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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6042_Библиотеки_им_академика_М_И_Перельмана.pdf
181
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_58
5 8
Holocord Myelopathy with Thoracic Stenosis
58.1 Definition
Holocord myelopathy is myelopathy of the entire spinal cord from the cervicalmedullary junction to the conus medullaris.
58.2 Incidence
One case reported by CH Kim et al.
58.3 Etiology
The authors hypothesize that in spinal stenosis in general myelopathy is due to ischemia caused by mechanical pressure on the cord vasculature and it is localized to the
site of stenosis and mostly irreversible. In their case the whole cord is involved.
They found the disturbance of cerebral spinal fl uid (CSF) fl ow dynamics as shown
by MRI and Ciné MRI and that recovery was achieved after decompression.
58.4 Pathology
It was found by Klekemp et al. that altered dynamics of CSF leads to interstitial
edema below the stenotic lesion due to the pressure gradient between the subarachnoid space (SAS) and the spinal cord. The same authors suggested that interstitial
edema is a presyrinx state and that it may alter the extramedullary fl uid dynamics.
Abstracted from Kim et al. ( 2003 )
182
58.5 Clinical Picture
The patient was a 64 year old male presenting with paraparesis with sensory level
at T10.
58.6 Diagnosis
Diagnosis is based on the following criteria:
• MRI: Intramedullary high signal intensity involving the whole spinal cord on a
T2 weighted image and suspicious stenosis at T11 level. Ciné MRI showed
obstruction of the CSF circulation at T11 level.
58.7 Management
Patient had posterior decompression procedure, which lead to improvement of neurological defi cit, resolution of myelopathy and resumption of CSF circulation.
Reference
Kim CH, Chung CK, Kwon BJ, Kim HJ. Holocord myelopathy with thoracic stenosis: case report
and hypothesis. Spinal Cord. 2003;41(12):696–9.
58 Holocord Myelopathy with Thoracic Stenosis
183
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_59
5 9
Myelitis due to Hypertrophic Dens
59.1 Definition
This is a condition of upper cervical cord compression due to hypertrophied dens,
i.e. increased diameter (15 mm or larger). It is usually associated with atlantodental
osteoartheritis and may have developmental narrowing of the atlas ring.
59.2 Incidence
This is a very rare entity. One case was recorded by Moskovich and Crockard and
another by Okada et al.
59.3 Etiology
Etiological factors are abstracted from Okada et al.:
• Osteoarthritis of the antlantodental joint causes hypertrophy of the dens, similar
to osteoarthritis of the extremities. At the same time, this will reduce the internal
diameter of the atlas ring, predisposing the cord to compression. There is also
slight increase in the thickness of the anterior arch of the atlas.
• Atlantoaxial subluxation in developmentally small atlas ring complicated by
hypertrophic dens. Atlantoaxial instability may in time cause atlantodental osteo-
arthritis with subsequent dental hypertrophy.
Both conditions are post-traumatic.
Abstracted and reported by Okada et al. ( 2000 )
184
59.4 Pathology
This unique case reported by Okada et al. is the only source and one has to quote the
pathology they reported:
“The sagittally cut section of the cranium 2/3 of the hypertrophic dens showed
thickening of the cortical bone of the anterior and cranial parts of the dens.
Distribution of the cancellous bony trabeculae was also dense in the anterior and
cranial parts. The apical portion of the dens, where the ala ligaments was attached,
showed marked degeneration of the enthesis, including irregularity or disruption of
the tide marks and obscure Sharpey’s fi bers. Partially overlapped tide marks were
observed. The ALA ligament showed diffused myxoid degeneration and many fi ssure formations. Fibroblastic cells or tenocytes in the ligament had severely ballooned. In the anterior part, the cartilage showed severe fi brillation with myxoid
degeneration of the matrix. Multiple fi ssure, which extend into the basal layer of the
cartilage, were also observed. Chondrocystes were arranged in a columnar fashion,
although their nuclei and lacunar structures were frequently obscure. In the posterior part, where the cruciate ligament had supported the dens, the cortex was covered by fi brous tissue. The continuity of the cortex was partially disrupted, and
dense fi brous tissue had proliferated with immature bone formation. There was no
apparent calcifi cation.
