PART 4 in Microsoft Office

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dystonia of the neck, ocular palsies, or a picture resembling pseudobulbar palsy If the classic abnormalities of eye movements are present, the diagnosis is not dif cult When only a parkinsonian syndrome without tremor is present, the main diagnostic consideration is striatonigral degeneration or the corticobasal-ganglionic syndrome, described below Treatment L-Dopa has been of slight but unsustained bene t in some of our patients, and combinations of L-dopa and anticholinergic drugs have been entirely ineffective in others A marked response to these drugs should, of course, suggest the diagnosis of Parkinson disease Recently, the drug zolpidem, a GABA-nergic agonist of benzodiazepine receptors, has been reported to ameliorate the akinesia and rigidity of PSP (Daniele et al); however, these observations require corroboration Benztropine or trihexyphenidyl have been somewhat helpful in reducing dystonia but botulinum injections may be a better alternative if there is focal affection Treatment of the sleep dif culties and urinary incontinence are of great assistance to the patient and family A feeding tube becomes necessary in advanced cases

Over the years, the authors have observed several elderly patients, both men and women, in whom the essential abnormality was a progressive asymmetrical extrapyramidal rigidity combined with signs of corticospinal disease Sometimes a mild postural action tremor (beginning unilaterally and suggestive in some respects of Parkinson disease) was added The parkinsonism is generally unresponsive to L-dopa These cases have come to be known on our neurology service by the names corticobasal-ganglionic or cortical-basal degeneration The clinical relation of such cases to corticostriatospinal degeneration, described earlier, is indeterminate, and more interesting, based on the nding of tau inclusions, is a possible biologic relationship to frontotemporal dementia, Pick disease, and to progressive supranuclear palsy, as noted below The patients, though able to exert considerable muscle power, cannot effectively direct their voluntary actions Attempts to move a limb to accomplish some purposeful act might result in a totally inappropriate movement, always with great enhancement of rigidity in the limb and in other affected parts, or the limb may drift off and assume an odd posture, such as a persistent elevation of the arm without the patient s awareness a kind of involuntary catalepsy The disorder of limb function has some of the attributes of a limb-kinetic or an ideomotor apraxia (see Chap 3), but the hand postures, involuntary movements, and changes in tone are at times more reminiscent of what has been described as the alien hand Some patients exhibit an anosognosia Babinski signs, impaired eyelid or ocular motion (up-gaze paresis or abnormal saccadic movements), or lingual dyskinesias, frontal release signs, and dysarthria occur in some affected individuals Another distinct group had dementia as an early feature, as described by Grimes and colleagues With progression of the disease, the limbs rst on one side of the body and then on both and the cranial muscles become involved; apraxia and variable combinations of rigidity, bradykinesia, hemiparesis, sensory ataxia, and postural and action tremor and sometimes myoclonic jerking nally render the patient helpless unable to sit, stand, speak, or take care of basic needs Apraxia of gaze, eyelid opening and closure, and stimulus-sensitive myoclonus appear in some cases Mental deterioration occurs late and only in some cases Occasionally, there is some involvement

of lower motor neurons with resulting amyotrophy Two of our patients had myoclonus as an early feature, one displaying it only on one side of the face, the other in an arm The condition progresses for 5 years or more before some medical complication overtakes the patient Wenning and colleagues have described a series of 14 such patients in whom the diagnosis was con rmed at postmortem examination The most common early symptom was an asymmetrical clumsiness of the limbs, in half of the patients with rigidity and in one- fth with tremor; these features are now considered to be the most characteristic early features of the process As the illness progressed, almost all the patients developed an asymmetrical or unilateral akinetic-rigid syndrome, which may be considered the essential motor disorder of this disease; various forms of gait disorder; and dysarthria Stimulus-induced or spontaneous myoclonus and pyramidal signs, mentioned in other reports and frequent in our cases, were not prominent, and fewer than half the patients exhibited an alien-hand phenomenon; limitations of vertical gaze and frontal lobe release signs eventually became apparent in half of the patients Postmortem examination of several of our colleagues patients, reported by Rebeiz and colleagues, has disclosed a combination of ndings that stamps the disease process as unique Cortical atrophy (mainly in the frontal motor-premotor and anterior parietal lobes) was associated with degeneration of the substantia nigra and, in one instance, of the dentatorubrothalamic bers The loss of nerve cells was fairly marked, but there was no gross lobar atrophy, as occurs in Pick disease The neuronal degeneration was more on one side of the brain than the other There was moderate gliosis in the cortex and underlying white matter Many of the residual nerve cells were swollen and chromatolyzed with eccentric nuclei, a state that was called achromasia by Rebeiz and colleagues; it resembled the central chromatolysis of axonal reaction More recently, in over half the cases, the affected neurons and adjacent glia have been shown to be lled with a particular con guration of tau protein, thereby linking the disease to frontotemporal dementia and Pick disease, as already mentioned The remaining cases have shown tau deposition that is more similar to that of progressive supranuclear palsy, or Alzheimer pathology, or nonspeci c cell loss with replacement gliosis Both CT and MRI have demonstrated asymmetrical cerebral and pontine atrophy, and PET studies have revealed thalamoparietal metabolic asymmetries a greater reduction of glucose metabolism on the side of the most extensive lesion (Riley et al) In subsequent observations of such cases, other investigators have searched unsuccessfully for clues as to the cause and pathogenesis of this disease There is no family history No organ other than the CNS is affected The progression is relentless None of the drugs in common use for spasticity, rigidity, and tremor has been helpful We know of no attempt to transfer the disease to primates Marinescu has described a somewhat different disease resembling more a severe form of Alzheimer disease, but with signs of both pyramidal and extrapyramidal disease (rigidity, tremor, nystagmus, incoordination, confusion, disorientation, and loss of memory) There was amyloidosis of blood vessels (so-called Scholz s perivascular plaques) in the cerebral white matter as well as in the liver and kidney The disease bears some resemblance to, and may indeed be identical to, the corticostriatospinal degeneration ( spastic pseudosclerosis ) of Jakob, discussed on page 913 The relation of this disease to the corticopallidospinal and the pallidopy-