Episodic giggling

Introduction For the onset of autonomic symptoms, it is characterized by episodes of smirk, which suddenly stops for a few seconds or tens of seconds. There is no loss of consciousness during the attack, and dozens of episodes can occur every day without any incentive. Although the patient is often happy, the facial expressions give people a feeling of stupidity. Hypothalamic hamartoma is the causative source of this disease, and surgery is the best way to eradicate this disease. The preferred method is to perform microsurgery. In the treatment, carbamazepine is generally preferred, and sodium valproate may also be selected.

Cause

The hypothalamic hamartoma induces this symptom, and there are two types of neuroepithelial tumors. One type is formed by the interstitial cells of the nervous system (ie, glial cells), called gliomas. The other type is formed by parenchymal cells of the nervous system (ie, neurons) without a general name. Since both types of tumors cannot be completely distinguished from pathogens and morphologies, and gliomas are often more common, neuronal tumors are included in gliomas. Tumors of neuroepithelial origin are the most common intracranial tumors, accounting for approximately 44% of the total number of intracranial tumors. Hypothalamic hamartoma is characterized by ectopic "brain tissue" at the gray nodule. Pathological sections show that hamartoma is composed of various neurons with well-differentiated, irregularly shaped and irregularly distributed, astrocytes and ganglion cells. Scattered between fibrous matrices, where fibrous connective tissue and vascular structures are not apparent. Electron microscopy showed that there were small round bodies of different sizes in the periphery of the neurons. The protrusions contained numerous vesicles and microtubules. The synaptic structure was visible. Occasionally, there were myelin axons, and there were a large number of high-density secretory granules at the ends. Some people have confirmed the secretion of GnRH secretory granules by immunohistochemistry, indicating that the hypothalamic hamartoma has certain neuroendocrine function. The large number of neurons and synapses suggest that the neurons in this part are extremely active and have a wide range of connections with the peripheral limb system, which may be related to diencephalic epilepsy.

an examination

Related inspection

Brain CT examination of brain MRI

1. CT examination

The plain scan is an equal-density space-occupying lesion located in the posterior aspect of the pituitary stalk, the inter-foot pool, the anterior chamber of the pons, and the saddle-upper pool. The larger tumor can compress the deformation of the third ventricle, and there is no enhancement after injection.

2. MRI examination

It is currently the preferred and best imaging method. Typical manifestations are: located behind the pituitary stalk, between the optic chiasm and the midbrain, with round or elliptical masses in the gray nodules and papillary areas, with clear boundaries, pedicled or pedunculated; upwardly protruding into the third ventricle Shaped or elliptical bulge. The tumor signal is uniform, mostly similar to the cerebral cortex. T1W1 is equal signal. In a few cases, the signal is slightly lower than the cerebral cortex, and the individual is slightly higher signal; T2W1 is equal or high T2 signal change. There was no enhancement after injection of the enhancer. MRI maximizes the relationship between HH and important surrounding structures.

3.EEG check

In the early days, when the patient had only a ridiculous seizure, the interictal scalp EEG was usually normal. As the disease progresses, the form of seizures becomes diversified: the background activity of the patient's EEG is diffusely slowed, and the epileptic discharge, inhibition, or visible of the single or bilateral temporal or frontal lobe can be seen in the interictal phase. Irregular, comprehensive, slow-wave discharge. EEG during episodes is characterized by diffuse low-voltage rhythmic fast activity or comprehensive EEG background suppression.

Differential diagnosis

It needs to be differentiated from the following diseases: craniopharyngioma, saddle glioma or germ cell tumor. The most important of these diseases, except for abnormal tumor signal or density, is the tendency of the lesions to be strengthened and progressively increased.

1. Hypothalamic hamartoma, also known as gray nodular hamartoma, is a rare intracranial congenital malformation that occurs frequently in children. It is mainly characterized by excessive estrogen levels, early development of secondary sexual characteristics, and increased bone age. A ridiculous episode with no incentives. Some people think that the hypothalamic hamartoma originates from the papillary or gray nodules, which is caused by the dislocation of the globules on the 35th to 40th day of pregnancy. It is a midline neural tube regurgitation syndrome formed by normal brain tissue. The ectopic mass, the nerve cells that make up this deformity are similar to the nerve tissue in the gray nodules, accompanied by normal glial cells.

2, from imaging, pathology, clinical signs and many other aspects. The ridiculous episode is mainly characterized by recurrent episodes, no ridicule and content, but can not control the stereotypes, unnatural laughter, even crying, sudden sudden stop, lasting 5 seconds to 1 minute, frequent episodes, awake or Can occur in sleep, can be accompanied by a variety of other seizures, including secondary generalized tonic attacks, complex partial seizures. The sedative episode can be divided into symptomatic and spontaneous according to whether there is a clear lesion in the patient's brain. The sneaky episode can also be divided into simple and non-simple sedative episodes according to the time of symptom onset, whether there is emotional change and the state of conscious damage. Simpleness mainly occurs under the age of 5, and the patient has no emotional changes and disturbances of consciousness other than laughter, and almost no other epilepsy symptoms.

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