I. Abadie’s syndrome, Department of Neurology, Baicheng Central Hospital, Du Quanyue
Abadie’s syndrome refers to the loss of pressure and pain sensation in the Achilles tendon in patients with spinal consumption (a kind of substantial syphilis). It is an important sign for the diagnosis of spinal consumption. It has a high rate of positivity in all stages of the spinal cord consumption disease, and is therefore useful for early diagnosis. It is often positive on both sides, and the degree of achilles tendon dysesthesia may be inconsistent. The relationship between this sign and other symptoms of spinal consumption is as follows.
(a) The sign is not necessarily concurrent with the lightning-like pain of the Achilles tendon and is not associated with superficial sensory disturbances of the lower extremities.
(b) It is mostly accompanied by loss of ankle reflex.
(3) The pressure pain sensation caused by squeezing the gastrocnemius muscle is still preserved.
This syndrome is occasionally seen in hysteria and spinal cord compression syndrome, but these two diseases still have other parts of the nociceptive deficit.
Adie’s syndrome
Also known as tonic pupil syndrome; pupillary tension disorder.
Etiology and mechanism] The etiology is not clear. The site of the lesion may be: (1) the ciliary ganglion and ciliary nerve, or its nearby lesions; (2) the upper cervical medulla; (3) the nucleus accumbens lesion. However, the mechanism of why this syndrome is accompanied by loss of knee tendon reflex is still unknown.
Clinical manifestations】 Most often occurs in women under 30 years of age. Pupillary tension and loss of knee tendon reflex are the main symptoms. The pupil is dilated on one side, and the light response and adjustment response are absent. The pupil can be slowly constricted by bright light exposure for more than half a minute, and the pupil can also be slowly constricted when the eyes meet for five minutes. This phenomenon is also known as Adie’s pupil or tonic pupil, and Adie’s classifies the syndrome as follows.
(a) Complete type: The pupil is tonic and the knee tendon reflex is absent.
(ii) Incomplete There are four conditions.
1. pupillary tonicity only.
2. non-stereotyped pupillary tonicity (iris palsy).
3. disappearance of the knee tendon reflex accompanied by amorphous pupillary tonicity.
4. disappearance of only knee tendon reflexes.
Differential diagnosis
Adie’s pupil is also seen in the following diseases.
(a) Actinic nerve paralysis (oculomotor paralysis) is divided into complete and incomplete. Complete oculomotor nerve paralysis has three features, namely flaccid ptosis of the upper eyelid, the eye in an external inferior oblique position, dilated pupils, and loss of light response. The head is often turned to the opposite side of the motor nerve palsy. Protracted ophthalmic nerve palsy may cause degeneration and pseudo-Von Grafe’s sign.
(b) Transtentorial hernia (herniation of the cerebellar fissure) The presence of Adie’s pupil in the short term suggests the occurrence of herniation of the cerebellar curtain. This is due to the pulling and compression of the arteriovenous nerve caused by the herniation of the subiculum. The pupil on the side of the lesion first narrows in the early stages of herniation and then gradually increases in size. When bilateral herniation of the gyrus or downward displacement of the brainstem involves the bilateral arteriolar nerve, the pupils may be dilated bilaterally. Pupil dilatation occurs first, followed by extraocular muscle paralysis, oculomotor disorders and ptosis. In addition, brain herniation is often accompanied by impaired consciousness, hemiparesis, denervation and altered vital signs.
(iii) Other Eye injuries, glaucoma, congenital syphilis, hysteria, xyloplasmic schizophrenia, and atropine poisoning can all present with Adie’s pupil, which is not difficult to identify by medical history and the presence or absence of the knee tendon reflex.
Apert’s syndrome
Apert’s syndrome is also known as acromegaly; acromegaly syndrome.
