Tiger eye sign

Introduction Tiger eye sign is a symptom of globus pallidus substantia nigra and pigmentation. The MRIT2 weighting of the head shows that the bilateral globus symmetry high signal "Tiger Eye Sign" is the most characteristic manifestation of this disease, almost seen in all PANK2 gene mutations. Typical HSD patients, so "Tiger Eyes" can be used as the main basis for imaging diagnosis.

Cause

The cause is not fully understood. It is generally considered to be autosomal recessive inheritance. Taylor used DNA linkage studies to find that the disease-causing gene is located in chromosome 20p12.3~p13.

an examination

Related inspection

Brain Doppler Ultrasound (TCD)

According to the phenomenon of progressive extrapyramidal symptoms in adolescence, dystonia, myotonia and spastic paralysis of the lower extremities, MRI examination T2WI showed a typical "tiger eye sign", which can diagnose the disease. The SPECT imaging of aqua blue tissue cells or 59Fe-labeled iron salts in a positive family, bone marrow macrophages, or peripheral blood lymphocytes can be confirmed by radioactive accumulation and slow disappearance in the bilateral basal ganglia.

Differential diagnosis

1. juvenile paralysis agitans Ramsay Hunt (1917) reported that the disease is similar to adult Parkinson's disease, most patients have sporadic, Van Bogaert has reported familial cases, autopsy found lenticular atrophy and black Quality, globus pallidus cells are missing. The performance of mental symptoms is not obvious, the progress is slow, and the onset can still walk for 10 to 20 years, which can be identified.

2. In the case of Chediak-Higashi disease, nearly half of the patients showed mental retardation, seizures, cerebellar ataxia and Parkinson's syndrome. Chronic polyneuropathy is the main manifestation.

3. Early-onset Huntington's chorea is 5 to 14 years old or as early as 1 to 4 years old, with a family history. Nearly 5% of Huntington's chorea is adolescent, with mental retardation, slow speech, strong muscles, small gait, increased flexion of limbs and trunk, and dance-hand and foot movement, occasionally Babinski sign. Some adolescent patients have no muscle rigidity, and seizures are more common in adult type. Behavioral abnormalities, violations and posture tensions, as well as myoclonus and cerebellar ataxia can occur. In the end, the patient can't speak, opening mouth, flexing his limbs, clenching his fists, and twisting his torso.

4. The state of myelin (status dysmyelinatus) is an undetermined disease proposed by Vogt, and the myelinated fibers and nerve cells of the lenticular nucleus disappear. The performance of the extrapyramidal muscle is strong, and gradually develops hand and foot hyperkinesia. Eventually, the limb is weak or paralyzed, resulting in flexion deformity, often misdiagnosing Parkinson's disease.

5.Lafora-boby may have muscle rigidity, muscle shock, movement and tremor, and may have seizures and dementia.

6. Leigh disease is rare in clinical practice. Extrapyramidal muscle rigidity occurs gradually in children's late or adulthood. CT shows the formation of nucleus cavity.

7. The disease still needs to be associated with hepatolenticular degeneration, heavy metal poisoning, encephalitis sequelae, and Segawa type of L-dopa-responsive dystonia, dentate nucleus red globus pallidus Identification of dynasty body atrophy (DRPLA), neurological symptoms are similar to Wilson's disease, but no evidence of liver involvement or copper metabolism disorders. Many of the diseases in this group have tendon and extrapyramidal signs such as dance - hand and foot, dystonia, etc., and need to be combined with clinical and auxiliary examination data to identify.

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