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Language, praxis, and the right hemisphere: clues to some mechanisms of consciousness.

The linguistic capacity of each separate cerebral hemisphere was examined in a 15-year-old, callosally sectioned, normally right-handed male. The results demonstrated that while the right hemisphere was not capable of expressive speech, it could comprehend nouns and verbs, and also possessed the motor engrams necessary to carry out verbal and pictorial commands. In addition, the mute hemisphere was found to be capable of spelling the names of visually presented items by arranging letters as well as by writing with the left hand. Finally, the manner in which the left hemisphere dealt with the overt bodily response to commands presented to the right hemisphere suggested clues to what we feel are mechanisms by which a personal sense of conscious reality is created in the normal brain.

Adolescent↗

Right unilateral dyscopia of letters in alexia without agraphia.

In two cases of pure alexia with computed tomography (CT) evidence of lesions in the left occipital lobe, the left hand copied letters normally, but the right hand copied defectively. The findings reinforce the disconnection theory of alexia without agraphia.

Aged↗

Hemispheric control of the writing hand: the effect of callosotomy in a left-hander.

We report a case of a left-hander with left hemispheric language. After callosotomy, he could use the left hand to write only random letters and digits, and he became right-handed for writing. Therefore, writing by left-handers with left hemispheric language dominance may be accomplished by transcallosal transmission of the linguistic content from the left hemisphere to the right.

Adult↗

Primary writing tremor and myoclonic writer's cramp.

Four patients with primary writing tremor had a focal, task-specific tremor that responded to anticholinergic drugs. Physiologic features included EMG activity alternating in antagonist muscles, 5- to 20-microV cerebral potentials evoked by stretch of pronator teres, and no C-reflexes. Another patient had myoclonic jerks of the forearm on attempts to write ("myoclonic writer's cramp") that also responded to anticholinergic drugs; EMG activity appeared synchronously or alternating in antagonist muscles. These disorders have features of dystonia and enlarge the spectrum of writer's cramp.

Adult↗

Kinetic predominant essential tremor: successful treatment with clonazepam.

Fourteen patients with marked kinetic tremors of long duration but no other major neurologic signs are described. A positive family history of essential tremor, mild postural tremor, and tremor suppression with alcohol suggest that the condition is a variant of essential tremor. Kinetic tremors had a frequency of 3.5 to 6.0 Hz and an alternating EMG pattern. Propranolol caused improvement in some patients, but clonazepam treatment resulted in tremor suppression in all patients. Kinetic tremor without cerebellar signs is a subtype of essential tremor with pharmacologic responsiveness to clonazepam.

Aged↗

Focal task-specific tremor and dystonia: categorization of occupational movement disorders.

We studied 28 patients with focal task-specific disorders. Three groups were identified, according to the occurrence of focal tremor alone (group I, n = 10), dystonia alone (group II, n = 8), and tremor-dystonia combination (group III, n = 10). Four of six patients in groups I and III showed improvement with beta blocking agents alone, and another patient improved with primidone and metoprolol. Four of seven patients in groups II and III improved at least moderately with only anticholinergic medication. The coexistence of focal tremor and focal dystonia in the same limb in 10 patients (group III) suggests a link between these focal disorders. We conclude that focal tremor and focal dystonia are related to generalized essential tremor and generalized dystonia. A scheme which conceptualizes these interrelationships is proposed.

Adolescent↗

Usefulness of a writing device in writer's cramp.

Fifteen of 20 patients with writer's cramp treated with a writing device noted an improvement in their writing ability, some quite marked. Despite this benefit, nine patients preferred to use alternate methods. In the absence of adverse effects, we suggest that trials of writing devices be the first recourse for all patients with writer's cramp requiring treatment.

Adult↗

The agraphia of Alzheimer's disease.

