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Spastic Tetraplegia As an Initial Manifestation of Familial Alzheimer's Disease

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Spastic Tetraplegia As an Initial Manifestation of Familial Alzheimer's Disease J7ournal ofNeurology, Neurosurgery, and Psychiatry 1995;59:395-399 395 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.4.395 on 1 October 1995. Downloaded from Spastic tetraplegia as an initial manifestation of familial Alzheimer's disease N Sodeyama, M Shimada, T Uchihara, K Yanagisawa, H Fujigasaki, K Yamaguchi, M Matsushita, M Yamada Abstract Case reports Two sisters with familial Alzheimer's dis- PATIENT 1 (III-5, FIG 1) ease developed spastic gait disturbance Patient 1 had non-consanguineous parents. as an initial manifestation. Their gait dis- Her history disclosed a fracture of the fifth turbance progressed gradually, followed lumbar vertebra in a traffic accident at the age by dementia a few years later. Post- of 51, and standard radical mastectomy for mortem examination of one of the left breast cancer at the age of 58. Neither was patients disclosed degeneration of the associated with neurological sequelae. Her thalamus and corticospinal tract in addi- first neurological symptom was spastic gait tion to numerous senile plaques and disturbance at the age of 57. At the age of 60, neurofibrillary tangles in the Department of neocortex, memory impairment and decreased mental Neurology, Tokyo both of which were confirmed by activities in daily life were noted. She also Medical and Dental immunohistochemistry. This is the first showed difficulty in dressing and using instru- University, Tokyo, report in which clinicopathological eval- Japan ments of daily life due to spastic paresis in her N Sodeyama uation is sufficient to establish a new upper limbs. Her spastic tetraplegia and T Uchihara variant ofAlzheimer's disease presenting dementia progressed gradually. At the age of K Yanagisawa initially as spastic tetraplegia. H Fujigasaki 64, she became bedridden and showed fre- M Yamada quent nocturnal delirium and emotional (7 Neurol Department of Neurosurg Psychiatry 1995;59:395-399) incontinence. On admission, she was alert but Diagnostic Pathology, disoriented for time and place. She correctly Kanto Teishin named only two of 10 objects presented. She Hospital, Tokyo, Japan Keywords: familial Alzheimer's disease; spastic answered with only a single word to some M Shimada tetraplegia; thalamic degeneration simple questions. She could not repeat a short K Yamaguchi sentence. Her score on the Mini mental state Department of Alzheimer's disease is a degenerative disorder scale was 4. Cranial nerves were normal Psychiatry, Faculty of of the brain characterised clinically by pro- for mild Medicine, University except dysarthria. Weakness, atrophy, of Tokyo, Tokyo, gressive dementia and pathologically by senile postural tremor, hyperreflexia, and muscle con- Japan plaques and neurofibrillary tangles.' 2 tracture in all limbs, and bilateral Babinski's M Matsushita Although some cases with unusual clinico- signs were noted. There was neither sensory Correspondence to: pathological features have been classified as deficit nor cerebellar signs. She was inconti- Dr N Sodeyama, http://jnnp.bmj.com/ Department of Neurology, atypical Alzheimer's disease, such diagnoses nent of urine and faeces. Blood, urine, and Tokyo Medical and Dental are uncertain.34 We describe the clinicopatho- CSF examinations were University, Yushima 1-5-45, unremarkable. An Bunkyo-ku, Tokyo 113, logical findings in two siblings who developed EEG showed a moderate degree of gener- Japan. progressive spastic tetraplegia followed by alised slowing. An EMG and nerve conduc- Received 9 January 1995 dementia. Immunohistochemical examina- and in revised form tion study were normal. Brain MRI showed 1 1 May 1995 tions confirmed the diagnosis of Alzheimer's moderate frontotemporal atrophy (fig 2A). Accepted 1 June 1995 disease. Single photon emission computed tomogra- phy (SPECT) showed diffuse cerebral hypo- on September 28, 2021 by guest. Protected copyright. perfusion. Cervical spinal cord MRI was unremarkable except for mild spinal canal stenosis. She was diagnosed as having atypical Alzheimer's disease. During the next six months her general condition deteriorated further with decubitus ulcers and pneumonia. At the age of 65, she died of empyema. The mI 1 general necropsy showed empyema, multiple erosion of the gastric mucosa, atrophy of the pancreas, small stones in the bladder, myoma v bo uteri, and an adenoma in the thyroid. Gross neuropathology The weight of the unfixed brain was 1030 g. The brain showed no abnormalities exter- nally. Coronal sections of the cerebrum, cere- bellum, and brainstem were unremarkable. [ 0 Unaffected ** Affected / Dead Purulent pachymeningitis at the lumbar level was noted. Transverse sections of the spinal Figure 1 Family pedigree. cord showed no abnormalities. 