59.5 Case Report
The picture of upper cervical myelopathy is present with pain and limitation of neck
movement, weakness of the upper and lower extremities, sensory loss, and
hyperrefl exia.
59.6 Diagnosis
Diagnosis is based on the following criteria:
• Clincal picture, history of trauma
• neurological exam
• radiographic series and frontal tomograms:
• cobra-head-like hypertrophic dens
• osteoarthritisof the atlantoaxial joints
• the AP diameter of the dens is increase in the sagittal plane
• the anterior arch of the atlas is thickened and sclerotic
• the atlantodental space is increased
• MRI of the spinal cord shows upper cervical cord compression between the
hypertrophied dens and the neural arch of the atlas.
59 Myelitis due to Hypertrophic Dens
185
59.7 Management
To relieve the myelopathy, the authors recommended decompression procedures,
with or without occipitocervical fusion. Details can be seen in the article.
Reference
Okada K, Sato K, Abe E. Hypertrophic dens resulting in cervical myelopathy: histological features
of the hypertrophic dens. Spine. 2000;25(10):1303–7.
Reference
187
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_60
6 0
Myelopathy due to Léri’s Disease
60.1 Definition
It is considered relatively benign disease affecting the skeleton and adjacent soft
tissue, but it causes much pain, deformity and considerable functional morbidity.
Synonyms include melorheostosis, fl owing hyperostiosis, osteopathia hyperostotica, or sclerosing bone dysplasia.
60.2 Incidence
Only a few cases have been reported affecting the vertebrae; according to Reznik
and Fried, since the disease was described in 1922, about 400 cases have been
reported.
60.3 Etiology
The cause of the disease is unknown. It is not hereditary and it affects genders
equally. It is thought to occur as an embryonal metameric disturbance which causes
a failure in intramembranes and to a lesser extent, endochondral ossifi cation.
Another hypothesis is that it is of neural origin (sometimes herpes zoster) associated
with the sensory nerve roots with resultant scarring and hyperostiosis. It may occur
with other types of bone sclerosis such as osteopoikilosis and osteopathia striata.
According to Zeiller et al. ( 2005 ), abnormality of sensory spinal nerves enables the
disease to affect its sclerotome, which is why the disease is almost always unilateral
and affects single sclerotome more frequently than multiple ones.
Abstracted from Reznik and Fried ( 2005 )
188
60.4 Pathology
According to Reznik and Fried: As a mesodermal disease Léri’s disease tends to be
segmental involving one or more sclerotomes, one bone (monostatic) or multiple
bones (polyostatic). Skeletal abnormalities may be restricted to one side of the body
(hemimelic) or to a single limb (monomelic), most predominately lower limb. The
bones involved are the diaphysis of the long bones, pelvis, small bones of the hands
and feet, rarely the ribs, vertebrae, skull, or facial bones. In the vertebrae the bone is
markedly sclerotic, causing compromise of the spinal canal and compression
myelopathy, and also foraminal stenosis leading to root compression. Extradural
fi brolipomatous tissues were described by Graver and Raby also Vivian. Schellhammer
et al. ( 2006 ) , report a case of intraspinal lipoma associated with the disease (German).
The disease is seen in early childhood and 40–50 % of cases are seen by 20 years.
60.5 Clinical Picture
Vertebral involvement leads to spinal stenosis and compression myelopathy manifested by: back pain (in mild cases may be the only symptom). With cord compression there will be sensory changes and motor weakness and eventually paresis.
Hyperosteosis of the extremities may be detected, by swelling, hyperpigmentation,
scleroderma, tissue calcifi cation, muscle atrophy, and myosclerosis. Skin lesions,
vascular anomalies and joint contractions may be associated with melorheostosis.
60.6 Diagnosis
Diagnosis is based on the following criteria:
1. Clinical picture, the age varies wildly from birth to 64 years.
2. Radiography shows hyperosteotic bone disease. The radiological appearance of
wax fl owing down a candle in longitudinal bones. For more details see Motimaya
and Meyers (
2006 ).
3. CT scan showed: block level
4. MRI of the spine: melorheostosis and reduced spinal canal with cord
compression.
