Vasospasms of the intracranial arteries are a well-known complication of subarachnoid hemorrhage and are also frequently encountered in other disorders such as migraine, cerebral vasculitis or reversible cerebral vasoconstriction syndrome. In contrast, recurrent spontaneous vasospasms of the extracranial circulation appear to be extremely rare and have most often been associated with migraine. We present a patient with recurrent strokes due to spontaneous transient vasospastic occlusions of the internal carotid artery (ICA) without migraine over a time period of at least 13 years. Initially, the patient had presented with a bilateral ICA occlusion and a cerebral infarct on the right side. While the right ICA remained occluded, a reopening of the left ICA could be detected 3 days after this initial event. In subsequent years, both duplex sonography and magnetic resonance angiography revealed recurrent occlusions of the left ICA, which resolved spontaneously within days. This case and other rare previous reports indicate that recurrent non-migrainous vasospasms of the extracranial carotid artery likely reflect a distinct entity which can cause ischemic strokes.
Vasospasm; Internal carotid artery; Cerebral ischemia; Duplex sonography; Magnetic resonance angiography
Benign tremulous parkinsonism (BTP) is a tremor dominant syndrome characterized by mild, levodopa-resistant parkinsonism with limited disability or progression.
A 56-year-old woman presented with a 2-year history of tremor. Neurological examination revealed right-hand rest tremor and slow finger tapping with decreased amplitude; however, we did not observe posture tremor, rigidity, bradykinesia, or posture disability. She was diagnosed with Parkinson's disease (PD) and received levodopa/carbidopa, effectively treating her rest tremor. At the age of 61 years, reoccurrence of the rest tremor was successfully treated again with levodopa/carbidopa and selegiline. Approximately 11 years have passed since symptom onset and the patient shows no further disease progression.
This case broadens the characterization of BTP to include levodopa-responsive PD.
Benign tremulous parkinsonism; Essential tremor; Levodopa; Parkinson's disease; Rest tremor
Isolated accessory nerve palsy due to intracranial disorders is uncommon because intracranial accessory nerve injury usually occurs in case of a skull base tumor or trauma, resulting in one of multiple cranial nerve palsies. We report a very rare case of isolated accessory nerve palsy due to a large thrombosed aneurysm of the intracranial vertebral artery. Full recovery was achieved after surgery.
A patient complaining of transient numbness in the right side was referred to our hospital. An MRI indicated a large thrombosed aneurysm of the right vertebral artery. The aneurysm severely compressed the medulla oblongata. First, the proximal vertebral artery (VA) was clipped with an aneurysm clip to reduce the pressure inside the aneurysm. However, cerebral angiography revealed a partial recanalization of the right VA. The patient then underwent coil embolization of the right VA just proximal to the aneurysm clip. Subsequently, the right VA was completely obliterated. The patient was discharged without any neurological deficit. Two weeks later, however, she complained of right shoulder pain. Physical and neurological examinations demonstrated atrophy of the right trapezius and sternocleidomastoid muscle, leading to a deepening of the right supraclavicular fossa. The symptoms were considered to result from the right isolated accessory nerve palsy. Follow-up MRI showed that the VA aneurysm gradually decreased in size over a period of several months. At the same time, her symptoms disappeared completely.
We should keep in mind that isolated accessory nerve palsy can be caused by a large or giant vertebral aneurysm.
Accessory nerve palsy; Intracranial aneurysm; Thrombosed aneurysm; Vertebral artery
Interferon β-1a is a widely used immunomodulation treatment for multiple sclerosis. Liver function impairment is a common side effect and usually develops in the first 6 months after interferon use. Here, we describe 2 multiple sclerosis patients who developed delayed liver function impairment 5 years after receiving interferon β-1a treatment. Their liver function recovered after discontinuing interferon use, and further detailed hepatological evaluations excluded other etiologies of liver function impairment. Our case reports illustrate that liver function impairment induced by interferon β-1a can be delayed for 5 years after starting treatment and, probably, this is an idiosyncratic reaction. Regular liver function monitoring in multiple sclerosis patients who receive interferon β is necessary even after the first 6 months of treatment, especially in those patients with concomitant use of other liver-toxic medications.
Multiple sclerosis; Hepatitis; Liver function impairment; Interferon β; Azathioprine
A 46-year-old male with juvenile myoclonic epilepsy was admitted to the neurological department for convulsive seizures just after lamotrigine was discontinued. On admission he was awake but had a right-sided hemiparesis with Babinski sign and ataxic finger-nose test on the left side. An MR scan showed a left-sided pontine infarction, an infarct in the left cerebellar hemisphere and a right vertebral artery dissection (VAD). The patient was treated with heparin and an oral anticoagulant for 6 months. Recovery of neurologic function was excellent. In patients with symptoms of disturbances of posterior circulation after epileptic seizures, VAD should be considered.