396 Sodeyama, Shimada, Uchihara, Yanagisawa, Fujigasaki, Yamaguchi, et al Figure 2 (A) Axial Tl J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.4.395 on 1 October 1995. Downloaded from weighted MRI of the brain ofpatient 1 showing moderate frontotemporal atrophy. (B) Plain CT of the brain ofpatient 2 showing the same distribution pattern of atrophy as in patient 1. Microscopic neuropathology Numerous senile plaques and neurofibrillary Jd; tangles were found in the whole cortical grey x.0 matter, caudate nucleus, putamen, hippo- campus, parahippocampus, and amygdala on methenamine-Bodian stain (fig 3). There were neurofibrillary tangles but no senile plaques in the locus coeruleus and neither in -'~~~~~~~~~~~~~~~~.; the substantia nigra. Numerous neuropil i,.~ 4vN 8i threads, diffuse plaques, ghost tangles, and amyloid angiopathy were also seen in the cere- brum. Some senile plaques contained degen- ,§ erative neurites. There was no definite spongy j change except patchy microspongiosis in the ;*! * isi; insula and motor cortex. In the thalamus, there were neither senile plaques nor neuro- fibrillary tangles. Severe astrocytic gliosis and neuronal loss had occurred in the medial part of the bilateral thalamus, especially the medial http://jnnp.bmj.com/ nucleus (fig 4). Neuronal loss in the cortical and tan Figure 3 Numerous senile plaques neurofibrillary locus coeruleus, basal methenamine-Bodian stain; magnification originally x 80 bar 50tpm. grey matter, amygdala, nucleus of Meynert, putamen, caudate nucleus, and hippocampus was mild. Neurons in the substantia nigra were preserved. The cerebral white matter was normal. There were no senile plaques or neurofibrillary tangles in on September 28, 2021 by guest. Protected copyright. 4 @- 2. the cerebellum. Betz cells of the motor cortex, *Ri B} 2S :'c: .: in i;| .! the internal capsule, and pyramidal tract s ^ e e the brainstem were preserved (fig 5A). .* rS .;g t R lj; * * and ilF Neuronal loss, mild astrocytic gliosis, si & ,,1s, w , :o- .* *- X were a .,, i. ,. slight spongiform change, however, A h St :. ffi .>z:e found in the external granular layer of the $ 4i ee motor cortex (fig 5B). The corticospinal tract iS * * ., tf :e is at all s ! IaW 4F showed pronounced symmetric pallor A similar g s J , qlr dk < EEi ' :blS, e; levels of the spinal cord (fig 6). in the bilateral anterior ,. was found .IS change * 4Nt ., ,2 b- ilb 4' funiculus. Anterior horn cells were intact Sl it t 4lr - . except for mild gliosis and some central chromatolysis. t:. To characterise senile plaques and neu- o..*:.. immunohistochemical :.-,>....:::. rofibrillary tangles, studies using antibodies to amyloid ,B protein,5 human tau protein,6 and prion protein7 were performed as previously described.78 Senile and the walls of the vessels were Figure 4 Section of the medial nucleus of the thalamus s,howing severe astrocytic gliosis plaques n x = tm. to amyloid /B andVneuronal8sS.loss;..;ehaematoxylin-eosin4,,<^'S4':a:.,^*.,n.stain;W:iws magnificatio originally 50; bar 50 immunostained by antibody Spastic tetraplegia as an initial manifestation offamilial Alzheimer's disease 397 Figure 5 Section ofthe J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.59.4.395 on 1 October 1995. Downloaded from motor cortex. (A) Betz ceUls were wellpreserved. The amount oflipofuscin deposited in the cytoplasm ofBetz cells is consistent with the patient's age; haematoxylin-eosin stain; magnification originally x 50; bar = 50 pm. (B) Neuronal loss, mild astrocytic gliosis, and slight spongiform change in the external granular layer are shown; haematoxylin-eosin stain; magnification originally x 20; bar = 200 pm. .1~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~. protein, but not by antibody to prion protein. in the lower extremities, diffuse hyperreflexia, Degenerative neurites of senile plaques and bilateral Babinski's sign, and dementia (a total neurofibrillary tangles were labelled by anti- IQ on the WAIS was 65). No sensory deficit body to human tau protein. Western blot was detected. Laboratory investigation was analysis of the brain tissue excluded the pres- unremarkable and EEG, EMG, and a nerve ence of an abnormal prion protein.7 conduction study were normal. Brain CT showed moderate frontotemporal atrophy (fig PATIENT 2 (III-3, FIG 1) 2B). Her neurological symptoms worsened The elder sister of patient 1 developed spastic gradually. At the age of 64, she became gait disturbance at the age of 53. At the age of bedridden. She could follow only simple com- 59, memory impairment, lack of initiation, mands and showed frequent emotional incon- and deterioration in homemaking perfor- tinence. On neurological examination at the http://jnnp.bmj.com/ mance were noted. Neurological evaluation at age of 67, she had lost all ability to communi-
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