pseudomyelitisation



Overview

A disorder of the brain associated with dysfunctional emotional expression characterized by frequent, involuntary, and uncontrollable crying or laughing, often at the wrong time, often associated with neurologic disease or injury, with medication as the primary treatment.

Definition

Pseudomyelitis optica is a condition characterized by frequent, sudden, uncontrollable, and inappropriate laughing or crying, and is a brain disorder associated with dysfunctional emotional expression.

Pseudomyelitis is usually found in neurological disorders or injuries that may affect the brain’s control of emotions, and is characterized by uncontrollable, sudden, and particularly intense crying or laughing that is unrelated to emotion, and laughing that cannot be stopped when feeling sad.

Pseudo medullary effects have limited therapeutic efficacy [1].

Morbidity

There is not much epidemiological information available on pseudo-extended medulla oblongata because it is difficult for people to realize that they or their family members and friends have pseudo-extended medulla oblongata, or to mistake it for depression or other mood disorders.

The number of people with the disease in the United States is speculated to be around 1.5 million abroad, but the true number of people with the disease may be higher [2].

One study reported that about 36.7% or so of patients with neurological disorders experience pseudo-delta effect [3].

Etiology

Causes of the disease

Pseudo medullary effect is usually caused by damage to the cortex of the motor areas of the brain and the associated cortical brainstem tracts following a traumatic brain injury or disease, such as stroke [1,4].

Stroke.

After the occurrence of cerebrovascular diseases such as stroke, when the lesion involves the medullary tracts bilaterally can lead to a pseudo medullary effect.

Multiple sclerosis

Multiple sclerosis is a white matter demyelinating disease of the central nervous system that is influenced by the role of genetically susceptible individuals and environmental factors. Involvement of the bilateral medullary tracts can lead to a pseudomyelinating effect.

Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis lesions do not usually involve the medullary swallowing centers, but may affect motor neurons in the medulla oblongata, resulting in a pseudomyelinating effect.

Other.

Brain tumors, Parkinson’s disease, encephalitis, cerebrospinal meningitis sequelae, dementia, traumatic brain injury, and other conditions involving the medulla oblongata may also cause pseudo medullary effects.

Pathogenesis

The cause of the disease is still unclear, and scientists hypothesize that it is caused by damage to the prefrontal cortex of the cerebral cortex. In addition, emotions such as depression and excitement leading to chemical changes in the brain are also involved [4-5].

Symptoms

Main Symptoms.

Patients may have persistent episodes, which are typically characterized by:

Emotional reactions that do not correspond to the mood or the intensity of the mood, usually crying or laughing, and are unpredictable, involuntary, have no antecedent, and can be without any apparent precipitating cause.

Outbursts typically last from a few seconds to a few minutes and may occur several times a day.

Patients may also experience sudden frustration and anger, or facial expressions that are disproportionate to the emotion [1,4-5].

Complications.

The pseudo medullary effect does not usually result in significant physical harm, but may cause embarrassment in the patient’s daily life and therefore lead to adverse effects such as social isolation, anxiety and depression.

Medical treatment

Department of Medicine

Neurology

If symptoms such as uncontrollable crying or laughing, or crying or laughing that does not correspond to the current situation are apparent, prompt medical attention is recommended.

Psychiatry

If you experience any of the above symptoms, you may also visit the Department of Psychiatry.

Preparation for medical treatment

Preparing for your visit: registering, preparing your documents, and frequently asked questions.

Tips for seeking medical treatment

Record the symptoms, duration and other relevant information to give the doctor more reference.

Preparation Checklist

Symptom list

Pay particular attention to the time of onset of symptoms, special behavior, etc.

Do you have repeated episodes of sudden, uncontrollable crying or laughing?

Is there frequent crying or laughing that is out of place and out of time?

Are there frequent facial expressions that do not match the mood?

Are there repeated episodes of frustration and outbursts of anger?

How long have the symptoms lasted?

Medical History Checklist

Has the patient suffered from any neurologic-related disorders?

Has the patient had any previous extensive brain injuries or lesions and other psychiatric disorders?

Checklist

Test results from the last six months to bring to the doctor’s office

Specialty tests: electroencephalogram, head CT, magnetic resonance imaging (MRI), etc.

Psychological examination: Pathological Laughing and Crying Scale (PLACS), Center for Neurological Studies – Mood Instability Scale (CNS-LS).

Medication list

Medication use in the last 3 months, if available in boxes or packages, carry with you to the doctor’s office

Antidepressants: tricyclic antidepressants (e.g., promethazine, clomipramine, etc.) and selective 5-hydroxytryptamine reuptake inhibitors (e.g., fluoxetine, paroxetine, sertraline), etc.

Diagnosis

Diagnosis is based on

Medical History.

The following history may be present, but the history is not necessary for the diagnosis.

The patient has a neurologic disorder.

The patient has had a traumatic brain injury.

Clinical manifestations

Frequent crying or laughing for no known reason, or crying or laughing inconsistent with the current situation, sudden frustration and anger, and facial expressions disproportionate to the mood.

Specialized tests

Head CT, Magnetic Resonance Imaging (MRI)

The main purpose is to rule out brain lesions.

Electroencephalogram (EEG)

The main purpose is to rule out epilepsy.

Psychological examination

The following two questionnaires are helpful in determining whether laughing and crying may be PBA, but they do not establish a diagnosis.

