Bilateral Temporal Lesions Research Articles

Neurosyphilis Presenting as Syndrome of Limbic Encephalitis Mimicking Herpes Simplex Virus Neuro-Infection Diagnosed Using CXCL13 Point-of-Care Assay—Case Report

The syndrome of limbic encephalitis is a severe clinical condition with heterogenous aetiopathogenesis. A common pathogen causing the infectious syndrome of limbic encephalitis is herpes simplex virus (HSV), but rare cases caused by Treponema pallidum have also been reported. We present the case of a 46-year-old man who presented with sudden onset of headaches, nausea, vomiting, and short-term loss of consciousness with clonic convulsions and subsequent disorientation and aphasia. Examination of the cerebrospinal fluid (CSF) revealed lymphocytic pleocytosis and magnetic resonance of the brain revealed bilateral temporal lesions. Clinical, radiologic, and biochemical examinations of CSF suggested encephalitis caused by HSV. However, the positivity of CXCL-13 chemokine in the CSF by a rapid point-of-care assay suggested active spirochetal infection and led to further serologic investigation. The definitive diagnosis of neuro-syphilis was concluded by positive intrathecal synthesis of immunoglobulins against Treponema pallidum. Penicillin therapy led to a rapid improvement, and the patient was discharged home after three weeks. Due to memory problems and irritability, after eighteen months, he came for a follow-up neurological and psychological examination. The psychological examination revealed a significant deficit in executive functions and behavioural changes. Neurosyphilis should be considered in the differential diagnosis of limbic encephalitis with lymphocytic pleocytosis in cerebrospinal fluid, and CXCL-13 may help to achieve diagnosis.

Anti-GABAB receptor encephalitis associated with combined small cell lung carcinoma

Autoimmune encephalitis with antibodies against the gamma-aminobutyric acid-B receptor is a relatively rare disease. We report a case with characteristic symptoms of limbic encephalitis associated with combined small cell lung carcinoma. The brain magnetic resonance imaging showed bilateral temporal lesions and the photoemission tomography revealed regional heterogenous metabolism across the brain. The double labeling of anti-gamma-aminobutyric acid-B receptor autoantibodies both in the tissues of neuroendocrine and small cell neoplasia was a unique feature of this patient.

Hrayr Terzian (1925-1988): a life between experimental neurophysiology and clinical neurology.

This article provides a biographic and scientific profile of prof. Hrayr Terzian (Addis Abeba 1925-Verona 1988). In 1966, Terzian was appointed professor of the clinic of nervous and mental diseases in Cagliari, and in 1970 moved to Verona, where until his death he led the Neurology Clinic serving as the first rector of the local university. As a close friend of the psychiatrist Franco Basaglia (1924-1980), Terzian contributed to the reform of the Italian mental health system. His interests and scientific contributions were extremely broad. He was the first to describe the electroencephalographic changes induced by the antipsychotic chlorpromazine and, together with Henri Gastaut (1915-1995) and his wife Yvette, to describe the rolandic arched ("mu") rhythm. He contributed to elucidate the inhibitory mechanisms of the cerebellum on the antigravity postural tone and provided the first description of Klüver-Bucy syndrome in humans. Always attentive to the social and political aspects of medical practice, prof. Terzian achieved outstanding results both in experimental neurophysiology and in clinical neurology. Besides offering a tribute to Hrayr Terzian, this article provides a brief historical overview of the different clinical pictures caused by bilateral temporal lesions: from the first animal observations by Sanger Brown (1852-1928) and sir Edward Albert Sharpey-Schäfer (1850-1935), later confirmed and expanded upon by Heinrich Klüver (1897-1979) and Paul C. Bucy (1904-1992), to the first cases of bitemporal lesions in humans, including that of the famous patient H.M. (Henry Gustav Molaison, 1926-2008).

A case of confusion and bilateral temporal lesions in a young woman

A 43-year-old right-handed woman with a medical history significant for methamphetamine abuse was admitted after presenting with a rapidly worsening confusion. The patient's family described 3 weeks of progressive disorientation, memory problems, anorexia with weight loss, and bilateral lower extremity neuropathic pain. Her only other pertinent medical history included a 10-pack-year history of tobacco use. On examination, her vital signs were normal except for a sinus tachycardia. She scored 15/30 on the Mini-Mental State Examination with loss of points primarily on orientation, recall, and attention. There were no abnormalities of her cranial nerves, motor examination, coordination, or gait. Sensory examination demonstrated mild hypoesthesia over her feet, otherwise normal. Initial laboratory values included a normal complete blood count, complete metabolic panel, and negative rapid plasmin reagin, HIV, and antinuclear antibodies. She had a positive toxicology screen for methamphetamine and a low-normal vitamin B12 level of 282. Her CSF analysis …

