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Have Already Been Described to Occur with Some 804 Letters, Correspondence, Book reviews have already been described to occur with Correspondence to: Dr D Michel, Service de Neu- some mutations. rologie, Hôpital de Bellevue, 42055 Saint-Etienne, There are only two previous reports Cedex 2, France. relating to three pairs of identical twins with CMT and known genetic defects. In the two 1 Goldsmith P, Rowe D, Jäger R, et al. Focal ver- pairs with the 17p11.2 duplication there was tebral artery dissection causing Brown- remarkable clinical variability.6 We have also Séquard’s syndrome. J Neurol Neurosurg Psy- seen a pair of identical twins with a P0 muta- chiatry 1998;64:415–16. 2 Gutowski NJ, Murphy RP, Beale DJ. Unilateral tion in whom there was marked variability in upper cervical posterior spinal artery syndrome early ages (unpublished data). Apart from the following sneezing. J Neurol Neurosurg Psychia- asymmetry of toe responses in one of the try 1992;55:841–3. probands, the genetically identical twins 3 Bergqvist CAG, Goldberg HI, Thorarensen O, et al. Posterior cervical spinal cord infarction described here are phenotypically very simi- following vertebral artery dissection. Neurology lar, suggesting that the expression of this 1997;48:1112–5. mutation was not influenced by other non- 4 Hundsberger T, Thömke F, Hopf HC, et al. genetic factors. Symmetrical infarction of the cervical spinal cord due to spontaneous bilateral vertebral Codon 39 seems to be of particular Sagittal T2 weighted MRI of the cervicodorsal artery dissection. Stroke 1998;29:1742. importance to Cx32 protein function as cord : high linear signal extending from C4 to 5 Masson C. Les accidents ischémiques médul- changing of the wild type amino acid has T5 vertebral levels. laires. Presse Med 1994;23:1723–8. caused CNS dysfunction in addition to the peripheral neuropathy. Moreover its expres- a predominantly right sided tetraparesia and Autonomic dysfunction and orthostatic sion does not seem to depend on non-genetic urinary retention. There was no history of factors, as might be expected in a hemizygous neck trauma. Cranial nerve examination was hypotention caused by vitamin B12 condition. normal. There was a right sided hemiplegia deficiency and a moderate left sided hemiparesis. Deep tendon reflexes were normal, right plantar Orthostatic hypotension sometimes is a WMJr was supported by grants from CAPES, reversible neurological complication of vita- FAPESP, and FAEPA (Brazil). response was extensor. There were bilateral spinothalamic problems below T4 with loss min B12 deficiency.12 Eisenhofer detected W MARQUES JR of touch sense in the right leg. Thus the deficient sympathetic catecholamine release M G SWEENEY in insulin tolerance testing,2 but the mech- NWWOOD examination was consistent with atypical, University Department of Clinical Neurology, right cervical Brown-Séquard’s syndrome. anism of orthostatic hypotension in vitamin Institute of Neurology, Queen Square, London, UK Biological investigations were normal. CSF B12 deficiency remains unclear. We report a protein was 0.34 g/l, glucose 2.54 mmol/l patient with vitamin B12 deficiency and S J WROE (2.10–4.20 mmol/l). There were 7 white cells reversible orthostatic hypotension, and dis- Department of Clinical Neurology, 3 Ipswich Hospital NHS Trust, Ipswich, UK and 25 red cells/mm . cuss the mechanism of this symptom. There were no oligoclonal bands. The A 77 year old man admitted to our hospital W MARQUES ECG was normal. There was no aortic had had unstable gait and urinary urgency for Department of Neurology, dissection shown on CT or MRI. Visual 6 months, clumsiness of the hands and School of Medicine of Ribeira˜o Preto, Brazil evoked potentials were normal. Somatosen- tingling sensations in the legs for 3 months, Correspondence to: Dr Nicholas Wood, Institute of sory evoked potentials were abnormal for the and, for a month, occasional dizziness on Neurology, Queen Square, London WC1N 3BG, standing. The dizziness was mild without any UK. Telephone 0044 171 837 3611 extn 4255; fax right lower limb at the cervical level. A sagit- 0044 171 278 5616. tal T2 weighted MRI showed linear cord high attack of syncope. He had no other symptoms signal from C4 to T5 vertebral levels consist- or signs of autonomic dysfunction but impo- ent with an ischaemic lesion (figure). On cor- tence and erectile failure were noted 10 years 1 Bone LJ, Deschênes SM, Balice-Gordon RJ, et al. Connexin 32 and X-linked Charcot-Marie- responding axial cuts, this was shown to before the onset of neurological symptoms. Tooth disease. Neurobiology of Disease involve the region of the anterior horns at He had not taken any medicine which would 1997;4:221–30. cervical level and to prevail on the right half aVect the autonomic nervous system. He did 2 Nicholson G, Corbett A. Slowing of central conduction in X-linked Charcot-Marie-Tooth of the spinal cord at dorsal level. MRI of the not have a habit of drinking. neuropathy shown by