About Paraneoplastic Neurological Disorders (PND) > Central Nervous System (Brain and Spinal Cord) > Paraneoplastic Encephalitis Associated with anti-Ma2 Antibodies

Paraneoplastic Encephalitis Associated with anti-Ma2 Antibodies

This type of encephalitis mostly affects the limbic system, hypothalamus and brainstem (Figure 1). Patients develop a combination of the symptoms.

Symptoms associated with the limbic system:

• mood changes (depression, irritability and anxiety),
• problems sleeping, and
• short-term memory problems.

The symptoms associated with memory problems are always severe and should not be confused with mild problems sometimes caused by lack of attention, or with chronic memory problems that some patients ( with other diseases) develop over months or years. In addition, many patients with limbic encephalitis develop seizures or spells (for example, sudden sensation of bad smell or taste, staring or lip smacking, cloudiness of consciousness) or sometimes, total loss of consciousness resulting in a “grand mal seizure.”

Symptoms associated with the brain stem:

• double vision
• blurry vision
• changes in the quality of the voice (slurred speech)
• vertigo
• dizziness
• difficulty swallowing fluids or solids
• problems in the control of breathing
• changes in the rhythm of the heart (dysrhythmias)
• tremor and slow movements (that may resemble Parkinson’s disease)
• progressive decrease of consciousness
• coma.

. There are a few characteristic symptoms that occur more frequently than in other PND:

• There is frequent involvement of the hypothalamus. This may cause excessive daytime sleepiness, sometimes with episodes of rapid and intense sleep (called narcolepsy). Other patients develop endocrinedisturbances that can affect the thyroid or suprarenal glands. This causes increased food intake and weight gain.

• In contrast to other types of paraneoplastic brainstem encephalitis, patients with anti-Ma2 antibodies have greater involvement of the upper part of the brainstem. Due to the structures located in this area, patients may have problems moving the eyes, first up and down and later sideways. Other symptoms include, double vision, and a jumping sensation of the visual field (called opscillopsia). Some patients can only move their arms and legs very slowly (hypokinesis) and appear rigid. They may have difficulty opening the eyes, or moving the lips. When they try to talk, their voice is barely audible to the point that sometimes they seem to be mute. However, they are able to understand other people and answer commands with signs (such as thumbs up or down).

When the patient is a man younger than 50 years, the tumor is almost always in the testis (Figure 2). Sometimes the tumor is so small that it may not be found despite many different tests. In these cases an ultrasound of the testes can be very useful.

When the patient (man or woman) is older than 50 years the associated tumor can be in the lung and other part of the body.

Prompt diagnosis of this type of encephalitis is very important because about 35% of patients greatly improve after treating the tumor and immunotherapy.