Two cases of cortical deafness & auditory agnosia with bilateral temporal lesions

症例は68歳と64歳の男性，対側側頭葉病変の既往があり，高血圧性脳出血で救急搬送された．当初，感覚性失語と思われたが，ABR波形は正常パターンで皮質聾と考えられた．皮質聾，聴覚失認は内側膝状体，聴放線，Heschl横回(Heschl’s transverse gyrus)の両側障害で起こり，患者はあたかも聾者のようにふるまったり，一見，失語と紛らわしいことがある．通常，側頭葉病変の既往に対側大脳の障害が加わったことで発症すると言われるが，臨床上，遭遇することは稀でありこのような既往を有する場合は注意すべきと考える．

007 Prolifération diffuse cornéo-conjonctivale – du vieux vin dans une bouteille neuve ?

Objective To study and present a previously unrecognized benign bilateral widespread conjunctival and corneal proliferative condition with a potential to affect vision. Methods A gradually progressive diffuse conjunctival proliferation extending on the cornea was noted in 16 patients (30 eyes). These patients were followed up for a mean of 6.5 years for the site, extent, and progression of the condition. Eight eyes had these lesions removed and studied histopathologically. Results Patients included 8 men and 8 women (mean age, 57 years). Two patients had bilateral nasal lesions only, 1 patient had bilateral temporal lesions only, 8 patients had both nasal and temporal lesions, and 5 patients had lesions that extended circumferentially. Pathological examination showed dense collagenous tissue, a moderate number of active fibroblasts, numerous blood vessels, and no elastotic degeneration in 5 of the 7 eyes. Two eyes showed mild subepithelial elastotic degeneration, and 1 lesion was difficult to interpret because of excessive intraconjunctival hemorrhage. None showed any inflammatory activity. In two patients the lesions showed early signs of recurrence following excision and was treated with intralesional 5FU injection. Conclusions The lesions appear to be a clinical variant of classic pterygium with the corneal encroachment being wider, irregular, and more extensive. Histologically, a noninflammatory nonelastotic collagenous accumulation appears to be a predominant feature unlike in classic pterygium where elastotic degeneration is the predominant feature.

Partial Kluver-Bucy syndrome as a delayed manifestation of head injury

After traumatic brain injury (TBI), the most disabling problems are generally related to neuropsychiatric sequelae, including personality change and cognitive impairment, rather than neurophysical sequelae. Kluver-Bucy syndrome (KBS) is a rare neurobehavioral condition, first described in 1937 as an experimental neurobehavioral syndrome in monkeys with bitemporal brain lesions. The syndrome in man was subsequently observed to be transient or permanent in a variety of neurodegenerative disorders and after traumatic, nontraumatic and infectious brain injury. However, partial KBS may occur in the absence of the classic bilateral temporal lesion, though rare. Pharmacological treatment of post-TBI neuropsychiatric sequelae consists of symptomatic, functional and hypothetical approaches. Specific pharmacological treatment consists of antipsychotics, anti-kindling anticonvulsants or a combination thereof. A case of partial KBS presenting as delayed manifestation of traumatic brain injury that improved with carbamazapine and antipsychotics is presented.

Diffuse Keratoconjunctival Proliferation

To report a previously unrecognized benign bilateral widespread conjunctival and corneal proliferative condition with a potential to affect vision. A gradually progressive diffuse conjunctival proliferation extending on the cornea was noted in 14 patients (28 eyes). These patients were followed up for a mean of 6 years for the site, extent, and progression of the condition. Six eyes had these lesions removed and studied histopathologically. Patients included 6 men and 8 women (mean age, 57 years). Two patients had bilateral nasal lesions only, 1 patient had bilateral temporal lesions only, 6 patients had both nasal and temporal lesions, and 5 patients had lesions that extended circumferentially. Pathology reports showed dense collagenous tissue, a moderate number of active fibroblasts, numerous blood vessels, and no elastotic degeneration in 3 of the 6 eyes. Two eyes showed mild subepithelial elastotic degeneration, and 1 lesion was difficult to interpret because of excessive intraconjunctival hemorrhage. None showed any inflammatory activity. The lesions appear to be a clinical variant of classic pterygium with the corneal encroachment being wider, irregular, and more extensive. Histologically, a noninflammatory nonelastotic collagenous accumulation appears to be a predominant feature unlike in classic pterygium where elastotic degeneration is the predominant feature.

