What is Valley Fever meningitis. How is coccidioidal meningitis diagnosed. What are the complications of untreated CM. How is coccidioidal meningitis treated. Why is lifelong treatment recommended for CM. How is patient response to therapy assessed.

Understanding Coccidioidal Meningitis: A Severe Complication of Valley Fever

Coccidioidal meningitis (CM) is a serious extrapulmonary complication of Valley Fever, caused by the spread of Coccidioides species to the central nervous system (CNS). This condition requires prompt diagnosis and treatment due to its potentially life-threatening nature.

Key Symptoms of Coccidioidal Meningitis

Patients with CM may experience various symptoms, including:

• Headache
• Blurry vision
• Photophobia
• Meningismus
• Cognitive decline
• Hearing changes
• Focal neurologic deficits

Diagnosing Coccidioidal Meningitis: Procedures and Findings

Diagnosing CM involves several procedures and analyses. How is coccidioidal meningitis diagnosed? The primary method is through a lumbar puncture and analysis of cerebrospinal fluid (CSF). This procedure is recommended for any patient with suspected or previously diagnosed coccidioidomycosis who presents with CNS-related symptoms.

CSF Analysis in Coccidioidal Meningitis

Typical CSF findings in CM include:

• Elevated white blood cell count with mixed or lymphocytic pleocytosis
• High protein levels (sometimes measurable in g/dL)
• Low glucose levels

The diagnosis is confirmed through positive serologic testing (ID/CF) or culture of CSF. Imaging studies can also be helpful in evaluating complications associated with meningitis.

The Importance of Timely Treatment: Consequences of Untreated CM

Untreated coccidioidal meningitis is invariably fatal. What are the consequences of untreated CM? Historical data from Vincent et al. showed that before the availability of antifungal treatments, all patients with CM died within 31 months. In combined statistics from five reports involving 117 patients, 91% died within one year, and all succumbed within two years.

Improved Outcomes with Modern Treatment

While the introduction of amphotericin B (AmB) and azoles has improved survival rates, morbidity remains substantial due to disease complications, treatment devices, and medication side effects.

Life-Threatening Complications of Coccidioidal Meningitis

CM can lead to several severe complications affecting the central nervous system. These include:

• Hydrocephalus
• CNS vasculitis
• Cerebral ischemia
• Infarction
• Vasospasm
• Hemorrhage
• Basilar meningitis
• Spinal cord involvement

Managing Hydrocephalus in CM Patients

Patients with hydrocephalus often require ventricular shunts for decompression. However, these shunts can lead to additional complications, including:

• Secondary infections
• Obstruction due to persistent coccidioidomycosis
• Abdominal pseudocysts

Multiple shunt revisions are not uncommon in CM patients. Repeated obstruction and fungal isolation may indicate the need for alternative antifungal therapy.

Treatment Approaches for Coccidioidal Meningitis

How is coccidioidal meningitis treated? The current preferred treatment for CM is oral fluconazole. While initial studies used a dose of 400 mg, most clinicians now start therapy with 800 to 1200 mg per day.

Historical Treatment: Amphotericin B

Before the advent of azoles, amphotericin B deoxycholate (AmB) was the only available treatment. However, it was ineffective when given intravenously and required frequent intrathecal (IT) administrations. Due to administration challenges and associated toxicity, AmB is rarely used as initial therapy today, although lipid formulations have been successfully used in salvage settings.

Comparing Treatment Efficacy

While there are no direct comparison trials between IT AmB and fluconazole, historical data shows:

• IT AmB response rates: 51-100% (studies before 1986)
• Fluconazole response rate: approximately 79%

Duration of Treatment and Patient Response

With fluconazole treatment, symptoms typically resolve within 4-8 months. However, CSF abnormalities may persist, especially in the presence of a shunt. Why is lifelong treatment recommended for CM? Due to an extremely high relapse rate (78% in a small series) when therapy is discontinued, lifelong treatment with azoles is recommended.

