A case of hereditary sensory and autonomic neuropathy type II with plantar ulceration.
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Charcot-Marie-Tooth Disease↗
Biomedical subjects
H Forta
Publications and source records attributed to H Forta.
Paired transcranial magnetic stimulation in hemifacial spasm.
OBJECTIVES: The aim of this study was to investigate the changes in cortical excitability in patients with hemifacial spasm (HFS) in order to examine the physiological state of facial nerve nucleus. METHODS: Nineteen patients with HFS and 13 control subjects were examined at rest. The procedure was repeated during voluntary contraction in 6 of control subjects. By paired transcranial magnetic stimulation, conditioning and test motor evoked potentials (MEP) were recorded from bilateral orbicularis oris muscles at interstimulus intervals (ISI) of 20, 25, 30, 50, 75 and 100 ms. RESULTS: In control subjects at rest, ISI's of 20, 25, 30 ms evealed facilitation of the test MEP and inhibition developed at ISI's of 75 and 100 ms. Test MEP facilitation in the contracting controls and on the symptomatic sides of HFS patients was significantly less than the resting controls. Asymptomatic sides of HFS patients behaved similar to the corresponding symptomatic sides and to the contracting controls, although the values were not statistically significant when compared to the resting controls. CONCLUSION: The lessening of facilitation found in this study can be interpreted as an abnormal finding that reflects the changes in the excitability of facial motoneuronal system in patients with HFS. However it is difficult to exclude the possibility that this finding can occur due to the normal behavior of cortex during voluntary or involuntary contraction.
Adult↗
Intrathecal methotrexate-induced acute cerebellar syndrome.
Intrathecal application of chemotherapeutics has been the mainstay of central nervous system prophylaxis in acute lymphoblastic leukemia treatment. We describe a patient who developed acute cerebellar syndrome after prophylactic intrathecal methotrexate administration and recovered spontaneously. To our knowledge, this is the first case of methotrexate-related acute cerebellar syndrome.
Acute Disease↗
Tuberculous meningitis in adults: review of 61 cases.
BACKGROUND: Tuberculosis is still a major cause of serious illness in developing countries. The objective of this study was to assess the clinical, laboratory, radiological and prognostic features of tuberculous meningitis (TBM) in immuncompetent adult patients. PATIENTS AND METHODS: Sixty-one patients with confirmed or presumed TBM seen over a 12-year period at the Neurology Department of Bakirköy Research and Training Hospital for Psychiatric and Neurological Disease, Istanbul, Turkey, were analyzed retrospectively. Patients were grouped according to the severity of meningitis on admission (stages I, II and III). They were also divided into two groups according to the presence of paradoxical response (progressive increase of lymphocytes or increase of polymorphonuclear cells instead of lymphocytes) in CSF samples. A combination of five antituberculosis drugs was used in the 1st month of treatment. Patients received antituberculosis therapy for at least 12 months. The outcome of the patients was defined on the basis of the 12-month Barthel index (BI) score (BI < 12 poor; BI >/= 12 good). For statistical analysis death was included in the poor outcome group. RESULTS: Paradoxical response in CSF findings was seen in 20 patients. There was no statistically significant difference between the outcome of the patients with and without CSF paradoxical response; however, new tuberculomas developed more frequently in the first group (p < 0.05). The overall mortality was 27.8%. Stage of disease was found to be independently associated with the 12-month outcome (OR 7.2; 95% CI 1.7-30.3, p = 0.007). CONCLUSION: In developing countries such as Turkey, tuberculosis is still an important public health issue. Early suspicion and appropriate long-term antituberculosis therapy together with corticosteroids may reduce mortality and morbidity in TBM patients.
Adolescent↗
MRI reveals reversible lesions resembling posterior reversible encephalopathy in porphyria.
We report a 20-year-old woman who had an attack of acute intermittent porphyria with seizures, hallucinations, autonomic and somatic neuropathy. T2-weighted MRI revealed multiple lesions which were no longer visible 3 months later. We suggest a similar mechanism to posterior reversible encephalopathy underlying cerebral symptoms in porphyria.
Adult↗
Magnetic resonance imaging in amyotrophic lateral sclerosis.
OBJECTIVE: We aimed to investigate the importance of magnetic resonance imaging for the diagnostic process of the upper motor neuron involvement. MATERIAL AND METHODS: Fifteen patients (Group 1) who were diagnosed with amyotrophic lateral sclerosis in the neurology department of the Sisli Etfal Hospital and 20 controls (Group 2) entered the study. Magnetic resonance imaging examinations were performed in the radiology clinic of the same hospital. T2 and proton density weighted axial images were obtained. These images were evaluated by two blind radiologists. The radiologists looked for two signs: the presence of a low signal intensity at the precentral cortex and the presence of symmetrical rounded foci of high signal intensity within the internal capsule on both T2 and proton density weighted images. RESULTS: For the first sign there was no statistically significant difference between the two groups but for the latter one there was a statistically significant difference. CONCLUSION: We think that the presence of the latter sign may make it possible for the radiologists to help the clinicians further in this difficult and confusing diagnosis.
Amyotrophic Lateral Sclerosis↗
Charcot-Marie-Tooth disease associated with Type 2 diabetes mellitus.
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Aged↗
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↗
Clinical, electrophysiological and magnetic resonance imaging findings in carpal tunnel syndrome.
OBJECTIVE: To assess magnetic resonance imaging (MRI) findings in carpal tunnel syndrome (CTS) and to compare them with electrophysiological findings. METHODS: Routine motor and sensory nerve conduction examinations and needle EMG were performed in 42 hands of 22 patients, who were clinically diagnosed as having CTS in at least one wrist. RESULTS: Of 29 wrists with clinically and electrophysiologically confirmed CTS, MRI could detect abnormality in 18 wrists (62%). Median nerve was found to be abnormal in MRI in 1 of 2 wrists with suspected clinical symptoms and proven CTS by electrophysiological examination. MRI was abnormal in 1 of 4 wrists with normal clinical and electrophysiological examination. MRI was abnormal in 46, 7% of wrists with mild CTS, in 61.6% of moderate CTS and in 100% of severe CTS. Volar bulging of the flexor retinaculum was detected in a single wrist with severe CTS. Enlargement of median nerve was observed in 3 of 5 severe CTS. CONCLUSION: MRI could be useful in the diagnosis of unproven cases in CTS. It also provides anatomical information that correlate well with electrophysiological findings in regard of the severity of median nerve compression.
Carpal Tunnel Syndrome↗