Severe amnesia following bilateral medial temporal lobe damage occurring on two distinct occasions

A comprehensive neuropsychological assessment was performed on a 38-year-old woman with drug-resistant right temporal lobe epilepsy before temporal lobectomy, during a 2-year follow-up period, and approximately 3 years after surgery when she developed a malignant glioma in the left medial temporal lobe (MTL). Both before and after epilepsy surgery, memory function was normal. When the tumour was discovered, the patient suffered from severe retrograde and anterograde amnesia, whereas working memory and the other cognitive abilities were preserved. Compared with other cases of bilateral temporal lesion, this case is peculiar because the damage occurred on two distinct occasions. It suggests that only one MTL can allow normal memory function, or can take over the function normally subserved by a dysfunctional contralateral MTL when the dysfunction is marked and prolonged, such as in chronic epilepsy.

Secondary Mania Following Traumatic Brain Injury

Secondary Mania Following Traumatic Brain Injury

Postencephalitic pure anomic aphasia: 2-year follow-up

We report a patient with pure anomic aphasia following encephalitis. Brain magnetic resonance imaging (MRI) revealed bilateral temporal lesions, and subsequent focal atrophy in the left anterior inferior temporal lobe. Over the course of a 2-year follow-up, the patient's naming difficulty persisted without other dysfunction of language or memory. These observations indicate a contribution of the left anterior inferior temporal region to object naming.

Partial Kluver-Bucy Syndrome: Two Cases

Kluver-Bucy syndrome (KBS) is a rare neuropsychiatric disorder that may be characterized by visual agnosia, placidity, altered sexual activity, hypermetamorphosis, and hyperorality. Patients with KBS present with a complex behavioral syndrome. KBS is usually associated with lesions of the amygdala or amygdaloid pathways. However, partial KBS may occur in the absence of the classic bilateral temporal lesions. Pharmacologic treatment options have been developed from the results of case reports, which suggest that carbamazepine and antipsychotics may be helpful. We present the cases of two patients with partial KBS who responded favorably to antipsychotic medication.

In Process Citation

Pure word deafness (auditory verbal agnosia) is characterized by an impairment of auditory comprehension, repetition of verbal material and writing to dictation whereas spontaneous speech production and reading largely remain unaffected. Sometimes, this syndrome is preceded by complete deafness (cortical deafness) of varying duration. Perception of vowels and suprasegmental features of verbal utterances (e.g., intonation contours) seems to be less disrupted than the processing of consonants and, therefore, might mediate residual auditory functions. Often, lip reading and/or slowing of speaking rate allow within some limits to compensate for speech comprehension deficits. Apart from a few exceptions, the available reports of pure word deafness documented a bilateral temporal lesion. In these instances, as a rule, identification of nonverbal (environmental) sounds, perception of music, temporal resolution of sequential auditory cues and/or spatial localization of acoustic events were compromised as well. The observed variable constellation of auditory signs and symptoms in central hearing disorders following bilateral temporal disorders, most probably, reflects the multitude of functional maps at the level of the auditory cortices subserving, as documented in a variety of non-human species, the encoding of specific stimulus parameters each. Thus, verbal/nonverbal auditory agnosia may be considered a paradigm of distorted "auditory scene analysis" (Bregman 1990) affecting both primitive and schema-based perceptual processes. It cannot be excluded, however, that disconnection of the Wernicke-area from auditory input (Geschwind 1965) and/or an impairment of suggested "phonetic module" (Liberman 1996) contribute to the observed deficits as well. Conceivably, these latter mechanisms underly the rare cases of pure word deafness following a lesion restricted to the dominant hemisphere. Only few instances of a rather isolated disruption of the discrimination/identification of nonverbal sound sources, in the presence of uncompromised speech comprehension, have been reported so far (nonverbal auditory agnosia). As a rule, unilateral right-sided damage has been found to be the relevant lesion.

Early Diagnosis of Herpes Encephalopathy Using Fluid-Attenuated Inversion Recovery Pulse Sequence

This study describes the usefulness of fluid-attenuated inversion recovery (FLAIR) pulse sequence for early diagnosis and treatment of herpes encephalitis. An 8-year-old boy with suspected herpes simplex encephalitis was examined by magnetic resonance imaging at 3 days post-symptom onset. Lesions in the right thalamus and insular cortex were detected by conventional T 2-weighted images and images from FLAIR pulse sequence. Bilateral temporal lesions, however, were only detected by FLAIR images.

