symptoms can include headache, sensitivity to light, neck stiffness, nausea, and vomiting. In some patients, fever is absent.[en.wikipedia.org]
  • Mollaret's meningitis also referred to as recurrent benign lymphocytic meningitis, is characterized by a transient and recurrent onset of neurological symptoms such as headaches, fever, meningismus and various neurological deficits that last for a few[symptoma.com]
  • He also described benign, recurrent endothelio-leukocytic meningitis in three patients who had short-lived recurrent attacks of fever, headache and vomiting caused by sterile meningitis, with 'fantomes cellulaires' (cell ghosts) in the cerebrospinal fluid[ncbi.nlm.nih.gov]
Seizure
  • Many people have Auras before seizures, which is like warning signs that a seizure is most likely coming on.[honeycomb.click]
  • Without proper care, symptoms can escalate to seizures and even coma. Thus, a greater understanding and knowledge of this disease will facilitate better patient care in emergency situations.[recurrentmeningitis.org]
  • Anti-seizure medications such as Gabapentin (Neurontin), Pregabalin (Lyrica), Lamotrigine (Lamictal) have also been used.[mollaretsmeningitis.org]
  • […] is distinguished by a recurring and sudden onset of headaches that may be quite severe, fever, photophobia, radiculopathy, paresthesias, arthralgia and in about 50% of cases, neurological symptoms - hallucinations, cranial nerve deficits, diplopia, seizures[symptoma.com]
Neck Stiffness
  • Symptoms can include headache, sensitivity to light, neck stiffness, nausea, and vomiting. In some patients, fever is absent.[en.wikipedia.org]
  • She had near-syncope, vomiting, chills, neck stiffness and dizziness, but denied any fever. The headache was progressive and bi-frontal.[dx.doi.org]
  • This site is intended for healthcare professionals General Practice Notebook Mollaret in 1944 was the first to describe a meningitic syndrome characterised by recurrent febrile episodes of 2-4 days duration with simultaneous headache, neck stiffness and[gpnotebook.co.uk]
  • Physical examination was remarkable for neck stiffness but no focal neurologic deficits. CT head was unremarkable.[ojhas.org]
Ataxia
  • ( Friedreich's ataxia , Ataxia telangiectasia , Hereditary spastic paraplegia ) Spinal muscular atrophy : Werdnig-Hoffman - Kugelberg-Welander - Fazio Londe - MND ( ALS , PMA , PBP , PP , PLS ) Extrapyramidal and movement disorders Parkinson's disease[wikidoc.org]

Workup

The diagnosis of Mollaret's meningitis may be difficult to make without adequate clinical suspicion, which can only be raised if a complete patient history is obtained. The duration of symptoms, their pattern, as well as recurrence and remission, are highly specific signs of Mollaret's meningitis. In fact, certain reports have emphasized that at least three episodes are necessary in order to consider Mollaret's meningitis in the differential diagnosis [1]. A complete physical examination may confirm signs and symptoms suggestive of a meningeal irritation. In that case, appropriate laboratory tests should be obtained. Cerebrospinal fluid (CSF) examination is recommended in all patients who exhibit signs of a central nervous system (CNS) infection, and a lumbar puncture is necessary to evaluate the CSF contents, but only after an imaging study of the endocranium (computed tomography) is performed. Lymphocytic pleocytosis (an increased number of lymphocytes in the CSF) is one of the most important signs of viral meningitis, while the presence of large granular plasma cells is a distinguishing feature of Mollaret's meningitis [1]. However, they can be detected only in the first 24 hours after symptom onset, and their monocytic-macrophage origin suggests that neutrophilic pleocytosis is also a possible finding, a rare feature seen in West Nile encephalitis as well [1] [6]. Protein levels might be elevated, whereas glucose levels are normal [1]. In addition to clinical and CSF criteria, detection of HSV (either HSV-1 or HSV-2) genetic material (DNA) in CSF by PCR methods is the gold standard for confirming the diagnosis [1] [4] [5] [6]. Because HSV is a rather common cause of viral meningitis, PCR testing should be done whenever CSF shows lymphocytic predominance [6].

Cerebrospinal Fluid Abnormality
  • fluid abnormalities may not be detectable for the entire time.[en.wikipedia.org]
  • fluid abnormalities may not be detectable for the entire time. [6] Diagnosis can be elusive, as Helbok et al. note: "in reality, many more weeks, even months pass by until the diagnosis is established.[forums.phoenixrising.me]

