How is diffuse gray matter zotisis diagnosed?

1.
Medical history

Female, 15 years old, acting complaint: episodic unconsciousness, convulsions of limbs for 3 hours

Current medical history: the patient suddenly appeared unconscious when sleeping, limbs convulsions, eyes staring upward, no vomiting and other discomfort, family members pulled his limbs, lasted more than 30 minutes after the above symptoms were relieved, consciousness, self-getting out of bed after urinating on the bed after lying unconscious again, limb convulsions, lasting about 3 minutes of symptom relief, about five minutes to reappear the above symptoms, lasting five minutes after relief, repeated attacks more than 10 times, accompanied by urinary incontinence, emergency dial "120", emergency arrival at the scene to give "diazepam 10mg "Jing pushed, admitted to our hospital, consciousness gradually improved, and was admitted to our department
with "congenital seizures".

Anamite: 4 years of history of "epilepsy", in the "Fourth Affiliated Hospital of Zhengzhou University", given "carbamazepine and sodium valproate tablets" treatment, still repeated seizures, half a year ago "Junhai Brain Hospital" treatment, given "Jiulong Phoenix pills, anti-epilepsy tablets, levetiracetam tablets" treatment, the effect is not good, still repeated attacks;

2.
Images

3.
Clinical diagnosis: epilepsy

4.
MR diagnosis

Diffuse gray matter zodes ectopia

Subcortical banded gray matter ectopia is a disorder
of brain development caused by abnormal neurodevelopmental migration.
During the embryonic stage, when neuronal mother cells cannot smoothly migrate from subependymal to the cortex due to various reasons (trauma, genetic mutations, drugs, etc.
), the surviving neuronal mother cells are abnormal during the migration process, forming the same or similar structure
as the normal cortex.


Jacob (1936) divided gray matter ectopia into subependymal (nodular) and laminar forms
.
The latter lesions are located in
the subcortex or deep white matter.
According to the extent of the lesion, it can be divided into focal
type and diffuse type.
Both types can have subependymal and non-subependymal gray matter ectopia
.

5.
Clinical manifestations

Epilepsy is the main clinical manifestation and medical therapy is suboptimal
.
Malformations are generally thought to be the primary cause
of seizures.
Small-focal gray matter ectopia may have no symptoms
other than intractable seizures.
Focal gray matter ectopia often has sluggishness, seizures, headache and abnormal brain development, which can be combined with microcephaly, corpus callosum dysplasia, cerebellar dysplasia, midbrain aqueduct stenosis, malformation of the large blood vessels and skeletal system of the heart
.
The patient's intelligence is not affected only in terms of the lesion, but due to frequent seizures that damage brain cells, the patient's intelligence is often lower than normal
.
Patients with gray matter ectopia are often accompanied by various malformations (corpus callosum hypoplasia, hyaline septal hypoplasia, occipital pond arachnoid cyst, etc.
).

VI.
Diagnosis

CT diagnosis

CT showed that the ectopic gray matter was high density compared with the surrounding white matter, the ectopic gray matter was isolated, circular, lobulated, columnar, gyrus-like lesions, accompanied by the extension of the sulci to the depths of the white matter, there was no edema area around the lesion, no mass effect, CT plain scan and enhanced scan showed that the density of the mass and the cerebral cortex was the same, a few lesions were slightly dense, and there was no edema and mass effect
around the general lesion.
When ectopic gray matter lesions are large, a mild mass effect may occur, which is difficult to distinguish from well-differentiated gliomas, because these tumors can also not be strengthened
.
Some patients with gray matter ectopia may not be
positive on CT scan.

MRI diagnosis

Lesions are often frequent, more common at the top, paraventricles, frontal, temporal, occipital lobes can also have lesions
.
MRI showed that the gray matter of T1WI ectopic was low-signal compared to the surrounding white matter, and the gray matter of T2WI ectopic was high-signal
compared to the surrounding white matter.
MRI clearly distinguishes the gray matter of the brain, regardless of the size of the ectopic gray matter lesion, in all pulse sequence images, the signal of normal gray matter is the same
.
MRI shows small-focal ectopic gray matter masses located around the ventricles, either around the ependymal or into the ventricles, or in
the white matter in the center of the semi-oval.
Ectopic gray matter masses are nodular, can be single or scattered, are located in one or both hemispheres, and are not necessarily connected to
normal gray matter.
Ectopic large clumps of gray matter are often connected to the gray matter of the normal cortex, often with mass effects, such as compression of the ventricles
.
After GD-DTPA injection, enhanced scanning was performed, and the lesions were not strengthened
.
According to the location of the lesion shown by MRI,

It has been argued that gray matter ectopia is divided into three types
.
(1) Periventricular type or nodular type: the lesion is located in the subependymal area, with the anteroposterior and posterior angles of the lateral ventricles and symmetrical distribution
.
(2) Plate type: ectopic gray matter lesions are distributed along the ventricle to the cortex, or in a bridge shape to connect
the ependymal to the cerebral cortical gray matter.
(3) Belt type: The lesion is diffuse and distributed, located between the lateral ventricle and the cortex, which can compress the ventricle
.

Generally, the plate type is more common, followed by the nodule type, and the band type is rare
.

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