Glossolalic jargon after a right hemispheric stroke in a patient with Wernicke's aphasia

Abstract We report the case of a 65-year-old monolingual right-handed man with 5 years of schooling who, after partial recovery of Wernicke's aphasia due to a left temporoparietal lesion, was affected by a second stroke involving the homologous regions in the right hemisphere. The clinical picture after the second stroke was characterized by a severe ‘glossolalic’ jargon: auditory comprehension was only moderately impaired. This case supports the association of severe, disinhibited jargon production with bihemispheric lesions. Furthermore, it indicates that partially preserved auditory comprehension can be found in a patient with bilateral temporal lesions involving Wernicke's area and its mirror region in the right hemisphere.

A Case of Pure Word Deafness and Auditory Agnosia Associated with Bilateral Temporal Lesions

A Case of Pure Word Deafness and Auditory Agnosia Associated with Bilateral Temporal Lesions

Temporal lobe lesions and perception of species-specific vocalizations by macaques.

Japanese macaques were trained to discriminate two forms of their coo vocalization before and after unilateral and bilateral ablation of the temporal cortex. Unilateral ablation of the left superior temporal gyrus, including auditory cortex, resulted in an initial impairment in the discrimination, but similar unilateral ablation of the right superior temporal gyrus had no effect. Bilateral temporal lesions including auditory cortex completely abolished the ability of the animals to discriminate their coos. Neither unilateral nor bilateral ablation of cortex dorsal to and sparing the auditory cortex had any effect on the discrimination. The perception of species-specific vocalizations by Japanese macaques seems to be mediated by the temporal cortex, with the left hemisphere playing a predominant role.

Role of inferior temporal cortex in interhemispheric transfer

Neurons in inferior temporal cortex of the rhesus monkey usually have large receptive fields that extend well across the midline into both visual half-fields. The responsiveness of these neurons to stimuli in the ipsilateral visual half-field depends on the splenium and anterior commissure, the same pathways necessary for interhemispheric transfer of visual habits. Since inferior temporal neurons have the same trigger features in both half-fields and are usually binocular, they may be the site of the interhemispheric neural convergence that underlies interhemispheric transfer. If so, bilateral removal of inferior temporal cortex should interfere with interhemispheric transfer even when the commissures are intact. To test this, monkeys were trained on pattern discriminations with one eye and then tested for transfer with the other eye. Five experimental monkeys received bilateral inferior temporal lesions and, to restrict input from each eye to one hemisphere, section of the optic chiasm. Ten controls received either bilateral temporal lesions alone, chiasm section alone or remained unoperated. Only the experimental animals showed impaired transfer. These results suggest that inferior temporal neurons mediate interhemispheric transfer by providing perceptual equivalence for patterns in the left and right visual fields, and, by implication, perhaps also for patterns in different parts of the same field.

A Case of Cortical Deafness-Deafness due to Bilateral Temporal Lesion

A 50-year-old, right-handed, male showed cortical deafness and Wernicke's aphasia after 14 hours' duration of atrial paroxysmal tachicardia.Neuroradiological study by 90Tm brain scan revealed hot lesions in the temporal area bilaterally and CT scan showed that a low-density area occupied in the both temporal gyri and insulas. The basal ganglia and thalamus (including medial geniculate bodies) were spared bilaterally.1) Pure tone audiometry revealed a bilateral mild hearing loss but the thresholds were unstable.2) Bekesy audiometry showed a temporary threshold shift bilaterally.3) Speech audiometry revealed lower discrimination rate than 25% bilaterally.4) Discrimination rate of musical instruments was remarkably low. Perception of rhythm and melody was markedly impaired.5) Sound localization for warble tone was well preserved.6) Auditory Brain Stem Response by click sounds was normal.7) Wave forms of the middle latency response were almost normal.However, thresholds of the slow vertex response were abnormally high in both ears. Thus, auditory pathway in the peripheral apparatus and brain stem appeared to be almost intact, but functionally, cortical or subcortical lesions were suspected bilaterally, Sensory aphasia was coincident. Other six cases of cortical deafness or auditory agnosia in the literature were reviewed, and the clinical findings and auditory disorders of these seven cases were remarkably similar to those of the case reported herein. In conclusion, we consinered that “auditory agnosia due to bitemporal lesions” for these cases was the better term “cortical deafness.”