Seizure; Vertebral artery; Dissection; Stroke
The porphyrias are a defect in the biosynthesis of heme which can be associated with different neurological symptoms during acute attacks such as peripheral neuropathy, mental disturbance and seizures. So far, there have only been a few case reports of status epilepticus, none of which were of epilepsia partialis continua (EPC). We present here two cases of hereditary coproporphyria (HCP) manifesting EPC as part of the clinical presentation.
The patients’ medical charts, EEG and imaging studies were carefully reviewed.
Case 1 is a 49-year-old male who first presented a tonic-clonic seizure. Case 2 is a 30-year-old male who came to the emergency room for a convulsive status epilepticus. Both evolved to EPC over the next days. EPC persisted despite several antiepileptic drug trials. Diagnosis of HCP was confirmed by a high level of urine, fecal and serum porphyrins in both cases and by genetic testing in one. Over the last 3 years, the first patient has continued to present non-disabling EPC and has had four tonic-clonic seizures associated with alcohol consumption. The second patient died from brain edema one month and half after admission.
Acute porphyrias should be included in the differential diagnosis of new onset status epilepticus, including EPC. Their recognition is important as it modifies significantly patient management, since many anticonvulsants are porphyrogenic.
Acute porphyria; Status epilepticus; Epilepsia partialis continua
Chemotherapy-related myelopathy mimicking subacute combined degeneration (SCD) has rarely been reported. We encountered a 35-year-old female with sensory ataxia after intrathecal chemotherapy. Spinal magnetic resonance imaging showed localized abnormal signal areas in the lateral and dorsal white matter, mimicking SCD. Diffusion imaging showed restricted water diffusion and increased microstructural complexity, and cerebrospinal fluid analysis showed increased levels of myelin basic proteins, indicating demyelinating myelopathy. Advanced diffusion imaging can provide more information on the microstructure of chemotherapy-related myelopathy.
Magnetic resonance imaging; Diffusion imaging; Spinal cord; Cytosine arabinoside; Methotrexate; Subacute combined degeneration
To report an unusual presenting manifestation of multiple sclerosis in a teenager.
We report the case of a 16-year-old male who was diagnosed with schizoaffective disorder a year prior to his neurological consultation. Neurological examination showed evidence of mild lower extremity spasticity with ataxia and dysarthria. Imaging revealed lesions suggestive of multiple sclerosis.
An organic basis for psychiatric disorders is often overlooked. Children presenting with symptoms suggestive of a mood disorder, decline in cognition or psychosis deserve a detailed neurological examination and appropriate imaging if indicated, before starting them on antidepressant or neuroleptic medication.
Multiple sclerosis; Adolescence; Mood disorder
We describe a patient with persistent cerebral achromatopsia occurring after bilateral occipital strokes. Blinded color recognition was assessed with a computerized experimental paradigm and the patient reported the degree of confidence in the response exactness on a visual percent scale. Color recognition was accurate and above chance (Fisher's exact test, p < 0.002). The degree of confidence in the answers showed a significant correlation with recognition scores (Spearman rank order correlation, p < 0.0001). These findings constitute the exceptional condition of what we called color anopsognosia (not knowing of seeing colors) and recall the theoretic figure of the ‘philosophical zombie’. However, the cognitive mechanisms of the dissociation between a subjective colorless vision and good performance for color naming still remain poorly understood.
Achromatopsia; Color agnosia; Blindsight; Cerebral achromatopsia; Philosophical zombie; Color vision
Cerebral cavernomas are thin-walled vascular lesions composed of dilated capillary spaces. De novo formation of cavernomas after cerebral radiotherapy has been suspected since 1994. They are mostly seen in children after irradiation of brain tumours. Radiation dose and the developing juvenile brain are predisposing factors causing cavernomas. However, the underlying mechanisms are still far from being understood. In adults, radiation-induced cavernomas (RICs) usually occur 10 years after a high cumulative radiation dosage of >30 Gy. Here, we report a 45-year-old man with new-onset focal epileptic seizures caused by a haemorrhagic lesion noted on cerebral computed tomography scan. Brain MRI showed the typical appearance of a ruptured cavernoma. Of note, a cerebral MRI scan 5 years earlier showed no corresponding lesion. The patient had been treated with haematopoietic stem cell transplantation for acute myeloid leukaemia (AML) 16 years before. As part of this procedure, total body irradiation (TBI) consisting of 12 Gy was administered. According to the data from the literature, the typical delay from irradiation and a former normal brain MRI scan, we assume that our patient suffers from a RIC. To our knowledge, this is the first documented adult AML patient with a RIC treated with TBI. We aim to increase awareness among neurologists for the association of cranial irradiation or TBI and de novo cavernomas in patients suffering from malignant diseases.
Radiation-induced cavernoma; De novo cavernoma; Total body irradiation
Myelitis is one of the rarest neurological complications of the varicella zoster virus (VZV) infection. Focal muscle weakness with or without sensory disturbance occurs in approximately 5% of the cases after acute VZV infection, with complete recovery in 50–70%.