Hypothesizing that agraphia in Alzheimer's disease (AD) reflects disturbances in multiple cognitive domains, we evaluated writing samples from 33 patients meeting strict criteria for probable AD. We found agraphia to be common on a standard narrative writing task. When compared with 41 education- and age-matched normal control subjects, AD patients had significantly lower writing scores, wrote significantly fewer words, mentioned significantly fewer categories of information, and were significantly more likely to make writing errors. On stepwise regression procedures, neuropsychological measures of visuoperceptual impairment and disease severity were the strongest predictors of agraphia, but other analyses indicated that measures of language, praxis, and attention could also contribute significantly to agraphia. On two writing tasks, we failed to confirm the previous contention that agraphia is a marker for familial AD. However, there was a highly significant interaction between family history, oral naming, and writing: patients with nonfamilial AD, but not those with a family history of dementia, showed a strong correlation between naming and writing performance. We conclude that agraphia in AD can be variously determined and that agraphia is not a reliable marker for familial disease.

Aged↗

Micrographia due to focal cerebral lesions as seen in the dysarthria-clumsy hand syndrome.

Five patients with dysarthria-clumsy hand syndrome developed micrographia. Four had lacunar infarctions involving the putamen or the genu of the internal capsule; the fifth patient had a small hemorrhage in the putamen. Micrographia may be a sign of focal cerebral disease, and basal ganglia dysfunction may be involved in the supratentorial dysarthria-clumsy hand syndrome.

Aged↗

The syndrome of 'pure akinesia' and its relationship to progressive supranuclear palsy.

Five patients developed a stereotyped syndrome of progressive akinesia of gait, speech, and hand-writing without rigidity, tremor, or dementia. The symptoms did not improve with levodopa. These clinical findings conform to a syndrome described repeatedly in Japan since 1974 as "pure akinesia." Evidence has indicated that pure akinesia often represents a pre-ocular motor, and in some cases an ocular motor-sparing, form of progressive supranuclear palsy (PSP). Although we found disorders of eyelid movements, none of the patients demonstrated a gaze palsy on clinical examination. Four patients underwent eye movement recordings. The two patients with the longest disease duration had slow or small vertical saccades. These findings support the notion that patients with pure akinesia may develop a vertical gaze palsy, similar to that in PSP, late in their course. Our patients show that pure akinesia occurs in North America as well as in Japan. Recognition of the syndrome of pure akinesia may suggest the diagnosis of PSP before the development of abnormalities of ocular movement.

Dementia↗

Impaired inhibition in writer's cramp during voluntary muscle activation.

We used paired transcranial magnetic stimulation (TMS) to evaluate inhibitory mechanisms in eight patients with writer's cramp during rest and isometric wrist extension. Both stimuli were 110% of the motor threshold; the interstimulus intervals (ISIs) were 20 to 200 ms in increments of 10 ms. Surface EMG was recorded from wrist extensors. In the symptomatic hemisphere, there was no significant difference in the amplitude of the test (second) motor evoked-potential (MEP) between patients and age-matched controls at rest. However, with voluntary muscle activation, inhibition of the test MEP by the conditioning MEP was significantly less in writer's cramp patients than in controls (p = 0.02). The difference was most prominent at ISIs of 60 to 80 ms in which inhibition is maximum. In the asymptomatic hemisphere, there was no significant difference between patients and controls in both rest and active conditions. The silent period was shorter in patients than controls on the symptomatic side (p = 0.003) but not on the asymptomatic side. We conclude that the inhibitory effects induced by magnetic stimulation are reduced in patients with writer's cramp, but only on the symptomatic side during muscle activation. This may relate to the overflow of muscle activity that characterizes this condition.

Adult↗

Micrographia after thalamo-mesencephalic infarction: evidence of striatal dopaminergic hypofunction.

A patient with left thalamo-mesencephalic infarction presented with micrographia in the right hand as the only motor sign. Brain MRI and 99mTc ethyl cysteinate dimer (ECD) perfusion SPECT revealed ischemic lesions in the left midbrain and the anterior thalamus, but not in the basal ganglia, whereas [123I]-IPT SPECT demonstrated decreased activity of the [123I]-IPT in the left striatum. The patient's micrographia may be related to a dysfunctional nigrostriatal dopaminergic system secondary to ischemic damage to the substantia nigra.

Cerebral Infarction↗