Charcot-Marie-Tooth disease associated with Type 2 diabetes mellitus.
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Aged↗
Biomedical subjects
H Forta
Publications and source records attributed to H Forta.
Middle latency auditory evoked potentials in patients with parkinsonism.
The aim of this study was to assess the middle latency auditory evoked potential (MLAEP) findings in idiopathic Parkinson's disease (IPD) and in patients who are regarded as having atypical parkinsonian disorders (AP) and to determine whether MLAEPs could contribute to the differential diagnosis of IPD and AP.MLAEPs were evaluated in 19 control subjects and in a total of 35 patients with parkinsonism, of which 27 had IPD and 8 had AP. Among IPD patients, P1 was absent in 1 nondemented patient with young-onset Parkinson's disease (YOPD) (5%) and in 2 of 7 demented (28.5%) IPD patients. In the AP group, 3 of the 7 (42.8%) nondemented patients and the one patient with dementia showed the absence of P1. The absence of P1 was found to be significantly higher in AP patients than IPD patients (p=0.0335).In conclusion, MLAEPs were found to be normal in nondemented IPD patients with only a few exceptions. The absence of P1 in nondemented patients with parkinsonian symptoms may bring the diagnosis of IPD into question. The absence of P1 could be detected in AP patients at least as often as in demented IPD patients. Thus, the measurement of MLAEPs may be a clinically useful adjunct to the clinical examination of patients with parkinsonism.
Journal Article↗
Reflex occipital lobe epilepsy.
Photosensitivity is a typical feature of photosensitive epilepsy which is usually considered a form of idiopathic generalized epilepsy. Partial seizures featuring visual symptoms are rarely reported in photosensitive epilepsy. In this study, we describe 13 neurologically normal patients in whom daytime seizures were always induced by television and began with elementary visual hallucinations, followed frequently by vomiting, headache and then secondary generalization. Three patients additionally reported nocturnal seizures, which have not been described in previous studies. Two of these latter patients had generalized tonic-clonic seizures, the other always awoke from sleep and could describe typical visual hallucinations at the beginning of the seizure. EEG features included normal background activity and occipital spikes or spike-waves in all but two patients. Eight patients also showed generalized epileptiform activity during intermittent photic stimulation. Seizure frequency was low in all. Apart from two patients, who refused treatment, all patients received antiepileptic drugs. Only one patient continued to have rare seizures after treatment; in the others seizure control was achieved with monotherapy. We conclude that reflex occipital lobe epilepsy is an idiopathic form of the benign partial epilepsies, which may overlap with one another.
Adolescent↗
The development of synkinesis after facial nerve paralysis.
In this study, the development of clinical synkinesis after facial nerve paralysis (FP) and its relationship to electrophysiological findings were investigated. Thirty-four patients who were examined within the first 5 days after onset of FP and who could also be followed up for at least 4 months were included in the study. Electrophysiological investigations consisted of: (1) recording of the direct responses by facial nerve stimulation at the stylomastoid fossa; (2) recording of the 'synkinetic spread' of the supraorbital nerve reflex to the lower facial muscles; (3) recording of the 'lateral spread responses' by stimulating the mandible and zygomatic branches of the facial nerve. Clinical synkinesis developed in 14 of 18 patients (78%) with a direct response ratio (DRr) of less than 40%. Among the 16 patients with a DRr of 40% or more, synkinesis was observed in 3 cases (18.7%) only. The DRr provided reliable information concerning the development of synkinesis. Forty percent seemed to be a reasonable limit to distinguish the high-risk group for the development of clinical synkinesis.
Adolescent↗
Primary reading epilepsy.
This is a report of a 23-year-old-man with primary reading epilepsy. He had had three generalized tonic-clonic seizures, each time beginning with visual illusions and occurring while reading a political science text in English with complicated words. He also described tightness and stiffness in the jaw and musculature of mastication for the past 2 years, which only lasted for a few seconds and only appeared while reading political science books in English. He noted that this sensation was associated with misreading of foreign and difficult words and disappeared when he stopped reading. This case report supports the view that difficult words and misreading are provocative factors evoking seizures in reading epilepsy.
Adult↗
Childhood occipital epilepsy: seizure manifestations and electroencephalographic features.
Childhood epilepsy with occipital paroxysms (CEOP) is an idiopathic localization-related epilepsy. A typical seizure in CEOP begins with visual symptoms, followed by hemiclonic seizures, complex partial seizures or generalized tonic-clonic seizures. Benign nocturnal childhood occipital epilepsy (BNCOE), characterized by nocturnal seizures with tonic deviation of the eyes followed by vomiting, has the same electroencephalographic features as CEOP. In this study, we report the seizure symptoms and electroencephalographic features of 21 cases with CEOP or BNCOE. Out of these patients, nine had BNCOE, six had CEOP, four had CEOP and BNCOE and the remaining two belonged to the incomplete syndrome because of no paroxysmal discharges in EEG. When the patients with BNCOE awoke from sleep, they had tonic deviation of the eyes and could describe visual symptoms. Patients with CEOP had seizures beginning with visual symptoms followed by loss of consciousness but no generalized convulsions. In three cases, in addition to the occipital spikes, independent centro-temporal spikes were recorded and in another three cases generalized spike-wave discharges were recorded. Such a combination suggests the idiopathic nature of these epilepsies. We concluded that in the diagnosis of CEOP and BNCOE, the seizure symptomatology is important even if the EEG can be considered normal.
Adolescent↗
Electrophysiological investigations and prognosis in idiopathic facial palsy.
In 35 patients with idiopathic facial palsy (FP) clinical and electrophysiological examinations were performed during the first month. The patients were followed up until the 4th month to establish the prognostic value of different electrophysiological tests. Electrophysiological investigations consisted of the recording of blink reflex (BR) and the recording of the direct response (DR) elicited by the stimulation of the facial nerve. FP recovered satisfactorily in 80% of the patients. In patients in whom marked paresis remained, DR amplitude ratio of the affected side to the healthy side (DRr) was less than 25%. BR indicated a poor outcome, if it was still absent at the first month. In general, BR and DR measurements gave similar information about prognosis. Rare exceptions could be explained by the representation of different mechanisms by these two methods, demyelination and axonal degeneration.
Adolescent↗