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].
Entire Body System
- 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]
Diagnosis and pathophysiology Clinical presentation is similar to bacterial meningitis with headache, fever, and positive meningeal signs. [cureus.com]
The fever and symptom gradually improved after admission of indomethacin, and he no longer complained any headache or fever after Day 7 ( Figure 1 ). [omicsonline.org]
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]
- Asymptomatic
Attacks that last several days, may be accompanied by generalized myalgia, and are separated by asymptomatic periods lasting weeks or months; 3. [omicsonline.org]
It is reasonable to obtain an MRI when patients are asymptomatic to ensure that no other abnormality is present which may be causing a recurrent chemical meningitis (e.g. intracranial epidermoid cyst 4 or neurenteric cyst 5 ) which have been reported [radiopaedia.org]
Do not kiss or share drinks, eating utensils or chap-stick with individuals who have active cold sores (or with anyone who has not been tested for HSV since it can be asymptomatic). [stdcheck.com]
- Varicella-Zoster Virus Infection
The herpes simplex virus is believed to play a role in the causation of Mollaret meningitis. Cases of Mollaret's resulting from Varicella zoster virus infection have been documented. [raredr.com]
[…] this problem with herpes simplex viruses, which cause cold sores and genital herpes. [6] [8] Cases of Mollaret's resulting from Varicella zoster virus infection, diagnosed by polymerase chain reaction ( PCR ), have been documented. [forums.phoenixrising.me]
"Clinical Features of Viral Meningitis in Adults: Significant Differences in Cerebrospinal Fluid Findings among Herpes Simplex Virus, Varicella Zoster Virus, and Enterovirus Infections". [en.wikipedia.org]
- Epilepsy
Restless legs - Stiff person Other degenerative / demyelinating diseases Alzheimer's - Pick's - Alpers' - Dementia with Lewy bodies - Leigh's demyelinating: Multiple sclerosis - Devic's - Central pontine myelinolysis - Transverse myelitis Seizure / epilepsy [wikidoc.org]
[…] reflexes clumsiness coordination problems loss of balance residual headaches hearing problems tinnitus speech problems dizziness learning difficulties (ranging from temporary learning deficiencies all the way to long term mental impairment) tachycardia epilepsy [recurrentmeningitis.org]
These can include: hearing loss or vision loss, which may be partial or total problems with memory and concentration recurrent seizures (epilepsy) co-ordination, movement and balance problems loss of limbs – amputation of affected limbs is sometimes necessary [nhs.uk]
[…] clumsiness, coordination problems, loss of balance, residual headaches, hearing problems, tinnitus, speech problems, dizziness, learning difficulties (ranging from temporary learning deficiencies all the way to long term mental impairment), tachycardia, epilepsy [mollaretsmeningitis.org]
- Collapse
I was in my second day of the Sheriff’s academy in San Bernardino County, California, when I collapsed in class and was rushed to the Emergency Department. They did a lumbar puncture and confirmed viral meningitis. [globalgenes.org]
I was in my second day of the Sheriff’s academy when I collapsed in the classroom and had to go to the emergency room. You can read my full meningitis story here. [michaelhrehor.com]
However, evidence of this phenomenon can only be found on neuroimaging (CT head with contrast or nuclear magnetic resonance imaging of brain) when asymptomatic as these cysts collapse after releasing squamous material that precipitates attacks. [ncbi.nlm.nih.gov]
Cardiovascular
- Tachycardia
Symptoms Symptoms of a full-blown recurrent viral meningitis (Mollaret’s) infection include: severe headaches fever (only seen in about half of cases) nausea vomiting sensitivity to light and/or sound malaise neck rigidity Rarer symptoms include: tachycardia [recurrentmeningitis.org]
Rarer symptoms include tachycardia, double vision and hallucinations. Because of the similarity in symptoms to benign migraine headache, sufferers of Mollaret’s have often been misdiagnosed by medical personnel. [mollaretsmeningitis.org]
The symptoms of a full-blown viral meningitis attack are: Severe headaches Fever Nausea Vomiting Sensitivity to light and/or sound Malaise Neck rigidity Rarer symptoms are: Tachycardia Double Vision Hallucinations Without proper care, symptoms can escalate [michaelhrehor.com]
Musculoskeletal
- Arthralgia
Presentation is usually with fever, myalgia and arthralgia and signs and symptoms of meningism, including neck-stiffness, nausea and vomiting, photophobia, although these are variable 1,2. Etiology The condition by definition is idiopathic. [radiopaedia.org]
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 [symptoma.com]
Arthralgias (n = 6) and urinary dysfunction (n = 5) were common. In 56 (61%) of the 95 episodes, case-patients received antiviral medication to alleviate symptoms. [wwwnc.cdc.gov]
Skin
- Skin Rash
• A distinctive skin rash (although not everyone will have this). What are the differences between viral and bacterial meningitis? Bacterial meningitis is the more dangerous of the two kinds of the illness. [express.co.uk]
The patient had no other chronic medical illnesses and no history of arthralgia, recurrent skin rash, or intravenous drug use. She had oral herpetic lesions as a child but no history of genital herpetic lesions or sexually transmitted diseases. [nejm.org]
- Eruptions
The attacks were often triggered by emotional stress or heat and were sometimes preceded by genital herpetic eruptions, for which he was referred to our clinic. [ncbi.nlm.nih.gov]