brain stem auditory cerebellum and brain stem was normal. Cer- Physical examination on admission de- evoked responses. J Neurol Neurosurg Psychiatry ebral angiography showed an irregular steno- tected no signs of anaemia, heart failure, or 1996; 61:43–6. sis of the right and left cervical vertebral dehydration. Neurological examination 3 Paulson H, Deschênes S, Scherer S, et al. CNS white matter abnormalities in a patient with artery typical of a dissection. The patient was showed dysaesthesia and decreased sensation X-linked Charcot-Marie-Tooth disease treated with oral anticoagulants. One year of all modalities in distal parts of all the limbs. (CMTX). Am J Hum Genet 1994;55:497. later, the sequelae were a spastic paraparesia Deep tendon reflex was absent in the lower 4 Latour P, Fabreguette A, Ressot C, et al.New mutations in the X-linked form of Charcot- with right sided central pain and mild urinary limbs, and Babinski’s sign was positive Marie-Tooth disease. Eur Neurol 1997;37:38– retention. MRI and MRA showed the resolu- bilaterally. Mild limb ataxia was seen in the 42. tion of the cord signal and normal right and four limbs, and Romberg’s test was positive. 5 Bell C, Willison H, Clark C, et al. CNS left vertebral artery. Haematological studies disclosed mild abnormalities in a family with a connexin 32 mutation and peripheral neuropathy. Eur J The cervical cord is mainly supplied by macrocytic hyperchromic anaemia (haemo- Hum Genet 1996;4(suppl 1):136 radicular arteries rising from the vertebral globin 14.0 g/dl, mean corpuscular volume 6 Garcia CA, Malamut RE, England JD, et al. artery. Thus,vertebral artery dissection can 104 fl, mean corpuscular haemoglobin con- Clinical variablility in two pairs of identical twins with Charcot-Marie-Tooth disease type lead to an ischaemia limited to the cervical centration 35.2 pg), with a few (3%) 1A duplication. Neurology 1995;45:2090–3. cord. Extensive ischaemia to the dorsal cord hypersegmented polymorphonuclear cells. (T5) is uncommon. Our results suggest that His serum vitamin B12 concentration was this area is sometimes supplied from the ver- markedly decreased (38 pg/ml; normal 249– Isolated ischaemia of the spinal cord tebral artery. Some authors state that this 938 pg/ml). Intrinsic factor and parietal cell due to bilateral vertebral artery region could be a critical zone and its vascu- antibodies were positive in the serum. Echo dissection larisation could be provided from the arterial cardiography showed no evidence of heart cervical cord region.5 The bilateral ischaemic failure. In a study of peripheral nerve Clinical features in vertebral artery dissection lesions extending through several cervical conduction, amplitudes of sensory nerve are rarely associated with an ischaemic lesion and dorsal segments are in favour of water- action potentials were slightly decreased in of the spinal cord.1 The few cases related and shed infarcts caused by hypoperfusion due to the lower limbs. The somatosensory evoked studied with MRI strictly involve the cervical bilateral vertebral artery dissection. potential on median nerve stimulation cord.2–4 We add another patient with sponta- showed a moderately prolonged central con- P GARNIER neous bilateral vertebral artery dissection in D MICHEL duction time. Urodynamic studies disclosed whom the particularity was an isolated exten- R PEYRON uninhibited neurogenic bladder with detru- sive ischaemia of the spinal cord from C4 to O BEAUCHET sor sphincter dyssynergia. T5 vertebral levels. Department of Neurology Results of the autonomic nervous system A previously healthy 45 year old woman F LE BRAS tests before and 6 months after treatment are had paresthesiae of the right ankle lasting a F G BARRAL given in the table. When the patient was tilted few days. Ten days later, she had a right sided Department of Neuroradiology, University Hospital up to 60 degrees, he experienced dizziness scapulothoracic pain and suddenly developed Saint-Etienne, 42055 Saint -Etienne, France and a significant fall in systolic blood pressure Letters, Correspondence, Book reviews 805 over 30 mm Hg with normal heart rate nal cord induce the dysfunction of postgan- response. His serum noradrenalin concentra- glionic fibres by a trans-synaptic eVect.78 tion was reduced at rest, and its increase after Vitamin B12 is related to the methylation CORRESPONDENCE tilting up was minimal. Sudomotor function reaction regulated by S-adenosylhomocystine was evaluated by sympathetic skin response and S-adenosylmethionine.9 This reaction 3 (SSR) and local sweat response to acetylcho- has a crucial role in the myelin formation 4 line (Ach). associated with neurological deficits in pa- Before treatment, the SSR amplitude was tients with vitamin B12 deficiency. Dysfunc- Sandifer’s syndrome and gastro- decreased, and the number and area of sweat tion in unmyelinated sympathetic neurons, oesophageal reflux disease droplets were decreased in responses to however, has not been shown.
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