Etiology and mechanism] In the past, the disease was thought to be caused by osteitis, rickets, amniotic fluid cord compression, congenital syphilis, rubella and other external causes, but now it is thought to be an autosomal dominant genetic disorder. It is generally sporadic. Both parents tend to be older, especially the father.
Clinical manifestations
(A) Acute head deformity: The head is pointed and short, the forehead is high, the coronal suture is early healed, the longitudinal axis of the skull is enlarged, and the large fontanelle is raised upward. The eye sockets are shallow, the eyeballs protrude, the distance between the two eyes increases, and the strabismus. The nose is small and flat, with a hooked nose. The upper jaw is hypoplastic, more protruding, the mouth cover is high, and sometimes the mouth cover is split, presenting a special appearance.
(B) Extremity deformity Syndactyly (toe) is mostly symmetrical to the left and right, and the degree is not equal. Skin fusion or complete bony fusion; partial fusion or complete fusion, with complete fusion of the 2nd, 3rd, and 4th fingers (toes) being the most common. In addition, the metacarpal bone is short and may fuse with the radius, limiting joint movement.
(c) Patients have various degrees of mental retardation, while the brain lacks specific pathological changes. Optic papilloedema is less common and optic nerve atrophy is more common.
[Differential diagnosis
(a) Crouzon’s syndrome, also known as hereditary craniofacial dysplasia, is a special type of premature closure of the cranial suture. It has a family history. Crouzon’s syndrome is characterized by premature closure of most cranial sutures, poor development of the maxilla and hydrocephalus, short anterior and posterior diameters of the skull, separation of the eyes and outward strabismus, receding nasal base, widening of the nasal arch, narrowing of the lower edge of the orbit, protrusion of the eye forward, and backbite of the upper and lower teeth. In addition to head and facial deformities, there are often headaches and brain crushing signs.
(B) Microcephaly is often caused by harmful environmental factors during fetal life. Occasionally, it is seen in autosomal recessive inheritance. The brain and skull development are impaired in this disorder, and the weight of the brain does not exceed 1000 grams when fully developed, and the maximum cranial circumference generally does not exceed 47 cm. There are also specific changes in the shape of the skull, with flat, narrow frontal and occipital areas and a slightly pointed top, in contrast to the fully developed facial bones. The scalp is thickened and the hair is coarse and dense. The stature is short and the intellectual development stays at the idiot stage.
IV. Argyll Robertson’s syndrome
Also known as: Ar-Ro’s pupil; reflex iris palsy.
Etiology and mechanism】 Argyll Robertson’s pupil is a unique sign of neurosyphilis. It is caused by the destruction of the light reflex pathway by syphilitic lesions (especially spinal consumption). Pupillary constriction is associated with lesions near the interneurons in front of the nucleus accumbens of the midbrain.
Clinical manifestations】 ① the retina is perceptive to light, i.e. the retina and optic nerve are not abnormal; ② the pupil is small; ③ the pupil reflex to light disappears; ④ the regulatory reflex is normal; ⑤ poisonous lentil base drops can cause pupil constriction, while atropine drops do not completely dilate the pupil; ⑥ the pupil morphology is abnormal (not round and irregular at the edges) and asymmetric; ⑦ these disorders are permanent, mostly bilateral, and occasionally unilateral.
Differential Diagnosis】 Argyll-Robertson’s pupil can be seen in neurosyphilis, but also in other diseases, called pseudo-Argyll Robertson’s syndrome. The affected pupil is unilaterally dilated 80% of the time, the light response is absent or delayed, the convergence response is mildly affected, and occasionally the affected pupil may be smaller than the normal pupil after slow contraction. Common causes include trauma affecting the midbrain; trauma to the eye or fossa; tumors of the midbrain periaqueductal region, such as tumors of the tetrahymena, pineal gland, third ventricle, and conduit; cerebrovascular disease (softening foci in the midbrain) and multiple sclerosis. In such cases, in addition to the presence of an a-lost pupil, there is often a vertical gaze palsy and other extraocular muscle paralysis.