Fifty neurological cases from the National Hospital by Adrian J. Wills, C. David Marsden

Fifty neurological cases from the National Hospital by Adrian J. Wills, C. David Marsden

By Adrian J. Wills, C. David Marsden

Using the wealth of fabric which has been provided by way of clinicians on the Grand Rounds on the nationwide health center for Neurology and Neurosurgery through the years, the editors have chosen fifty situations on which advisor neurologists at this famous establishment have supplied their specialist reviews every one case is gifted within the similar structure: heritage, exam, investigations, analysis, remark by means of knowledgeable from the health center and illustrations

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Fifty neurological cases from the National Hospital

Using the wealth of fabric which has been awarded by way of clinicians on the Grand Rounds on the nationwide health facility for Neurology and Neurosurgery through the years, the editors have chosen fifty instances on which advisor neurologists at this famous establishment have supplied their professional reviews each one case is gifted within the related layout: historical past, exam, investigations, analysis, remark by way of knowledgeable from the medical institution and illustrations

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Presentation in adult males is not uncommon. This is usually as an isolated, slowly progressive spastic paraparesis (adrenomyeloneuropathy). A similar presentation may also occur in heterozygous adult females. As in this case, there may be evidence for hypoadrenalism in laboratory tests, and there may also be clinical concomitants including hyperpigmentation. A positive family history may be an im portant diagnostic clue. The key investigation is measurement of serum very long chain fatty acids (VLCFA), which are elevated.

Characteristic serpiginous lesion on the cord surface demonstrated on an MRI scan. arteriovenous shunt at the level ofT 12 on the right. The shunt was closely associated with the neural foramen and there was evidence of reflux into epidural and spinal veins. In view of these findings, the shunt was successfully embolised by selective catheterisation of theT12 intercostal artery. Subsequent to this procedure the venous phase of angiography was seen to normalise, indicating successful closure of the fistula.

Ann Neurol 1983 ; 13: 232^ 4- 2 . 35 C ase 12 H isto ry A 3 3-year-old woman had normal early milestones and was good at sport when at school. At the age of 12 years she noticed stiffness of both calves after physical exertion. Her parents commented that her calves looked very ‘m uscular’ . Four years later she noticed difficulty running and by the age of 18 years her gait was described as waddling. From the age of 26 years she began to complain of upper limb weakness. Within 2 years she was walking with the aid of two sticks.

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