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Which syringomyelia is truly associated with multiple sclerosis?

Necrosis of the spinal cord within multiple sclerosis (MS) lesions was suggested as a putative cause of syringomyelic cavity development in MS. A number of evidences suggest however that mechanisms other than necrosis are pathogenetically relevant for cavity formation, possibly depending on the atypical topographical distribution of the demyelinative lesion and on the increased cerebrospinal fluid pressure into the central canal below the compression. Not coincidentally, the hypothesis of post-necrotic and ex-vacuo mechanisms leading to cavitation derives from Japanese studies where MS is characterised by high tissue destructive capability and, besides its rarity, has many differences from the more common Western MS type and similarities with the acute disseminated encephalomyelitis (ADEM). Our opinion is that different MS types (Asian and Western) are accompanied by nonuniform mechanisms of syrinx formation and that the Asian MS type shares common, post-necrotic mechanisms with ADEM.

Cerebrospinal Fluid↗

[Electro-clinical data in medullary forms of the Chiari malformation without syringomyelia].

The electrophysiological findings in 18 patients with Arnold-Chiari malformation (ACM) revealed by spinal symptoms excluding syringomyelic syndrome (Brown-Sequard syndrome, paraparesis with or without posterior column involvement, motor neuron syndrome or more complex myelopathies) are reported. In 16 cases, EMG disclosed abnormalities consistent with lesion of anterior horn cells restricted to upper limb in 7 cases, and generalized in 9 cases. In 2 cases, motor and sensory conductions were abnormal. The occurrence of such abnormalities in patients with ACM is emphasized as they could lead to confusion with amyotrophic lateral sclerosis or spinal muscular atrophy. Neuroradiological studies are therefore necessary owing to possible surgical treatment. The hypothesis relating lower motor involvement and ACM are reviewed.

Adult↗

[Syringomyelia discovered in the course of peridural anesthesia].

Neurological symptoms after epidural anaesthesia suggest complications due to anaesthetic procedure. We report the case of a child who underwent perineal surgery in a gynaecological position under general and epidural anaesthesia, who experienced the day after surgery hypoesthesia of the whole left lower limb without any motor deficit. Magnetic resonance imaging excluded spinal compression, but revealed syringomyelic cavity extending from T9 to T11. Electromyogram evaluation was normal. Clinical signs completely vanished within 24 hours. This case emphasizes that the apparition of neurological signs after central nerve blocks is not only a complication of regional anaesthesia, but may reveal unknown neuropathy or result from surgical position or surgical procedure. Meticulous neurological examination, magnetic resonance imaging and electromyogram are immediately required.

Anesthesia, Epidural↗

Syringomyelia presenting as paroxysmal arm posturing resembling seizures.

A 9-year-old female presented with daily episodes of medically refractory paroxysmal bilateral arm posturing, which had long been thought to be epileptic seizures. She also had other types of episodes, including daily staring spells and infrequent generalized tonic-clonic convulsions. Neurologic examination was normal except for delayed cognitive development. The results of previous electroencephalograms (EEG) were normal, and magnetic resonance imaging of the head revealed a Chiari I malformation. Video EEG monitoring revealed no EEG changes during the attacks, and magnetic resonance imaging of the spine revealed a large cervical syrinx associated with the Chiari malformation. The episodes of paroxysmal bilateral dystonic arm posturing resolved after surgical intervention for the syrinx. This report illustrates that cervical cord disease is an unusual although potentially treatable condition to be considered in the differential diagnosis of paroxysmal episodes with dystonic movements of the arms, even in the absence of other physical findings of myelopathy.

Arm↗