Assessing Treatment Response

How is patient response to therapy assessed? Evaluating a patient’s response to therapy primarily involves serial evaluation and clinical judgment. Favorable signs include:

• Return to premorbid functioning
• Decreasing CF titers
• Excellent adherence to medical care and therapy

Some patients with chronic meningitis may experience refractory illness with poor recovery or exceptionally slow improvement. In such cases, a combination of serology and repeated CSF evaluation may be necessary to assess microbiologic and serologic improvement.

Challenges in Managing Coccidioidal Meningitis

Managing CM presents several challenges for healthcare providers and patients alike. These include:

1. Long-term medication adherence
2. Monitoring for drug side effects
3. Managing complications such as hydrocephalus
4. Assessing treatment efficacy over time
5. Balancing quality of life with necessary interventions

Adherence counseling plays a crucial role in ensuring patients maintain their treatment regimen, given the lifelong nature of therapy required for most CM cases.

Emerging Research and Future Directions in CM Treatment

While current treatment options have significantly improved outcomes for CM patients, ongoing research continues to explore new avenues for more effective and less toxic therapies. Areas of focus include:

• Novel antifungal agents with better CNS penetration
• Immunomodulatory therapies to enhance the body’s response to infection
• Improved drug delivery methods to reduce systemic side effects
• Biomarkers for earlier diagnosis and treatment response monitoring

These research directions hold promise for further improving the prognosis and quality of life for patients with coccidioidal meningitis.

The Role of Prevention in Combating Coccidioidal Meningitis

Given the severity of CM and the challenges associated with its treatment, prevention plays a crucial role in combating this condition. Preventive measures focus on reducing exposure to Coccidioides spores in endemic areas. These may include:

• Avoiding activities that generate dust in areas where the fungus is prevalent
• Using air filtration systems in buildings located in endemic regions
• Wearing appropriate protective equipment when working in high-risk environments
• Educating at-risk populations about the signs and symptoms of Valley Fever

Early recognition and treatment of primary coccidioidomycosis may also help prevent progression to meningeal involvement.

The Importance of Ongoing Research

Continued research into the epidemiology, pathogenesis, and treatment of coccidioidal meningitis is essential for improving patient outcomes. This includes:

1. Developing more accurate diagnostic tools
2. Identifying risk factors for CNS dissemination
3. Exploring potential vaccine candidates
4. Investigating combination therapies for refractory cases
5. Studying long-term outcomes of patients on lifelong antifungal therapy

By advancing our understanding of CM, we can work towards more effective prevention strategies and treatment options for this serious complication of Valley Fever.

Patient Education and Support in Coccidioidal Meningitis Management

Managing coccidioidal meningitis requires a comprehensive approach that extends beyond medical treatment. Patient education and support play crucial roles in ensuring optimal outcomes. Key aspects of patient care include:

Understanding the Disease and Treatment

Patients and their families should be thoroughly educated about:

• The nature of coccidioidal meningitis
• The importance of adherence to long-term therapy
• Potential side effects of medications
• Signs of disease progression or treatment failure

Psychological Support

Living with a chronic condition like CM can take a toll on mental health. Providing access to psychological support services can help patients cope with:

• Anxiety and depression related to chronic illness
• Adjustment to lifestyle changes
• Stress management techniques

Support Groups and Resources

Connecting patients with support groups and resources can provide valuable peer support and practical assistance. These may include:

• Online forums for patients with Valley Fever and its complications
• Local support groups for individuals with chronic neurological conditions
• Resources for managing daily life with a chronic illness

By addressing the multifaceted needs of patients with coccidioidal meningitis, healthcare providers can help ensure better quality of life and improved treatment outcomes.

