Calcification of the Corpus Striatum and Dentate Nuclei Occurring in a Family by J
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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.14.4.253 on 1 November 1951. Downloaded from J. Neurol. Neurosurg. Pgchiat., 1951, 14, 253. CALCIFICATION OF THE CORPUS STRIATUM AND DENTATE NUCLEI OCCURRING IN A FAMILY BY J. FOLEY Fronm the Neurological Department, St. George's Hospital, London Dense symmetrical calcification of the corpus no previous illnesses of note, and on direct questioning striatum is rare, but of some general interest for there was no history of tetany or thyroid disorder. two reasons. First, in a significant number of the There was no family history of mental disturbance, reported cases this apparently degenerative condi- epilepsy, or endocrine abnormality. The patient had had seven children. The first died in convulsion at three tion is closely linked with a metabolic disorder, months, but the remaining six are well. Four years hypoparathyroidism, which may be idiopathic, before admission it was noticed that the patient was parathyroprivic, or of the variety in which the becoming slower in her movements, and that her memory parathyroid glands are histologically normal but was failing. At about the same time she began to have guest. Protected by copyright. the body is unable to respond to parathormone brief attacks of vertigo, in one of which she fell forwards, (Albright's pseudohypoparathyroidism). Secondly, cutting her head and leg; none of these attacks was in no other degenerative disease limited to a neuro- associated with loss of consciousness. One year before physiological system can the process be visualized admission her gait became unsteady, and after a further radiologically. In contrast with the rare gross eight months her legs were so weak and unsteady that under she was unable to climb stairs without assistance. Such calcification discussion, microscopically unsteadiness of the hands as occurred did not prevent demonstrable calcification occurring on a basis hqr from helping with the cooking. Attacks of vertigo, of hyaline degeneration in and around the vessels unassociated with deafness or tinnitus, occurred three in these regions is very common, and can, indeed, or four times daily and caused frequent falls. She also be found to a certain extent in normal brains. complained of feelings of numbness in the tips of the Such calcification formerly attracted much attention fingers and of tingling in the front of the thighs. She in certain inflammatory conditions, chorea, ence- denied impairment of memory or concentration and phalitis lethargica, polio-encephalitis, and cerebral considered her speech to be normal. She had occasional malaria; in intoxications with carbon monoxide, slight frontal headaches. She had never had any fits. nitrous and in condi- No change in facial expression had been noticed. Her oxide, lead; degenerative vision was good, as was her hearing. She admitted to tions such as amaurotic idiocy and the progressive unsteadiness of the hands and to clumsiness and stiffness pigmentary pallidonigral degeneration of Haller- of the legs ; sphincter control was normal. Her general vorden and Spatz , and in certain metabolic and health was good. %-ndocrine disorders than (other hypoparathyroidism) On examination she was seen to be a well-nourished http://jnnp.bmj.com/ such as Addison's disbase, cretinism, myxoedema, woman with a high colour, and healthy hair, skin, and and severe pernicious anaemia.. Calcification in nails; there was no thyroid enlargement, and general the basal ganglia gross enough to be visible radio- examination revealed nothing abnormal. Her blood logically occurs in two main groups of conditions: pressure was 135/60. Mentally she was distinctly dull, in association with parathyroid insufficiency, as with defective insight into her condition. Her memory for recent events was impaired; she was unable to is now well known, and in icerta rAre frnpilial subtract serial sevens, but could retain six digits forwards, instances. It is in the latter, of which the family and reverse three. On the Wechsler-Bellevue verbal reported below is the third in the literature, that intelligence scale her I.Q. was 79 ; there was little on September 25, 2021 by the calcification attains its most florid form. evidence of deterioration, and it was felt unlikely that she had ever been of more than low average intelligence. Case Report There was no dysphasia, dyspraxia, or evidence of a Mrs. M.E., aged 60, was admitted to the Maida Vale specific defect of parietal lobe function. Speech was Hospital for Nervous Diseases, London, under the care slow and slurred. The cranial nerves were normal of Dr. P. H. Sandifer on January 17, 1949. She had had except for a moderate deafness of the right ear due to 253 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.14.4.253 on 1 November 1951. Downloaded from 254 J. FOLEY chronic otitis media; in particular, vision was good; Mallory (1896) showed