Treatment

  • Antiviral treatment has been used successfully for prophylaxis and treatment.[ncbi.nlm.nih.gov]
  • In our patient, a treatment with indomethacin during the last attack resulted in a clear and rapid improvement of symptoms. Since this episode only mild relapses have occurred, all of which responded well to the same treatment.[ncbi.nlm.nih.gov]
  • Treatment with acyclovir may be beneficial in decreasing the severity and duration of attacks and in preventing further episodes. [Au?1].[ncbi.nlm.nih.gov]
  • Only 1 patient was offered prophylactic antiviral treatment during the study period (45 months).[ncbi.nlm.nih.gov]
  • The duration of clinical illness may have been shortened by treatment with intravenous acyclovir.[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis appears to be excellent, although the aetiology has not been established. In the CSF so-called endothelial cells are a typical finding, but their classification is not yet clear.[ncbi.nlm.nih.gov]
  • While acute episodes may cause significant symptoms, the long-term prognosis is excellent.[ncbi.nlm.nih.gov]
  • Chapters focus on specific disorders or groups of disorders and emphasize differential diagnosis, disease course, treatment, and prognosis. This edition has a new chapter on mitochondrial cytopathies.[books.google.de]
  • Treatment and prognosis Although each attack is self limited, given the strong association with HSV-2 therapy and prophylaxis with acyclovir or valacyclovir has been proposed 2 .[radiopaedia.org]
  • Aciclovir if HSV is shown on PCR testing. [ 1 ] Prognosis Herpes simplex encephalitis is a rare but very serious condition with a mortality around 70% that usually leaves survivors with neurological deficits.[patient.info]

Etiology

  • No causative etiology was identified although a series of investigations were performed, including studies of the blood and CSF, roentgenogram and nuclear scintinographs. To date, there has been no standard therapeutic modality for this disease.[ncbi.nlm.nih.gov]
  • Polymerase chain reaction amplification for HSV DNA was done to further explore one possible etiology for this disease, but was negative.[ncbi.nlm.nih.gov]
  • Etiology The condition by definition is idiopathic. See the discussion on terminology above.[radiopaedia.org]
  • The most common etiologic agent is Herpes Simplex Virus type 2 (HSV-2) (2), however HSV-1 has been isolated in some cases and occasionally the etiology is unknown.(3-5) Early diagnosis may prevent prolonged hospital stay, and exposure to unnecessary medications[ojhas.org]

Epidemiology

  • Epidemiology It is very rare and the literature is very limited. Presentation There is a rapid onset of symptoms that are typical of meningitis - eg, fever, headache and neck stiffness. The symptoms last between one and seven days.[patient.info]
  • A positive epidemiological history was recorded for 5 patients.[doi.org]
  • The epidemiologic setting (e.g., time of year, geographic locale, exposure to insects, diseases prevalent in the local community) and accompanying systemic manifestations may be helpful in making a presumptive diagnosis.[emedicine.com]
  • Epidemiology. Ann Neurol 1984 ; 16 : 283 –94. Tyler K, Adler D. Twenty-eight years of benign recurring Mollaret meningitis. Arch Neurol 1983 ; 40 : 42 –3. Stoppe G, Stark E, Patzold U. Mollaret’s meningitis: CSF-immunocytological examinations.[jabfm.org]
Sex distribution
Age distribution

Pathophysiology

  • The possible pathophysiology of this case is discussed.[ncbi.nlm.nih.gov]
  • Provides in-depth coverage of CSF examination and analysis, CSF physiology and pathophysiology, approach to diagnosis, and future directions in CSF analysis.[books.google.de]
  • The pathophysiology of Mollaret meningitis remains an enigma, and the therapy consists of supportive care. ( JAMA 236:1967-1969, 1976) Full Text Download PDF Full Text Cite This Citation Haynes BF, Wright R, McCracken JP.[jamanetwork.com]

Prevention

  • Treatment with acyclovir may be beneficial in decreasing the severity and duration of attacks and in preventing further episodes. [Au?1].[ncbi.nlm.nih.gov]
  • For this patient, long-term suppressive and patient-initiated therapies were conducted to prevent the recurrence of meningitis.[ncbi.nlm.nih.gov]
  • Abstract Pierre Mollaret is mainly known for his contributions to infectious diseases and their prevention.[ncbi.nlm.nih.gov]
  • Collaborative Meta-Analysis of Randomised Trials of Antiplatelet Therapy for Prevention of Death, Myocardial Infarction, and Stroke in High Risk Patients. ‎[books.google.es]
  • The Neurologist who cares for me has told me that there is no cure for Mollarets Meningitis, all that can be done is to try and prevent me from becoming ill again.[healthunlocked.com]

References

Article

  1. Shalabi M, Whitley RJ. Recurrent benign lymphocytic meningitis. Clin Infect Dis. 2006;43(9):1194-1197.
  2. Kallio-Laine K, Seppänen M, Kautiainen H, et al. Recurrent Lymphocytic Meningitis Positive for Herpes Simplex Virus Type 2. Emerg Infect Dis. 2009;15(7):1119-1122.
  3. Poulikakos PJ, Sergi EE, Margaritis AS, et al. A case of recurrent benign lymphocytic (Mollaret's) meningitis and review of the literature. J Infect Public Health. 2010;3(4):192-195.
  4. Mirakhur B, McKenna M. Recurrent herpes simplex type 2 virus (Mollaret) meningitis. J Am Board Fam Pract. 2004;17(4):303-305.
  5. Abou-Foul AK, Buhary TM, Gayed SL. Herpes simplex virus type 2-associated recurrent aseptic (Mollaret’s) meningitis in genitourinary medicine clinic: a case report. Int Med Case Rep J. 2014;7:31-33.
  6. Mandell GL, Bennett JE, Dolin R. Mandel, Douglas and Bennett's Principles and Practice of Infectious Diseases. 8th ed. Philadelphia, Pennsylvania: Churchill Livingstone; 2015.

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Last updated: 2018-06-22 02:52