Biomedical subjects
Publications and source records attributed to M Trillet.
A 33 year-old woman with multiple sclerosis developed bilateral palatal myoclonus. Rotatory pendular nystagmus and gaze-evoked nystagmus were present and analyzed by electro-oculographic recordings. Magnetic resonance imaging demonstrated abnormal signals in the pons. To our knowledge, this is the first report of palatal myoclonus in the course of a definite case of multiple sclerosis.
A 47 year-old man developed rapid visual loss, visual field defects and memory disturbances after radiotherapy with conventional doses for a pituitary metastasis from a renal carcinoma. CT and MRI did not show recurrent tumour, pituitary apoplexy or empty sella. Eventually, T2-weighted MRI images showed abnormal high signals in the optic chiasm, the left mesial temporal lobe and the right inferior frontal lobe, supporting the diagnosis of delayed radionecrosis. The role of chemotherapy associated with radiotherapy is discussed.
Neck pain and torticollis are very common symptoms, but they may reveal an intracranial or cervical neurological disease. Such diseases are easily diagnosed when these two symptoms are associated with others, but in some rare cases, and especially when it is isolated, torticollis may be mistaken for common neck pain. This is what happened in 21 cases and in some patients the neurological disease had been present for a very long time before it was diagnosed. Prior to admission, most patients were treated with massages, cervical collar or manipulations which sometimes resulted in deterioration. A search for atypical features of neck pain and torticollis should avoid delays in diagnosis.
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A 76-year-old man suddenly presented with loss of motivation for speaking. He had no aphasia, depression or dementia, but he did not want to speak with other people. CT scan showed hypodensities, presumably lacunes, mainly in the anterior limb of the two internal capsules. Global behavioral changes with loss of self psychic activation have been reported, but in our patient the disturbance was restricted to oral language. The role of activating cortico-subcortical loops is discussed.
We report two clinico-pathological cases with an initially hidden brainstem tumour presenting as chronic hydrocephalus of "idiopathic type". Diagnosis was established respectively one and two years after a successful shunting procedure, as repeated CT scan was performed because of gait deterioration. The first case was a bifocal glioblastoma invading the leptomeninges of the posterior fossa and spinal cord, and resulting in a communicating hydrocephalus. The second case was an ependymoma of the fourth ventricle leading to e non-communicating hydrocephalus. Rarity of such cases is emphasized.
A patient presented with dysorthographia associated with amnestic aphasia due to a left temporo-parietal hematoma. Reading, copying, graphism of letters were normal. Dictation showed better results for logatomes and regular words, without orthographical difficulty, than for those with orthographical problems (ambiguous and irregular words). The overall features suggested a disorder of the lexical system in which orthography of words known to patients is present. The phonological pathway of phoneme-grapheme conversion was preserved since errors were usually phonologically correct. Initially it was possible to conclude to a disorder of the semantic value of words in their visual form. Oral spelling, auditory reading and written denomination also showed more errors for words of difficult orthography. This patient presented a disorder of visual representation of words. Comparison of this case with the few reported ones showed that the latter often had amnestic aphasia and differed from our case in the associated lexical or phonologic alexia. A common feature of the lesions is involvement of the left angular gyrus is right-handed patients.
Pure agraphia is a disorder of written language without aphasia, alexia or apraxia. Its existence has long been disputed. We report 2 cases due to a left parietal hematoma. In the first patient, with a deep precuneate lesion, agraphia included disorders in handwriting and orthographic problems in copying or writing from dictation with the two hands. The second patient, with a lesion of the postero-inferior part of the left angular gyrus, made errors in choice and combination of letters during dictation, whereas these same letters were well-written and copying was normal. The first cases of pure agraphia were ascribed to frontal lesions and then confusional states. Current opinion tends to implicate parietal lesions of various types: superior or inferior, internal or external. Findings in the present 2 cases and those reported indicate that pure parietal agraphia is heterogeneous.
A magnetic resonance imaging study was performed in three symptomatic members of two families with a diagnosis of acetazolamide-responsive familial paroxysmal ataxia. A selective atrophy of the cerebellar vermis, mostly of the anterior part, was demonstrated in the three cases. The first ample documentation of a neuroanatomical abnormality in this condition was achieved in this study.
