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PubMed2006

Segmental spinal myoclonus and syringomyelia: A case report.

We report the case of a 75-year-old woman who developed involuntary jerks of the abdominal musculature. They occurred spontaneously or triggered by a forced inspiration or attempts to rise from the supine position. Electromyography (EMG) recorded abnormal bursts of muscle activity in the abdominal, thoracic paraspinal, and intercostal muscles up to the 3rd intercostal space. The bursts were bilateral, arrythmic and synchronous in all muscles. Magnetic resonance imaging (MRI) of the spine revealed a syringomyelic cavity between the T3 and T10 levels. The topological correlation between the EMG muscle activities and the MRI findings was consistent with spinal myoclonus arising from the thoracic spinal cord. The synchronous bursts in muscles depending from few adjacent spinal segments suggested the diagnosis of segmental spinal myoclonus (SSM). There are few reports of SSM related to syringomyelia in the literature.

Abdominal Muscles↗
PubMed1990

[Neuroarthropathy, kyphoscoliosis and progressive tetraparesis in cervical syringomyelia].

The clinical picture of syringomyelia with special emphasis of its effects of the locomotor system is described on the basis of an individual case history. A 64 year old female patient experienced recurring subluxations of the right shoulder due to a neuroarthropathy. A marked throraco-lumbar kyphoscoliosis was present since childhood. The neurological picture was dominated by symmetrical muscular atrophy of both hands and an incomplete spastic paraparesis. Myelography of high cervical region shows an enlarged epidural space and atrophy of the myelon. MRI reveals a syrinx within the atrophied cervical spinal cord. Most likely the spinal cord has been enlarged and compressed from within by the syrinx which subsequently collapsed leaving the atrophied cervical spinal cord.

Arthropathy, Neurogenic↗
PubMed2006

Radiologic presentations in relation to curve severity in scoliosis associated with syringomyelia.

The purpose of the study was to further explore the radiological presentations of scoliosis associated with syringomyelia in relation to curve severity. 87 children and adolescents were divided into three groups: Group I (10 degrees 60 degrees). Curves were classified into typical and atypical patterns in the coronal plane and the sagittal profile measured. Cerebellar tonsillar descent or syrinx patterns in relation to curve severity and the frequency of atypical curves were also investigated. The frequency of atypical curve patterns from Group 1 to 2 was 46.2%, 45.2%, and 40.7% respectively. 65.3% patients with typical curve patterns had atypical features in all of the three groups. There was a significant difference of kyphotic angle among the three groups. Both the degree of cerebellar tonsillar descent and syrinx patterns had no correlation with the curve severity or the frequency of atypical curves. These results show that radiographic presentations, including atypical curve patterns, atypical features in typical curve patterns, and a normal to hyperkyphotic thoracic spine may suggest the need for preoperative MRI. There is no evidence to suggest that the degree of cerebellar tonsillar descent and syrinx patterns have an effect on the progress of scoliosis and the frequency of atypical curves.

Adolescent↗
PubMed1991

Syringomyelia in a patient with rheumatoid subluxation of the cervical spine.

A patient with rheumatoid damage of the cervical spine with cervical cord compression, complaining of a typical syringomyelic syndrome is described. Magnetic resonance imaging revealed cord compression due to an upward displacement of the odontoid peg with a mass of pannus behind the dens, and a syrinx cavity extending from C1 to T1. We postulate that this patient's syringomyelia probably represents a complication of cervical rheumatoid arthritis that has not been described.

Arthritis, Rheumatoid↗
PubMed1991

[A case of spinal arteriovenous malformation associated with syringomyelia extended over the entire spinal cord that presented mainly as spastic paraparesis].

A 48-year-old housewife was hospitalized because of spastic paraparesis accompanied by dysesthesia and amyotrophy in her lower extremities, and by urinary disturbances. At the age of 35 years she noticed tingling sensations in her right foot and gradually her gait became spastic. In a few years lumbago and the spinal form of intermittent claudication developed. When she was 41 years old muscle wasting was noticed in her right leg, and in four years the same feature was also seen in her lower left limb. Plain X-ray films showed a wide cervical canal with no signs of basilar impression. On MRI we could see a polycyst-like syrinx that extended over the entire spinal cord. In addition the surfaces of the lower thoracic cord seemed irregular, but arteriovenous malformation itself was not visualized. Neither hydrocephalus nor Chiari malformation was accompanied. Needle EMG examination revealed some neurogenic changes in the muscles of both lower limbs. Cystometry proved uninhibited contractions of the detrusor muscle and the existence of detrusor-sphincter dyssynergia. Myelography showed an enlarged spinal cord above the D11-12 level with a shadow of worm-like appearance between the D7-12 level. CSF protein was elevated. Spinal angiography confirmed the presence of single coiled vessels with a nidus at D8-10 that were fed by bilateral D10 intercostal arteries and drained to D7, D9 or D12. Embolization of these abnormal vessels was conducted, and since then the patient's condition has remained stable. Vascular malformation is a rare cause of secondary syringomyelia. Clinical manifestations are determined mainly by the level of vascular malformation and are not reflective of the level of syrinx.(ABSTRACT TRUNCATED AT 250 WORDS)

Arteriovenous Malformations↗
PubMed1990

[Reactive-inflammatory and degenerative-dystrophic changes in the nervous system in experimental syringomyelia].