Coccidioidal Meningitis | UC Davis Center for Valley Fever

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3. About Valley Fever

The most deleterious extrapulmonary dissemination is the spread of Coccidioides spp. to the central nervous system (CNS) causing meningitis. A lumbar puncture with analysis of cerebrospinal fluid (CSF) should be done in any patient with suspected or previously diagnosed coccidioidomycosis presenting with a headache, blurry vision, photophobia, meningismus, decline in cognition, hearing changes, and focal neurologic deficit. As illustrated in a recent retrospective study there is no evidence to support routine CSF analysis in patients in at-risk groups (age, ethnicity, CF titer, etc. ) if they do not have CNS symptoms (1). The diagnosis of CM is based on a positive serologic testing (ID/CF) or culture of CSF. CSF analysis typically shows an elevated white blood cell count with a mixed or lymphocytic pleocytosis, a high level of protein (sometimes measurable in g/dL rather than mg/dL), and a low level of glucose. Imaging studies are helpful in evaluating complications associated with meningitis. Initial features of illness may be difficult to distinguish from other etiologies without detailed testing, notably tuberculosis and even autoimmune illnesses.

When left untreated, CM is uniformly fatal (2). In a historical series reported by Vincent et al, before the availability of antifungals, seventeen patients with CM were followed all of whom died within 31 months (2). This review also commented on the combined survival statistics described in five reports of 117 patients where 91% of patients with CM died within one year and all died within 2 years. Though the fatality has improved with the use of AmB and azoles, morbidity is still substantial due to complications from the disease, devices used for treatment management, and side effects of the medications as much higher recommended doses are necessary for prolonged period of time (3).

The most common life-threatening complications of meningitis include hydrocephalus, CNS vasculitis, cerebral ischemia, infarction, vasospasm and hemorrhage. Basilar meningitis and spinal cord involvement may also be encountered. In patients with hydrocephalus, a ventricular shunt is necessary for decompression. Such shunts, often placed distally into the abdominal cavity may develop secondary infections, obstruction due to persistent coccidioidomycosis, and/or abdominal pseudocysts (4). It is not uncommon for patients to require multiple shunt revisions. As illustrated in several case reports, repeated obstruction of the shunt and isolation of fungus should alert one to seek alternate antifungal therapy. Some clinicians have used steroids for vasculitis though this is considered anecdotal.

For treatment of CM, most clinicians prefer therapy with oral fluconazole (5). Although the dose studied in an uncontrolled clinical trial was 400 mg, it is common to begin therapy with 800 to 1200 mg per day of fluconazole (3, 6). Prior to the advent of azoles, amphotericin B deoxycholate (AmB) was the only drug of choice but was ineffective when given intravenously and required frequent administrations via the intrathecal (IT) route. Due to challenges of administration, toxicity associated with this route and lack of experience in utilizing this method, current practitioners seldom resort to recommending AmB as initial therapy although lipid formulations have been used in the salvage setting successfully (7). Though there are no trials comparing IT AmB and fluconazole, the response rate of IT AmB has ranged from 51%-100% in studies published before 1986 and with fluconazole the rate is near 79% (6, 8). With fluconazole symptoms resolve within 4-8 months though there is a delay in normalization of CSF abnormalities which may persist in the presence of a shunt. Based on clinical experience and due to an extremely high relapse of 78% noted in a small series when therapy is discontinued, lifelong treatment with azoles is recommended (9).

Assessing a patient’s response to therapy is primarily a matter of serial evaluation and clinical judgment. Favorable signs include return to premorbid functioning, decreasing CF titers, and excellent adherence to medical care and therapy. Some patients with chronic meningitis have refractory illness with poor recovery or exceptionally slow improvement. A combination of serology and repeated CSF evaluation may be necessary to assess microbiologic and serologic improvement. Adherence counseling, assessment of drug-drug interactions, therapeutic drug monitoring, and consideration of alternative antifungal therapy may be necessary. For CM patients who are failing treatment and/or have refractory coccidioidal disease, salvage regimens may be necessary. Both voriconazole and posaconazole have been used in this situation, with a growing body of case series and clinical experience to support their use.