that hyaline degeneration the fields and fundi ware normal and there was no in the media and adventitia of small vessels, with cataract. The jaw-jerk was brisk, though not unduly so. subsequent deposition of calcium salts, occurs most There was no spontaneous tremor of the upper limbs; frequently in the dentate nucleus, and that the here tone was slightly increased and there was a little weakness of extension of the wrists. Discrete movements droplets may fuse to form sheaths. The process of the fingers were clumsy and there was a well-marked affects the small arteries first, later the capillaries intention tremor, especially on the left. The tendon and veins. The principal difficulty, as later workers reflexes were brisk but equal; there was no grasp reflex. have realized, lies in determining at what stage the Sensation was normal. The abdominal reflexes were proc ss becomes pathological. In 100 brains, present, though sluggish. The lower limbs exhibited a affected by a wide variety of pathological condi- moderate degree of spasticity and weakness, especially tions and of all ages, Hurst (1926) found micro- on the right side, and there was marked incoordination scopical deposits in the vessels of the globus pallidus, in the heel-knee test. The deep reflexes were very brisk, chiefly in the small vessels of the anterior half of the but equal, and both plantar responses were extensor. nucleus, though often with sparing of the extreme Sensation was normal. rostral tip. In 68% there were free-lying, so-called Investigations.-A radiograph of the skull showed a degenerative bodies staining blue-black with haema- calvarium of normal size and shape; the sellar region toxylin (termed by earlier writers chorea corpuscles, was normal. The pineal gland was faintly calcified and Flechsig's granules, or siderophile bodies, and by lay in the normal position. The caudate and lenticular later workers pseudolime or pseudo-calcareous nuclei on each side were heavily and symmetrically in the and in calcified, and the dentate nuclei were similarly calcified concrements) globus pallidus, 30% (Figs. la, lb, and 2). Pneumoencephalography con- such bodies were also to be found in the putamen. firmed the position of the shadows (Figs. 3 and 4). They occurred solely in the putamen in only two guest. Protected by copyright. The size of the ventricles was at the upper limit of normal, cases. They were also to be observed more sparsely and the sulci of the frontal lobes were somewhat widened. in the internal capsule, but they have not been The blood Wassermann and Kahn reactions were nega- reported in the red nucleus, reticular substance tive. The cerebrospinal fluid was under normal pressure, of the midbrain or pons, or in the inferior olives. and normal in composition. The serum calcium level He considered that their distribution could not be was normal on three occasions (9-3, 10-4, and 11 mg. entirely correlated with that of iron, for they were per 100 ml.) and the serum phosphorus was 3-4 mg. never to be seen in the substantia nigra. The per 100 ml. Alkaline and acid phosphatase values were normal (11-5 and 1-7 units respectively). The deposits themselves are highly refractile, often urine was normal. In tests for toxoplasma the comple- laminated, devoid of lipoid, glycogen and amyloid, ment fixation test was negative, and the cytoplasm- and may be minute or sufficiently large to give a modifying antibody test positive 1/8. gritty feel to the knife. Ostertag's (1930) description Because' there was a cholesteatomatous deposit in of them accords well with that of other authors the attic a radical mastoidectomy was performed, after (Pick, 1902, 1903; Bassoe and Hassin, 1921; which the attacks of vertigo ceased. Weimann, 1922; Kasanin and Crank, 1935; Over the past two years the patient's condition has Eaton, Camp, and Love, 1939'; and Rand, Olsen, remained essentially unchanged, and the intracranial and occur in three forms. calcification has shown no increase in extent or Courville, 1943). They density. They may lie free in the tissues, unrelated to the Skull radiographs of her elder daughter (L.N., aged 28) vascular system, varying in size from minute points showed a mottled shadow on both sides in the position to laminated spheres larger than a ganglion cell http://jnnp.bmj.com/ of the anterior part of the lenticular nucleus (Figs. 5a, of the globus pallidus, or as " mulberry bodies " 5b, and 6). In the case of the younger daughter (C.E., formed by the conglomeration of several spheres, aged 21) only a very faint mottled shadow was visible these being encountered only in pathological condi- (Figs. 7a, 7b). Both daughters enjoyed good health, tions. Secondly, there may be deposited upon the were of average intelligence, and displayed no abnormal outer side of capillaries innumerable globules, like physical signs. Three of her four sons. aged 32, 27, and strings of pearls, or, usually in-pathological condi- 26, were examined radiologically, and in none was any intracranial calcification visible.