Dermatological herpes: Herpes dermatitis: seen in athletes (herpes gladiatorum), health care workers (herpetic whitlow) and in patients with eczema who become superinfected with HSV (Kaposi’s varicelliform eruption). [hopkinsguides.com]
Eyes
- 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]
CASE REPORT A 35-year-old Caucasian woman developed acute intractable headache, fever, neck pain, photophobia, and binocular horizontal diplopia. She had morbid obesity (body mass index of 47.5 kg/m2). [journals.lww.com]
However, transient neurologic abnormalities (seizures, diplopia, pathologic reflexes, cranial nerve paresis, hallucinations, and coma) occur in as many as 50% of cases. [lifeextensionvitamins.com]
- Visual Impairment
Many people have side effects between bouts that vary from chronic daily headaches to after-effects from meningitis such as hearing loss and visual impairment, nerve pain and twitches. [en.wikipedia.org]
- Visual Impairment
Many people have side effects between bouts that vary from chronic daily headaches to after-effects from meningitis such as hearing loss and visual impairment, nerve pain and twitches. [en.wikipedia.org]
Urogenital
- Genital Lesions
Herpes simplex virus type 2 meningitis in the absence of genital lesions: improved recognition with use of the polymerase chain reaction. Clin Infect Dis.1995;20(4):842-848. 3. Achard JM, Duverlie G, Schmit JL, Lebon P, Veyssier P, Fournier A. [cogprints.org]
Symptoms Fever blisters Fever — especially during the first episode Genital lesions — there may first be a burning or tingling sensation Blisters or ulcers — most often on the mouth, lips and gums, or genitals Enlarged lymph nodes in the neck or groin [powerofpain.org]
However, most patients with MM do not present with skin or genital lesions at the onset of their illness, and furthermore, many do not report previous genital infections (4,6,8). [journals.lww.com]
Herpes simplex virus type 2 meningitis in the absence of genital lesions: improved recognition with use of the polymerase chain reaction. Clin Infect Dis. 1995;20(4):842-848. Achard JM, Duverlie G, Schmit JL, Lebon P, Veyssier P, Fournier A. [ojhas.org]
Neurologic
- Meningism
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]
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]
- 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]
One year after the first infection, he developed severe recurrent attacks of headache associated with meningitis symptoms. [ncbi.nlm.nih.gov]
There is no specific treatment, so management involves supportive measures such as rest, fluids, and medicine to relieve the fever or headache.” [recurrentmeningitis.org]
Slide of a sample of cerebrospinal fluid (CSF) from a 41-year-old woman with headache, fever and nuchal rigidity. [cmaj.ca]
- 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
A physical examination that will include a comprehensive neurological examination may show a fever and neck stiffness. Sometimes neck stiffness can be more severe when you flex your neck or your legs. [verywellhealth.com]
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 of Mollaret meningitis, which last from 1 to 7 days, include headache, neck ache, fever, and neck stiffness. [rxlist.com]
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]
- Kernig's Sign
On her current admission, she had no focal neurological signs apart from nuchal rigidity. Kernig's sign was elicited. [ispub.com]
Kernig’s sign is the inability to straighten the leg when the hips are flexed to a 90-degree angle; Brudzinski’s sign is the spontaneous flexion of the hips and knees during attempted flexion of the neck. [uspharmacist.com]
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].
Cytology
- Cerebrospinal Fluid Abnormality
fluid", [2] cerebrospinal 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]
fluid",[2] cerebrospinal 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. [en.wikipedia.org]
Treatment
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]
Prognosis
While acute episodes may cause significant symptoms, the long-term prognosis is excellent. [ncbi.nlm.nih.gov]
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]
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]
Etiology The condition by definition is idiopathic. See the discussion on terminology above. [radiopaedia.org]
Epidemiology
A positive epidemiological history was recorded for 5 patients. [doi.org]
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]
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]
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]
Pathophysiology Meningitis is inflammation of the meninges. The meninges are three layers of connective tissue that surround and protect the brain and spinal cord. [verywellhealth.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]
Collaborative Meta-Analysis of Randomised Trials of Antiplatelet Therapy for Prevention of Death, Myocardial Infarction, and Stroke in High Risk Patients. [books.google.es]
Preventing HSV infections include: Abstinence or only having sex with one partner who has been tested and does not have the virus. Using condoms, which can help reduce risk –but not necessarily prevent–herpes infection. [stdcheck.com]
References
- Shalabi M, Whitley RJ. Recurrent benign lymphocytic meningitis. Clin Infect Dis. 2006;43(9):1194-1197.
- 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.
- 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.
- Mirakhur B, McKenna M. Recurrent herpes simplex type 2 virus (Mollaret) meningitis. J Am Board Fam Pract. 2004;17(4):303-305.
- 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.
- Mandell GL, Bennett JE, Dolin R. Mandel, Douglas and Bennett's Principles and Practice of Infectious Diseases. 8th ed. Philadelphia, Pennsylvania: Churchill Livingstone; 2015.