REFERENCES

1. Thompson G, 3rd, Wang S, Bercovitch R, et al. Routine CSF Analysis in Coccidioidomycosis Is Not Required. PloS one 2013; 8(5): e64249.
2. Vincent T, Galgiani JN, Huppert M, Salkin D. The natural history of coccidioidal meningitis: VA-Armed Forces cooperative studies, 1955-1958. Clin Infect Dis 1993; 16(2): 247-54.
3. Johnson RH, Einstein HE. Coccidioidal meningitis. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America 2006; 42(1): 103-7.
4. Hibbett DS, Binder M, Bischoff JF, et al. A higher-level phylogenetic classification of the Fungi. Mycol Res 2007; 111(Pt 5): 509-47.
5. Galgiani JN, Ampel NM, Blair JE, et al. Coccidioidomycosis. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America 2005; 41(9): 1217-23.
6. Galgiani JN, Catanzaro A, Cloud GA, et al. Fluconazole therapy for coccidioidal meningitis. The NIAID-Mycoses Study Group. Annals of internal medicine 1993; 119(1): 28-35.
7. Mathisen G, Shelub A, Truong J, Wigen C. Coccidioidal meningitis: clinical presentation and management in the fluconazole era. Medicine 2010; 89(5): 251-84.
8. Bouza E, Dreyer JS, Hewitt WL, Meyer RD. Coccidioidal meningitis. An analysis of thirty-one cases and review of the literature. Medicine 1981; 60(3): 139-72.
9. Dewsnup DH, Galgiani JN, Graybill JR, et al. Is it ever safe to stop azole therapy for Coccidioides immitis meningitis? Annals of internal medicine 1996; 124(3): 305-10.

Valley fever – Symptoms & causes

Overview

Valley fever is a fungal infection caused by coccidioides (kok-sid-e-OY-deze) organisms. It can cause signs and symptoms such as a fever, cough and tiredness.

Two coccidioides fungi species cause valley fever. These fungi are commonly found in soil in specific regions. The fungi’s spores can be stirred into the air by anything that disrupts the soil, such as farming, construction and wind.

People can then breathe the fungi into their lungs. The fungi can cause valley fever, also known as acute coccidioidomycosis (kok-sid-e-oy-doh-my-KOH-sis). Mild cases of valley fever usually resolve on their own. In more-severe cases, doctors treat the infection with antifungal medications.

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Symptoms

Valley fever is the initial form of coccidioidomycosis infection. This initial, acute illness can develop into a more serious disease, including chronic and disseminated coccidioidomycosis.

Acute coccidioidomycosis (valley fever)

The initial, or acute, form of coccidioidomycosis is often mild, with few or no symptoms. Signs and symptoms occur one to three weeks after exposure. They tend to be similar to flu symptoms. Symptoms can range from minor to severe, including:

• Fever
• Cough
• Tiredness
• Shortness of breath
• Headache
• Chills
• Night sweats
• Joint aches and muscle soreness
• Red, spotty rash, mainly on lower legs but sometimes on the chest, arms and back

If you don’t become ill or have symptoms from valley fever, you may only find out you’ve been infected later. You may find out when you have a positive skin or blood test or when small areas of residual infection in the lungs (nodules) show up on a routine chest X-ray. The nodules typically don’t cause problems, but they can look like cancer on X-rays.

If you develop symptoms, especially severe ones, the course of the disease is highly variable. It can take months to fully recover. Fatigue and joint aches can last even longer. The disease’s severity depends on several factors, including your overall health and the number of fungus spores you inhale.

Chronic coccidioidomycosis

If the initial coccidioidomycosis infection doesn’t completely resolve, it may progress to a chronic form of pneumonia. This complication is most common in people with weakened immune systems.

Signs and symptoms include:

• Low-grade fever
• Weight loss
• Cough
• Chest pain
• Blood-tinged sputum (matter discharged during coughing)
• Nodules in the lungs

Disseminated coccidioidomycosis

The most serious form of the disease, disseminated coccidioidomycosis, is uncommon. It occurs when the infection spreads (disseminates) beyond the lungs to other parts of the body. Most often these parts include the skin, bones, liver, brain, heart, and the membranes that protect the brain and spinal cord (meninges).

