Rare Emergent Neurological Conditions - Recognition and Treatment

Introduction

The healthcare providers from the emergency department (ED) encounter many emergency conditions which are common such as status epilepticus (seizure that lasts for a prolonged period or a series of seizures without interictal recovery), strokes (interruption of blood flow to the brain), or intracranial hemorrhage (bleeding in the skull). Apart from these, there are rare neurological conditions that are also encountered which require immediate attention because of their progressive ill effects. Read the below article to know more about these conditions along with their diagnosis and management.

What Are Rare Emergent Neurological Conditions?

Rare emergent neurological conditions are conditions that require immediate medical attention since they progress rapidly, cause complications, and have life-threatening consequences. Some rare emergent neurological conditions are:

1. Opsoclonus Myoclonus Syndrome (OMS): A rare neurological condition with two main features, which are opsoclonus, the rapid involuntary movement of the eyes, also called the dancing eyes, and myoclonus in which there are sudden involuntary muscle twitches or jerks. Apart from these, these individuals may also have sleep disturbances, irritability, and behavioral changes.

2. Fahr Disease: It is also called idiopathic basal ganglia calcification, which causes deposits of calcium in certain parts of the brain, especially in the basal ganglia. This condition can also occur due to underlying autoimmune conditions or infections. Sometimes it may also be related to genetic mutations. The symptoms include abnormality in the movements, tremors, dystonia (muscle contractions that are involuntary), seizures, and psychiatric symptoms.

3. Locked-in Syndrome: It is a condition that causes paralysis of voluntary muscles, including speech. This occurs due to damage to the brainstem due to brainstem tumors, hemorrhage, stroke, or injury. Therefore, these individuals undergo complete paralysis but consciousness and cognitive functions are preserved.

4. Acute Hemorrhagic Leukoencephalitis (AHLE): It is a severe condition that causes bleeding and inflammation in the white matter of the brain. It often occurs due to bacterial or viral infection or an autoimmune response from the infection. The patient has symptoms such as seizures, headaches, altered consciousness, and difficulty in speaking, and it has a rapidly progressive course.

5. Cauda Equina Syndrome (CES): This condition occurs when the cauda equina (bundle of nerves at the end of the spinal cord) becomes damaged. The symptoms include lower back pain, radiculopathy (compression of the root, which causes pain in the buttock, legs, and thighs), muscle weakness, sensory disturbances, sexual dysfunction, and dysfunction in the bladder.

6. Spinal Cord Compression: This occurs when there is compression on the spinal cord and can be caused due to herniated disc (a ruptured disc), spinal fracture, spinal tumor, spinal stenosis (narrow spinal canal), or infections such as rheumatoid arthritis (an autoimmune disorder that affects the joints). The symptoms include pain in the neck or back, paralysis in the limb, muscle weakness, altered sensation, sexual dysfunction, and loss of bladder and bowel control.

7. Acute Transverse Myelitis (ATM): A condition that causes inflammation of the spinal cord. It can be due to immune-mediated disorders due to bacterial infections or autoimmune disorders. It causes sensory disturbances, muscle weakness, dysfunction in the bowel and bladder, localized or radiating pain, and autonomic dysfunctions such as changes in blood pressure, abnormal sweating, or abnormal heart rate.

8. Acute Bacterial Meningitis: It is a severe infection that causes inflammation of the protective layers of the brain and spinal cord called meninges. It can be due to bacterial infections such as Neisseria meningitidis, Streptococcus pneumoniae, or Haemophilus influenzae type B. The symptoms include headache, fever, neck stiffness, photophobia, vomiting, nausea, altered mental status, and skin rashes.

How to Recognize Rare Emergent Neurological Conditions?

The recognition of rare emergency neurological conditions depends on the specific condition and may require a unique approach. A multidisciplinary approach should be used to diagnose the conditions accurately. In general, the diagnosis includes:

• Obtaining Medical History: It is essential to obtain a complete medical history, such as a history of medications, family history, previous surgeries, and any recent illness.

• Physical Examination: A thorough physical examination should be assessed, such as vital signs and neurological conditions such as motor function, reflexes, sensory perception, mental status, seizures, headache, visual changes, cranial nerve function, and coordination.

• Neuroimaging: Imaging tests such as CT (computed tomography), and MRI (magnetic resonance imaging), can be used to visualize the spinal cord, brain, and surrounding structures.

• Laboratory Tests: Tests such as blood tests, examination of specific biomarkers, and cerebrospinal fluid analysis can be done, which can help in identifying infections, metabolic disorders, autoimmune markers, and genetic abnormalities.

• Electrophysiological Studies: This includes the evaluation of heart activities such as electroencephalography (EEG), and electromyography (EMG). These can also be used in the evaluation of muscles, nerves, and the brain. Therefore, these help in the diagnosis of peripheral neuropathies (damage of nerves outside the spinal cord and brain).

• Genetic Testing: In some conditions, identification of genetic mutation helps in the diagnosis of the disorder and can further help in treatment planning.

How to Treat Rare Emergent Neurological Conditions?

The treatment of rare emergent neurological conditions depends on the specific condition, but in general, the treatment approach includes

• Supportive Care: This includes providing adequate oxygenation, addressing pain and discomfort, and maintaining hemodynamic stability (maintaining blood flow). Apart from these, the vital signs should be monitored, management of fluids, hydration, and nutrition.

• Medications: This includes the use of anti-inflammatory drugs, antiepileptic drugs, immunosuppressants, and pain medications.

• Neurosurgical Interventions: In some cases, neurosurgical interventions should be done, such as tumor resection, decompressive craniectomy (a procedure in which a portion of the skull is removed for the expansion of the swelling), and evacuation of hematoma or abscess

• Immune Therapies: This is indicated in the case of autoimmune disorders to reduce the immune response. The therapies include plasmapheresis (exchange of blood plasma), intravenous immunoglobulin (IVIG, which includes a combination of antibodies), and immunomodulatory medications (help in modifying the immune system).

• Rehabilitation and Physical Therapy: This phase is important in the recovery of the functions of the body and quality of life. Therapies such as occupational therapy, physical therapy, and speech therapy can help in restoring motor and cognitive function,

• Genetic Counseling and Management: Some conditions may have an inheritance pattern. Therefore the family members should be educated about the condition and guide family planning.

Conclusion

The recognition and treatment of rare emergent neurological conditions require a patient-centered approach and should be tailored to the specific needs of the patient. Early recognition, accurate diagnosis, and swift intervention help in better outcomes for individuals with these conditions. Because of the complexity and rare conditions, a multidisciplinary approach is recommended and should involve health professionals such as neurosurgeons, neurologists, rehabilitation specialists, critical care specialists, and geneticists.