This report describes two rare cases of elderly patients with VZV myelitis secondary to dermatomal zoster rash. Patient 1 was a 79-year-old woman who developed paraplegia, numbness and decreased sensation in the left arm and below thoracic (Th)-10 after sacral zoster. Spinal cord MRI showed a high-signal-intensity lesion at the cervical spinal nerve 2 on a T2-weighted image. Patient 2 was a 73-year-old man who developed right flaccid leg weakness and urinary retention after right dorsal Th 5–8 zoster. Spinal cord MRI showed a high-signal-intensity lesion at Th 3–4 on a T2-weighted image. In both cases, although the conventional single polymerase chain reaction (PCR) assays all showed negative results, the original nested PCR assay detected VZV DNA in the cerebrospinal fluid (CSF) specimen collected on admission. In addition, the anti-VZV IgG antibody by enzyme immunoassay and antibody index were elevated in the CSF specimens during the clinical courses of both patients. On the basis of these findings, both patients were diagnosed with VZV myelitis and were treated with high-dose acyclovir and corticosteroid. This combined treatment was appropriate and effective for the improvement of their functional outcomes.
The detection of VZV DNA in CSF by nested PCR assay and the evaluation of the antibody index to VZV had significant diagnostic value.
Varicella zoster virus; Myelitis; Enzyme immunoassay; Antibody index; Nested polymerase chain reaction assay
A 71-year-old right-handed man was admitted to our hospital with right hemiparesis and sensory impairment associated with mild aphasia. Although aphasia gradually resolved within 2 weeks after stroke onset, his writing ability remained disturbed. A computed tomography (CT) scan at stroke onset revealed a hematoma in the left thalamus, but no cortical lesions were observed. Further, a single-photon emission CT (SPECT) scan showed decreased blood flow in the left thalamus, in the cortical region extending from the left superior temporal gyrus to the parietal lobe, and in the frontal lobe. It is possible that agraphia may have directly resulted from the thalamic lesion, but SPECT findings strongly suggested that a general decrease in left cortical function concomitant with a disruption of the thalamocortical and cortico-thalamocortical projection fibers produced these cognitive deficits.
Cerebral hemorrhage; Agraphia; Aphasia; Stroke
We report the detailed documented case of a 57-year-old homosexual HIV-positive man with bilateral cochleovestibular deficits as a first symptom of syphilis infection in early stage II disease. As a morphological substrate, a strong enhancement of both inner ears and vestibulocochlear nerves were found on gadolinium-enhanced MR scans. The serological tests identified an active infection with Treponema pallidum. After a high-dose treatment with penicillin G and prednisolone, the auditory and vestibular functions and the MR morphology of the vestibulocochlear nerves and inner ears on both sides returned to normal.
Cochleovestibular deficit; Neurosyphilis; HIV
Diabetic hemichorea-hemiballism with non-ketotic hyperglycemia is usually a benign syndrome. Here, we report a 78-year-old woman with persistent hemichorea (HC) for longer than 1 year with a recurrence after rapid correction of hyperglycemia. Following the disappearance of the characteristic T1 hyperintensity at 3 months after onset, an MRI demonstrated T2* hypointensity and atrophic changes in the contralateral striatum, suggesting irreversible neuronal loss and some vascular proliferation. The electrophysiological examination using transcranial magnetic stimulation revealed significantly shorter cortical silent periods (CSPs) on the contralateral primary motor cortex (M1), possibly in relation to cortical hyperexcitability. We have applied 10 daily sessions of low-frequency repetitive transcranial magnetic stimulation (rTMS) over the contralateral M1 to reduce the hyperexcitability. The HC was suppressed during and for several minutes after rTMS with prolongation of CSPs. After rehabilitation therapy, the patient was able to walk independently with a walker. We suggest that the combination of low-frequency rTMS and rehabilitation therapy may be a possible choice in medically refractory involuntary movements.
Hemichorea; Hyperglycemia; Repetitive transcranial magnetic stimulation; Basal ganglia; MRI; Rehabilitation
We present the case of an aphasic 77-year-old stroke patient with left distal M1 occlusion who received rt-PA for thrombolysis while on oral anticoagulant treatment with dabigatran (150 mg b.i.d.). Coagulation parameters were normal (thrombin time 20 s, aPTT 20 s, INR 1.08) and the patient improved from an NIHSS of 15 to 5 within 24 h with sonographic evidence of M1 recanalization. She did not develop intracranial bleeding complications but showed unusually large diffuse skin ecchymoses. In our report, we give an overview of all reported cases of thrombolysis under dabigatran anticoagulation and discuss the questions of medication adherence under novel oral anticoagulants (NOA) and the safety of NOA in terms of secondary intracerebral hemorrhage after stroke.
Anticoagulation; Atrial fibrillation; Stroke; Thrombolysis; Fibrinolytic; