Biomedical subjects
Publications and source records attributed to J Haan.
Two patients with cerebral venous thrombosis are described. In both patients laboratory findings suggested an underlying haematological disorder and bone marrow biopsy showed a myeloproliferative disorder. Both chronic cerebral venous thrombosis and early myeloproliferative disorders are difficult to diagnose. Their combined occurrence may be less rare than is frequently supposed.
We describe a patient who suffered from ophthalmic trigeminal neuralgia as an isolated symptom of a noninvasive pituitary tumor. Bromocriptine appeared to provoke the attacks within hours. This provocation could be prevented by domperidone. After adenomectomy, both spontaneous and induced attacks disappeared. Acute transient cell swelling may be the mechanism by which bromocriptine induced the neuralgic attacks.
In a prospective study on the effect of shunting, 22 patients diagnosed as suffering from normal pressure hydrocephalus (NPH) were investigated by means of temporary external lumbar drainage (ELD). Five patients had to be excluded from the study because of complications of ELD or definitive shunting. ELD correctly predicted the outcome of shunting in all of the remaining 17 patients. The value of external lumbar drainage in NPH is discussed on the basis of personal experience and data from the literature. It seems to be a safe and valuable tool for predicting the outcome of definitive shunting procedures.
Examining the members of a family the validity of chemical analysis, neurophysiology and neuroimaging for the diagnosis of metachromatic leukodystrophy (MLD) is discussed. As the arylsulfatase A is not decreased in all cases, the neuroimaging (cranial computerized tomography--at a less extend magnetic resonance imaging) gains a particular diagnostic significance. But neither for neuroimaging nor for neurophysiological findings the results are specific. The histological findings have to back up the diagnosis.
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Methods which produce findings relevant to diagnosis and therapy of cervical vertebral canal stenosis are described. Especially MRI provides insights into the structure of the myelon; abnormal image signals may possibly correlate with cervical myelopathy based on disorders of microcirculation.
Clinical examination, spinal fluid laboratory data, evoked potentials (EP), cranial computed tomography (CT), and magnetic resonance imaging (MRI) were compared for their value in diagnosing multiple sclerosis (MS) in 51 patients aged 18-60 yrs. MRI was superior to CT imaging: supratentorial lesions were found in 90% of the patients with confirmed MS. These lesions are not specific, however, as vascular processes, vitamin B12 deficiency or chronic encephalitis, can show similar MRI-patterns. The detection of infratentorial lesions in symptomatic patients was poor (8% compared to 64% pathological EP-findings). Thirty-eight patients (= 75%) could be classified correctly as "confirmed MS" according to Bauer-criteria without any CT or MR imaging. In the remaining 13 patients presenting classificatory problems due to normal CSF, first manifestation or possible alternate processes, MRI helped for the final diagnosis in only 6 cases. The value of MRI in diagnosing MS seems to lie in its superiority to CT in excluding other pathological processes or for research rather than in its value for the confirmation of the diagnosis. In a minority of cases, it can detect supratentorial lesions in patients with pure spinal symptoms, normal CSF, or first manifestations.
Five Patients with vitamin B12 deficiency were examined by means of MRI, CCT, VEP, BAEP, EEG, and CSF. In 3 patients medianus-SEP and EMG/ENG were recorded as well. Partly, findings of MRI, CCT, VEP, BAEP, SEP, and CSF were similar to those in multiple sclerosis. This is not very surprising considering that the central nervous system lesions in vitamin B12 deficiency consist of disseminated demyelination. Because of this all these investigative techniques must be considered non-specific. Appraisal of findings is only possible in connection with the case history, clinical findings, and supplementary diagnostic measurements.
Two patients with idiopathic normal pressure hydrocephalus are described suffering from so called 'falling spells'. Both patients showed marked improvement after ventriculoatrial shunting, and the falling spells ceased. The literature concerning falling spells is reviewed. The possible pathogenesis and the relationship to shunting are discussed.
In any patient with unexplained basal ganglia symptoms or dementia, the possibility of idiopathic hemochromatosis (IHC) should be considered. The findings of magnetic resonance tomography, discussed in this case report, lead to the conclusion that IHC is very likely to induce these syndromes. This is of clinical importance as IHC can be successfully treated by phlebotomy.
A case of central pontine myelinolysis in a 38-year-old alcoholic is reported. The findings initially and over a course of almost 2 years, including clinical data, magnetic resonance imaging (MRI), cranial computerized tomography (CCT), evoked potentials (BAEP, VEP, SEP), EEG and EMG/ENG are presented and discussed in relation to other recent case reports, with special regard to the CCT findings. The important role of CCT is emphasized, although negative CCT findings probably do not allow exclusion of the diagnosis. MRI can be expected to play an important part in the detection of central pontine myelinolysis. The importance of BAEP as a possible method for monitoring must be clarified by further research.
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Investigations were made on 113 patients suffering from CFS. Definite or highly probable diagnosis often was set up by anamnesis, observation and clinical findings. In 93.8% the EEG was abnormal (seizure patterns 35.8%, focal abnormalities 5 8.4%, focal seizure discharges 14.1%). CCT showed pathological findings only in 58.4%. It was unavoidable in the diagnosis of tumors which were rarely found (8.8%). It showed localised brain lesions of various etiology in 31.8%, seldom perinatal brain damage or unspecific hydrocephali (17.7%). It is concluded that anamnesis, observation, clinical findings and EEG are the primary diagnostic steps in suspected CFS. EEG is the best for the patients survey, CCT, even if mostly indispensable because of its high evidence in morphological brain impairment often is of little or no use in he diagnosis of CFS, EEG and CCT are unavoidable in medical certificates.
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