The results of immunomorphologic and histochemical investigations of 12 rabbits with experimental syringomyelia are presented. Chronic pachymeningitis of the hind brain, resulting from the administration of kaolin leads to the disorders of liquor circulation on the level of outlet of the fourth ventricle this being a start mechanism for the cavity formation in the spinal cord. The reactive inflammatory, degenerative and immune reactions accompany the formation of cavity in the nervous tissue. The formation of cavity is followed by asymmetrical segment demyelination and reparative hyperplasia of the astroglial cells and gliosis of the cavity walls.

Animals↗
PubMed1990

Syringomyelia: a retrospective study Part II: Diagnostic and therapeutic approach.

In a retrospective study of 62 cases of syringomyelia, spanning thirteen years, we investigated the diagnostic value of the different imaging techniques, and the outcome of the several forms of therapy. Magnetic resonance imaging appears to be the examination of choice, being most informative and least invasive. When surgical treatment is indicated, syringoperitoneal shunting will stabilize or improve the condition in a substantial number of cases. Indications for the other surgical procedures are discussed.

Adolescent↗
PubMed1990

Syringomyelia: a retrospective study. Part I: Clinical features.

We performed a retrospective study of 62 patients with syringomyelia. Most noteworthy among the clinical features in our patient population are the following: segmental sensory disturbances are usually less restrictive than the classical thermoanalgesia, pain is a presenting symptom in almost 50% of cases, and skeletal abnormalities of the "status dysraphicus" are very common. The different hypotheses about the pathogenesis are also discussed.

Adolescent↗
PubMed1990

[Surgical management of syringomyelia associated with Arnold-Chiari malformation, primary IgA deficiency and chromosomal abnormality--a case report].

A rare case of syringomyelia associated with Arnold-Chiari malformation, primary IgA deficiency and sex chromosomal abnormality is reported. A 26-year-old Ethiopian black male was admitted with a complaint of hypalagesia of his left arm and face for 10 years. Neurological examination on admission revealed dissociated sensory loss of his left arm and face. Mild motor weakness of his hand and rotatory nystagmus on left gaze were also noticed. Plain craniogram of lateral view showed small posterior cranial fossa with low positioned inion and platybasia. MRI with T1-weighted images in sagittal plane revealed tonsillar herniation reaching C1 and syrinx extending from C2 to lumbar region. Although no episode of infectious disease nor allergy were experienced, blood analysis disclosed low serum level of IgA (7 mg/dl). The values of other immunoglobulins were within normal range. IgA in saliva was not detected, too. According to the clinical history and symptoms, a diagnosis is of primary asymptomatic IgA deficiency was obtained. Karyotype analysis showed inversion of Y chromosome. In an attempt to avoid anaphylactic shock on blood transfusion in a patient with IgA deficiency, autologous blood was prepared before surgery. Decompressive craniectomy of the posterior fossa with posterior arch of C1 and C2 was performed together with syringosubarachnoid shunt at Th 6-7 level. Postoperative course was successful and slight improvement of sensory disturbance was obtained. No respiratory or wound infection was occurred. The association of these three anomalies is very rare and genetical relationship is not known. From surgical point of view, it is conceivable that preoperative management in a case of asymptomatic IgA deficiency is uneventful.

Adult↗
PubMed1990

Syringomyelia.

Fluid cavities extending beyond several segments within the spinal cord comprise a pathologic entity that is not a disease, but a condition with many possible causes. Hindbrain herniation with or without associated arachnoid thickening is the most common cause, followed by spinal arachnoiditis, especially with paraplegia and also intraspinal tumors. The presentation may be variable, particularly in the hindbrain-related cases, and the final clinical picture is convergent because the pattern of destruction of the cord is determined by planes of weaknesses. It is almost always a surgical condition. The destructive forces are those of fluid cutting into the tissues, and expeditious and careful surgery is often successful, particularly if practiced early. The surgery should be planned to deal with raised CSF pressure and hydrocephalus, then the likely filling mechanism. Suck, the development of pressure differences acting over several seconds and deforming parts of the neuraxis such as a HBH, may provide a filling mechanism. Slosh, the impulsive rapid movement of CSF within the syrinx, both upward and downward, provides a continuing destructive force and may also be partly responsible for filling. Drainage of the syrinx may be a useful supplementary procedure and is conveniently combined with operation on the nonhindbrain-related cases. Drainage in these cases should not usually be at the widest part of the syrinx, tempting though that may be, but should be combined, where possible, with an attempt to correct the filling mechanism present at the site of the pathology. The ideas in this article are simple, but the surgical management of the various problems is not easy. The syringomyelic cord is fragile, neurologic losses are likely to be permanent, as are orthopedic problems such as Charcot's joints and kyphoscoliosis. Future management problems will include cases in which HBH or syringomyelia comes to light as an unexpected finding on MRI. If neurologic deficits are allowed to develop, they are likely to be irreversible and permanently affect the patients.