5. Bone biopsy: sclerosed bone matrix with narrow Haversian canals
60.7 Management
Surgery consists of:
1. Decompression laminectomy and root decompression.
60 Myelopathy due to Léri’s Disease
189
2. Fusion may be needed in lumbar cases. See lumbosacral fusion by Robertson et al.
( 2003 ) .
Rehabilitation consists of:
1. In absence of neurological manifestation–the uses of diphosphonates, non-
steroidal anti-infl ammatory drugs, local steroid injections, analgesics, bracing,
and physical therapy.
2. For deformity of limbs— fasciotomy, tendon lengthening, capsulotomy, osteot-
omy may be needed and in uncorrectable deformities amputation may be consid-
ered as a last resort.
References
Motimaya AM, Meyers SP. Melorheostosis involving the cervical and upper thoracic spine: radio-
graphic, CT, and MR imaging fi ndings. AJNR Am J Neuroradiol. 2006;27(6):1198–200.
Reznik M, Fried GW. Myelopathy associated with melorheostosis: a case report. Arch Phys Med
Rehabil. 2005;86(7):1495–7.
Robertson PA, Don AS, Miller MV. Painful lumbosacral melorheostosis treated by fusion. Spine.
2003;28(12):E234–8.
Schellhammer F, Rapp M, Saleh A. Melorheostosis of the HWS in an intraspinal lipoma. Rofo.
2006;178(8):822–3.
Zeiller SC, Vaccaro AR, Wimberley DW, Albert TJ, Harrop JS, Hilibrand AS. Severe myelopathy
resulting from melorheostosis of the cervicothoracic spine. A case report. J Bone Joint Surg
Am. 2005;87(12):2759–62.
References
191
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_61
6 1
Idiopathic Primary Spinal Myoclonus
61.1 Definition
Spinal myoclonus is a rare movement disorder, characterized by myoclonic involvement of a group of muscles supplied by a few contiguous segments of the spinal cord.
61.2 Incidence
Primary spinal myoclonus is extremely rare.
61.3 Etiology
The etiology is unknown and is coined as idiopathic. There are theories to its cause:
• Loss of inhibitory function of local dorsal horn interneurons.
• Abnormal hyperactivity of local anterior horn neurons.
• Aberrant local axon re-excitations
• Loss of inhibition from supra-segmental descending pathways.
61.4 Pathology
The condition has no detectable pathology. Pathophysiology is discovered by electrophysiological changes. In secondary category one or more of the following may
be found:
• Spinal infection
• Spinal tumors
Abstracted from Campos et al. ( 2003 )
192
• Vascular disease
• Demyelinating diseases
• AIDS
• Spinal anesthesia
61.5 Clinical Picture
From the case described by Campos et al. a female, 26 years old with negative history of neurological disease, drugs, trauma, etc. She developed involuntary, spontaneous synchronous myoclonic jerks of the anterior wall of the neck, submadible
region, and the nape muscles resulting in neck extension. The contractions were
bilateral and rhythmic at of 1 Hz. The contractions were not controlled by will and
did not cease during sleep. They increased by emotion and neck extension. Tests
that included MRI, neurological, immunodefi ciency, metabolic, and endodermal
tests were all negative. However, electrophysiologic studies showed needle elctromyelography (EMG) irregular bursts, discharges of the motor units with a rate of
1–3 Hz in the anterior cervical muscles and posterior paraspinal muscles excluding
trapezius and sternomastoid. The affected muscles are the infrahyoid (geniohyoid,
thyrohyoid, sternothyroid, sternohyoid, and omohyoid). Supplied by C1 and junction of C2-3, the paraspinal muscles involved are: semispinalis capitis, rectus capitis
posterioris, obliquus capitis superioris, and splenius capitis, which all depend on the
C2 segment. All the muscles tested by EMG showed burst charges.
61.6 Diagnosis
Diagnosis is based on:
1. Clinical picture
2. EMG
3. Negative MRI and all other tests (see Clinical Picture above)
61.7 Management
Since the condition has no detectable cause, Botox® block of the affected muscles
resulted in relief, which stayed in their report for 5 months.
Reference
Campos CR, Limongi JC, Machado FC, Brotto MW. A case of primary spinal myoclonus: clinical
presentation and possible mechanisms involved. Arq Neuropsiquiatr. 2003;61(1):112–4.
61 Idiopathic Primary Spinal Myoclonus