Pathological Laughing and Crying Scale (PLACS): mainly questions about the episodes, including how long they lasted, how they relate to your mood and social situation, and how depressed you felt afterwards.

Center for Neurological Studies-Less Stable Mood Scale (CNS-LS): focuses on questions about symptoms, such as how often do you have them? How do they make you feel?

Diagnostic Criteria

There is no uniform diagnostic criteria regarding the pseudo medullary effect [6], and the current tendency is to make a comprehensive diagnosis based on the patient’s symptoms and medical history combined with psychometric assessment.

One of the more clinically used diagnostic criteria in recent years is as follows [7].

Basic criteria

The patient has an involuntary or exaggerated emotional expression due to a brain disease, including laughing, crying, or other emotional manifestations.

Episodes can occur on a daily basis and the emotional response is exaggerated or inconsistent with the subjective emotional state, and are not induced by an obvious stimulus.

The episodes result in significant distress or cause social or occupational dysfunction.

The emotional expression cannot be explained by other neurologic disorders, psychiatric disorders, or a substance.

Supporting Criteria

The presence of the following would be more supportive of the diagnosis.

The patient may be accompanied by autonomic changes (e.g., facial flushing) and signs of pseudo medulla oblongata (e.g., tongue weakness, dysarthria, and dysphagia).

The patient may exhibit irritability.

Differential Diagnosis

Pseudo medullary effects are differentiated from bipolar disorder and depression.

Bipolar disorder

Similarities: Both can present with sudden laughter, rage, and depression.

Differences: Patients with bipolar disorder exhibit abnormally high and persistent emotions, irritability, accelerated or exaggerated thinking, etc., whereas patients with pseudo medulla oblongata experience crying and laughing that is uncontrollable or inconsistent with the current situation.

Depression

Similarities: Both have behaviors such as sudden laughter and depression.

Differences: Patients with pseudo medulla oblongata effect mainly exhibit sudden as well as uncontrollable, whereas patients with depression mainly exhibit significant and persistent low mood, which may be accompanied by slow thinking, reduced volitional activity, sleep disturbances, loss of appetite and other somatic symptoms of discomfort.

Treatment

Aim of treatment: to control the symptoms of pseudo medullary effect, to establish normal interpersonal relationship and good behavioral pattern.

Treatment principle: drug treatment is the mainstay [4].

Medication

Dextromethorphan/Quinidine

Mainly controls pseudo medullary effect symptoms and helps to control crying and laughing outbursts, especially in patients with multiple sclerosis and amyotrophic lateral sclerosis [8], well tolerated [9], was the first therapeutic drug approved by the FDA [10].

Antidepressants.

mainly control the symptoms of pseudomyelomeningocele and help to alleviate the frequency and severity of pseudomyelomeningocele episodes.

Commonly used drugs include tricyclic antidepressants (e.g., promethazine, clomipramine, etc.), selective 5-hydroxytryptamine reuptake inhibitors (e.g., fluoxetine, paroxetine, sertraline, etc.) and norepinephrine/5-hydroxytryptamine reuptake inhibitors (e.g., venlafaxine, duloxetine), etc. [7].

When used, it should be noted that the dose for the treatment of pseudo-extended medullary effects is generally lower than the dose of medications used to treat depression.

Other.

Some studies have suggested that behavioral interventions may play a role in the treatment and rehabilitation of patients with pseudo medullary effect, but further research is needed [11].

Prognosis

Cure.

Since the disease that causes it is usually chronic, pseudo-extended medullary effect is also very difficult to cure completely, and can only control its symptoms.

Prognostic factors

There may be several factors that influence its prognosis.

Patient’s condition: the severity of the disease and whether it is completely disconnected from society.

Medical conditions: including diagnosis, treatment, and level of care.

Family support: emotional sympathy and companionship.

Harmfulness

Frequent inability to self-control crying and laughing can interfere with normal life and social interactions, causing pain and embarrassment to many patients and their families, placing an additional burden on the family, and impeding the rehabilitation of many patients with neurological disorders back into their social roles.

Daily

Daily Management

Dietary management

Diet should be light and nutritious.

Attention should be paid to supplementing nutritious, high-protein, high-calorie, easy-to-digest foods.

Life Management

Exercise more and keep in a good mood.

Continuously take slow deep breaths during episodes of pseudo medullary effect until you can control your emotions.

After the patient experiences sudden emotions, relax your head, neck and facial muscles to reduce tension.

Psychological support

The patient’s symptoms are obvious, which will affect the patient’s socialization and life, and it is easy to produce pain and embarrassment. The patient’s psychological activities should be understood in time and psychological counseling should be provided to the patient.

Follow-up and review

Regular follow-up helps to find out the treatment efficacy of pseudo medulla oblongata effect in time.

The time of follow-up should be set by the specialist according to the patient’s specific condition or follow the doctor’s instructions for regular review.

Prevention

Pseudomyelitis can’t be prevented, but positive steps can be taken to raise awareness of the condition, and doing the following may help reduce the risk.

Discuss pseudo-extended medulla oblongata effects with those around you to the point where you are not surprised when emotions strike.

If you feel like laughing or crying, change your posture to adjust your emotions.

Patients with neurological related disorders or traumatic brain injury are advised to be closely monitored and seek prompt medical attention.