Cerebrospinal Fluid Pressure↗
PubMedDate not supplied

[Hemangioblastoma associated with syringomyelia and syringobulbia in a patient with rheumatoid arthritis].

A 80-year-old woman with a 25-year history of seropositive, erosive rheumatoid arthritis (RA) presented with a neurological clinical picture consistent with cervical cord compression, secondary to a hemangioblastoma with medulla, cerebellum and spinal cord involvement, with associated syringomyelia and syringobulbia. We present a review of the literature remarking the low frequency of this tumor (1%-2% of all intracranial tumors) and its exceptional presentation over the age of 70. We have not been able to find no cases of hemangioblastoma associated with rheumatoid arthritis, thus, we think, this association may be casual. Nevertheless, it emphasizes the need for careful evaluation of patients with rheumatoid arthritis and neurological symptoms before ascribing them to complications due to the rheumatoid disease itself.

Aged↗
PubMed1977

[Syringomyelia with severe osteo-articular changes].

Attention was drawn to a patient after admission to the Central Institute of Oncology, and syringomyelia was diagnosed on the basis of X-ray films taken by the author. The deformations were symmetrical and they were localized in the area of innervation of the C VI, VII, D I segment. The radiological diagnosis was proved by the neurological examinations.

Bone Diseases↗
PubMed1989

Current possibilities of diagnosing syringomyelia.

The diagnostic contribution of radiological examinations to the verification of the diagnosis of syringomyelia is assessed. A case report of the authors' own patient is given with the diagnosis confirmed by nuclear magnetic resonance.

Adult↗
PubMed1989

[Evaluation of conductivity in motor nerve fibers of the ulnar nerve in syringomyelia and neuropathy using 6 stimulation indicators].

The ulnar nerve's motoric fibre conduction velocity was stated to be insignificantly disordered in 32 patients with syringomyelia, whereas 47 neuropathic patients with unknown etiology showed the disorder mentioned above as occurring by far more frequently and in a more extent. From 6 stimulative indices, those of distal extremities velocity had maximum of deviations. Nevertheless, the indices of longer sections revealed the most numerous pathologic findings, especially in determining forearm velocity. The obtained findings are compared with 233 control subjects.

Adult↗
PubMedDate not supplied

[Clinico-roentgenologic signs of osteo- and arthropathy in children with syringomyelia].

Syringomyelia is a severe dysontogenetic lesion of the nervous system with polymorphous clinical and x-ray manifestations. The authors followed up 10 children aged 4-12 with osteo- and arthropathies, and presented a clinical and x-ray picture characterizing this process. They described in detail x-ray signs of this lesion over time and the formation of neoarthroses in the fracture areas, changes in zones of preliminary calcification and epiphyseal cartilage as well as in flat pelvic bones.

Bone Diseases↗
PubMedDate not supplied

[Diagnosis and microsurgical treatment of syringomyelia].

The results of examination (96) and microsurgical operations in 39 patients with syringomyelia are discussed. The average age and duration of the disease before the operation were, respectively, 36 and 11 years. Radiological and electrophysiological methods were used in establishing the diagnosis. Operative treatment included restoration of c.s.f. circulation at the level of the craniovertebral junction in 12 and ultrasonic myelotomy in the entry zone of the posterior radices in 27 patients. The results were positive in most patients with a catamnesis of 6-27 months, mainly when the disease was of a short duration.

Adolescent↗
PubMed1989

Syringomyelia with Arnold Chiari I malformation: a report of four cases.

4 cases of syringomyelia with type I Arnold Chiari malformation was seen presenting mainly with dissociated sensory loss, weakness of hands and upper motor neurone signs in the lower limbs. The first patient improved with posterior fossa decompression. The second and fourth patient appeared not to have improved after surgery, and the third case refused operation. One patient showed cord atrophy on myelogram presumably due to a collapse of the syrinx. One of the patients was unusual in that the syrinx extended down to segment T11. Magnetic resonance imaging of the posterior fossa and the cervical cord, to date, is the most useful procedure for diagnosis.

Adult↗
PubMed1989

[Syringomyelia from the neurosurgical viewpoint].

Indications for, and methods of, surgical treatment in patients with syringomyelia are described. In 16 patients a syringomyelic cavity was drained. Clinical, radiological and operative findings and results are reported and discussed. In six of twelve patients followed up, progressive deterioration was arrested by operation and two patients improved.

Adolescent↗