Signs and symptoms of disseminated disease depend on the body parts affected and may include:

• Nodules, ulcers and skin lesions that are more serious than the rash that sometimes occurs with initial infection
• Painful lesions in the skull, spine or other bones
• Painful, swollen joints, especially in the knees or ankles
• Meningitis — an infection of the membranes and fluid surrounding the brain and spinal cord

When to see a doctor

Seek medical care if you are over 60, have a weakened immune system, are pregnant, or are of Filipino or African heritage, and you develop the signs and symptoms of valley fever, especially if you:

• Live in or have recently traveled to an area where this disease is common
• Have symptoms that aren’t improving

Be sure to tell your doctor if you’ve traveled to a place where valley fever is common and you have symptoms.

Causes

Valley fever is caused by a person inhaling spores of certain fungi. The fungi that cause valley fever — Coccidioides immitis or Coccidioides posadasii — live in the soil in parts of Arizona, Nevada, Utah, New Mexico, California, Texas and Washington. It’s named after the San Joaquin Valley in California. The fungi can also often be found in northern Mexico and Central and South America.

Like many other fungi, coccidioides species have a complex life cycle. In the soil, they grow as a mold with long filaments that break off into airborne spores when the soil is disturbed. A person can then inhale the spores.

The spores are extremely small and can be carried far by the wind. Once inside the lungs, the spores reproduce, continuing the disease cycle.

Risk factors

Risk factors for valley fever include:

• Environmental exposure. Anyone who inhales the spores that cause valley fever is at risk of infection. People who live in areas where the fungi are common — especially those who spend a lot of time outdoors — have a greater risk.

  Also, people who have jobs that expose them to dust are most at risk — construction, road and agricultural workers, ranchers, archaeologists, and military personnel on field exercises.

• Race. For reasons that aren’t well understood, people of Filipino and African heritage are more susceptible to developing serious fungal infections.
• Pregnancy. Pregnant women are vulnerable to more-serious infections when they get the infection during the third trimester. New mothers are vulnerable right after their babies are born.
• Weakened immune system. Anyone with a weakened immune system is at increased risk of serious complications. This includes people living with acquired immunodeficiency syndrome (AIDS) or those being treated with steroids, chemotherapy and anti-rejection drugs after transplant surgery. People with certain autoimmune diseases, such as rheumatoid arthritis or Crohn’s disease, who are being treated with anti-tumor necrosis factor (TNF) drugs also have an increased risk of infection.
• Diabetes. People with diabetes may have a higher risk of severe lung infections.
• Age. Older adults are more likely to develop valley fever. This may be because their immune systems are less robust or because they have other medical conditions that affect their overall health.

Complications

Some people, especially pregnant women, people with weakened immune systems — such as those living with human immunodeficiency virus (HIV)/AIDS — and those of Filipino or African heritage are at risk of developing a more severe form of coccidioidomycosis.

Complications of coccidioidomycosis may include:

• Severe pneumonia. Most people recover from coccidioidomycosis-related pneumonia without complications. Others, such as people of Filipino and African heritage, and those with weakened immune systems, may become seriously ill.
• Ruptured lung nodules. A small percentage of people develop thin-walled nodules (cavities) in their lungs. Many of these eventually disappear without causing any problems, but some may rupture, causing chest pain and difficulty breathing. A ruptured lung nodule might require the placement of a tube into the space around the lungs to remove the air or surgery to repair the damage.
• Disseminated disease. This is the most serious complication of coccidioidomycosis but it’s uncommon. If the fungus spreads (disseminates) throughout the body, it can cause problems including skin ulcers, abscesses, bone lesions, severe joint pain, heart inflammation, urinary tract problems and meningitis — a potentially fatal infection of the membranes and fluid covering the brain and spinal cord.

