Ghana: Syringo-Myelia, the Neuroscientist’s Demise

Dankyi A. Beeko, MD

29 June 2011

opinion

If a man narrated to you the smell of ‘burning meat’ sent him one day scuttling into the kitchen first, as he entered the house from work, you might accuse him of inordinately exaggerating. This narrative was nevertheless the true history of a lady, who as a result of carrying a disease in the in the spinal cord, could not detect the sensation of hot/cold. She stood in the kitchen with her hand on a hot plate until “her palm burned,” but didn’t realise it.

This was a sensational report in the early-eighties from the Medical Academy of Duesseldorf, Germany. The cause was detected, and surgery instituted with satisfactory results. God, in his wisdom, created the human being as a mammal, and he “added” the nervous system, out of the developmentally outermost layer, called the ectoderm.

To fit into the position, where the nervous system usually comes to sit, and function in such a way that we can walk, run, jump, feel it is hot, cold, or too cold, or too hot, the ectodermal tissue, which evolves to form the nervous system (all within the 1st Trimester), does it!

It further differentiates into two entities that must “synchronically” work together, and they are the central and the peripheral nervous systems.

They must function together like “a horse and carriage.” It is when this situation falters that we might encounter the situation such as the housewife’s hand burned like meat, and she did not realise it.

It is true that when we have our eyes closed, we still are able to tell whether an object is round, smooth, rough, or possesses a lot of corners, etc. When pinched or touched with a hot or freezing object, we react accordingly, and ordinarily, correctly.

As mentioned above, our nervous system consists of what is called the central nervous system, which in mammals such as us humans, consists of the brain and the spinal cord.

There is the peripheral nervous system, and that consists of “cables” that emanate one time from the skin, another time from cells in the brain, and like those from the skin, join in forming the bundles that end on organs in the body or the skin.

The bundles are called nerves, and example is the sciatic nerve (the thickest in our body; so thick and so tough, a man could hang himself on it).

It is in the leg. There are innumerable nerve roots, which converge to form the various nerves. The brain is encased in a bony cage called the skull.

The skull encompasses a space of about two litres in capacity, out of which the brain takes a litre-and-a-half. The rest is cerebrospinal fluid and arterial versus venous blood. The skull allows left and right, and from front to back each side, twelve pairs of nerves designated as cranial nerves (the optic is the best example).

There is a big exit in the back (called occiput), which is so big that it is called “foramen magnum.” Out of which exits the medulla oblongata turned spinal cord. As mentioned previously, cables pass up from the periphery into the brain, and back from the brain into the periphery, each bringing in messages or sending them out.

Two simple examples are the “spino-thalamic tract” – carrying impulses from the exterior along the spinal cord, to the thalamus – and it registers pain. Another is the thecortico-spinal tract. It makes sure movement is formulated.

So, as you might imagine, the spinal cord could be compared with a busy multi-track London, or Tokyo railway system, which carries coaches long and short distances. The Creator allowed only one small canal in the center of this thick-traffic structure, on which “fast trains” only traverse, in milliseconds.

This canal usually doesn’t hurt the nervous functions of the individual. There are some unfortunate circumstances however, which may not augur so well for the spinal cord of an individual.

It is “cavitation” within the spinal cord, which Von Ollivier named for the first time in 1824, “syringomyelia.” He met a cavity in the core of the spinal cord, and with the Latin/Greek root, SYRINGX. Etiologies of syringomyelia are varied and many. Congenital syringomyelia exists.

But, following trauma to the spinal cord, and thereupon development of hematomyelia, it has been observed, for months, and in the observation of the author, after as many as three years or longer. It may accompany an intra-spinal cystic astrocytoma (at times, “pencil-glioma”).

It may accompany the cranio-cervical malformation, designated as Arnold-ChiariType II, and beyond. Much as it should be obvious that functional impairment should follow the genesis of such disaster, patients may carry the lesions for many years (decades), before dysfunction may lead to their seeking the attention of the Physician.

An episode, such as accompanied the lady mentioned in the opening chapter, is extreme, but burns at the fingers of cigarette smokers, so subtle that they may carry on for years unobserved.

Cranio-cervical junction lesions may extend into the region of the brain-stem, with symptoms classical for syringe-bulbia. The affected is most often the cervical segment of the spinal cord and less commonly, the upper thoracic until the 4th thoracal.

Motor and sensory (SENSORY DISSOCIATION) impairment may render the individual not capable of carrying out any livelihood. Occasionally, Neuro-imaging (CAT-Scan, MRI, and Neuro-electrical examinations, EPs, EMGs), may collectively throw light on the diagnosis.

Surgery for cases where an Astrocytoma may be part of the problem may arrest symptomatically, after the tumor has been excised, and re-growth doesn’t occur.

In many instances, however, regrowth may rob the Neurosurgeon, as well as the patient, any fun thereof. Occasionally, there is the reward of objective, as well as subjective improvement following “a syringe-subarachnoid-shunt procedure”. It may be a fact, as stated by many an author, that enthusiasm in doing surgery isn’t rewarded with an impressive grade of improvement. Death, due to involvement of the brain-stem, as mentioned earlier on is catastrophic, but it occurs. Incidence (occurrence) of syringe-myelia is low.

This Author has dealt with 23 cases, in 32 years. There were 15 males and 8 females (a ratio of M:F OF ROUGHLY 2:1). There were five patients of age under ten. The children were in the group of CNS- malformations.

It must be mentioned that frequently, drawing a differentiation between ALS Syringo-myelia on clinical assessment alone may pose a problem. Neuro-imaging comes in very useful.

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