Particular clinical pictures of ALS may occur during the course of some "benign gammapathies". We observed 12 patients (age range 57 to 74 years; 9 men-3 women) with initially benign gammapathy (9 IgG, 1 IgA, 2 IgM) associated to a clinical picture of progressive anterior horn and pyramidal tract involvement. These cases led us to recognize some particularities of gammapathy-associated ALS: Relative frequency of asymmetrical clinical manifestations, rarity of bulbar signs; Decrease of sensory nerve conduction velocities without evidence of sensory clinical symptoms (8 out 9); Increase CSF protein content including the monoclonal component, axonal degeneration and immunostaining evidence of the paraprotein fixation observed on nerve biopsies (5 out 7 cases). Association of ALS and gammapathy is not fortuitous as shown by epidemiology, experiments, pathology and effects of different immunological treatments as related in this study. Demonstration of infra-clinical neuropathy face to a clinical syndrome of ALS should prompt too careful screening for a gammapathy.
A 31 year-old patient suffered from a subacute and major dementia, sixty months after whole brain irradiation with 54 grays for a pinealoma. Clinical features and biological investigations led to a diagnosis of hypothalamic insufficiency. A dramatic clinical recovery followed therapy with hydrocortisone and thyroxine. An hypothalamic radionecrosis and a vascular mechanism are presumed.
Cerebral blood flow determinations using the Xenon inhalation test in two patients with transient global amnesia showed overall decreases during the attack with a more marked fall in temporal-inferior frontal flow in one case and in temporal flow in the other. In the latter patient the temporal anomaly was reversible after naloxone injection but clinical improvement was lacking. Blood flow determinations in a further patient with transient global amnesia, after 2 mg of ergotamine tartrate plus caffeine, showed a global decline in flow without focal anomaly, with recovery after ten days. Values obtained in two other patients 24 and 38 hours respectively after typical transient global amnesia attacks were normal. The effect of naloxone appears related to a direct neuronal action although the role of endorphins in this affection is not conclusive. Comparison of these anomalies with those noted by Reichle with position emission tomography imaging suggests a primary neuronal mechanism.
The electrophysiological findings in 18 patients with Arnold-Chiari malformation (ACM) revealed by spinal symptoms excluding syringomyelic syndrome (Brown-Sequard syndrome, paraparesis with or without posterior column involvement, motor neuron syndrome or more complex myelopathies) are reported. In 16 cases, EMG disclosed abnormalities consistent with lesion of anterior horn cells restricted to upper limb in 7 cases, and generalized in 9 cases. In 2 cases, motor and sensory conductions were abnormal. The occurrence of such abnormalities in patients with ACM is emphasized as they could lead to confusion with amyotrophic lateral sclerosis or spinal muscular atrophy. Neuroradiological studies are therefore necessary owing to possible surgical treatment. The hypothesis relating lower motor involvement and ACM are reviewed.
A 26-year-old male with a history of pulmonary sarcoidosis showed clinical, myelographic and intra-operative evidence of a C5-C6 spinal cord tumour, which was diagnosed by biopsy as a pure intramedullary granuloma. Among the 29 published cases of histologically proven cord sarcoidosis, only five presented with pure parenchymatous infiltration without meningeal involvement. Corticosteroids are the most useful therapy, and surgery is indicated only for minimal biopsy when the parenchyma is invaded.
Presenting symptoms in 4 patients with acute benign cerebral angiopathy were headaches over several days, with a transient neurologic disorder in two cases. Angiography showed diffuse and segmental distal narrowing alternating with dilatations. In one patient, the radiologic anomalies persisted to a milder degree in the same region 2 months later, while the reduced cerebral blood flow failed to return to normal. The pathophysiology of this syndrome would appear to result initially of vasospasm in particularly reactive subjects, especially young women. Its cause appears to vary from one case to another: post-partum disorders, infection or inflammation, meningeal hemorrhage, paroxysmal hypertension. Adjuvant factors could be unusual effort, medication or a migraine context. In spite of this pathogenetic uncertainty this angiopathy in usually considered as an isolated, benign and non-relapsing disease.
UNLABELLED: This family is the tenth reported world-wide and the second reported in Europe. Two of 3 brothers, their mother and probably their maternal grandmother were affected. CLINICAL FEATURES: paroxysmal bouts of ataxia and dysarthria, nystagmus of permanent gaze, the dominant autosomal transmission, the benign nature of the affection and the remarkable efficacy of acetazolamide were typical of familial paroxysmal ataxia. Specific points emphasized are the presence of a mild pyramidal syndrome as a permanent finding in 2 of these patients, and the anomalies of visual (longer lag period) and somesthetic (slowed conduction rate in the lemniscus medialis) evoked potentials probably the result of extension into other pathways than the cerebellar system. The finding of some degree of glucose intolerance, noted previously (Aimard, Vighetto et al., 1983) raises the question of the place of this disease in the group of paroxysmal ataxias due to pyruvic metabolism disorders.
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