Prevention

There is no vaccine to prevent valley fever.

If you live in or visit areas where valley fever is common, take common-sense precautions, especially during the dry season following a rainy season when the chance of infection is highest.

Consider these tips:

• Wear a mask.
• Avoid very dusty areas, such as construction sites.
• Stay inside during dust storms.
• Wet the soil before digging in it, or avoiding soil if you’re at higher risk of infection.
• Keep doors and windows tightly closed.
• Clean skin injuries with soap and water.

US drought resurrects fungus that causes fever and meningitis

09/02/2022 at 19:15

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Photo: Global Look Press

Fires, floods and the record sting of 2022 certainly pose a great danger to human health. But dry weather in the United States in California has brought to life a disease-causing fungus that spreads by airborne droplets.

It’s called Coccidioides, and it causes Valley Fever. Its symptoms are crushing headaches and sinus infections. Worst of all, it can turn into meningitis.

Former financial planner and father of two, Rob Purry, first contracted a fever and then contracted meningitis. He complained that the illness had taken away all his health and all the money the family had.

Coccidioides requires a dry and hot climate to survive. Therefore, the disease, originally characteristic of the hot plains of California, is becoming more common. This was announced by infectious disease expert Dr. Royce Johnson.

Most of the western US is already in drought, added Morgan Gorris, an Earth system researcher at Los Alamos National Laboratory.

“The western half of the US is still expected to be fairly dry and Valley Fever is expected to spread,” he predicted.

It is important to note that soil, if left undisturbed, will not necessarily lead to inhalation of the disease. But any disturbance to the layer, whether it be a full-scale archaeological site or a burrowing animal, will release the fungus from the soil. Pathogenic spores can travel up to 75 miles (120 km).

Scientists recently warned that the next global pandemic could be fungal. Therefore, the growing spread of Coccidioides is of particular concern.

See also: “The number of cases of monkeypox infection in the world has exceeded 50 thousand”

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  Anastasia Kukova

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“I lost everything”: a debilitating disease that can be caught out of thin air in California

The disease that changed Rob Purdy’s life began with a severe headache – the pain first appeared on New Year’s Eve in 2012 and lasted for several months, says The Guardian.

It wasn’t until several emergency room visits, several doctors, and misdiagnosis that he realized what was wrong with him. A Bakersfield, California resident had meningitis caused by valley fever. This disease, also called coccidioidosis, is caused by the fungus Coccidioides, endemic to the soil of the southwestern United States. The diagnosis was followed by years of debilitating illness, struggles to find effective treatments, and other difficulties.

“It took everything: my health,” Purdy said. “It had a huge impact on my family. We lost everything, our financial security, our entire pension.”

A father-of-two is among the small percentage of people who develop severe forms of valley fever—most do not become ill after infection, and very few have severe symptoms. But for those who develop the chronic form of the disease, it can be devastating.

Valley fever is afflicting California’s Central Valley, and experts say the number of cases could increase in the American west in the future as the climate crisis makes the landscape drier and hotter. Kern County, north of Los Angeles at the end of the Central Valley, has reported a significant increase over the past decade. The county where Purdy lives had about 1,000 cases in 2014 and more than 3,000 in 2021.

Testing and awareness of fever have improved in recent years, but there has also been a significant rise in incidence, said Dr. Royce Johnson, medical director of the Valley Fever Institute in Bakersfield. He attributes this to climate and weather: the fungus needs dry and hot conditions.

The fungus grows in the mud as a thread that segments, breaks off and flies up to 75 miles – even sea otters have been infected with it. People can contract valley fever by digging into undisturbed soil or by simply breathing in the air. Those who work outdoors are at greater risk. Approximately 40% develop the disease, which can be mild, and 1% develop severe consequences.

According to the latest available data, there were about 20,000 cases of valley fever reported to the CDC in 2019 in the US, Arizona, and California, and an average of about 200 related deaths each year from 1999 to 2019.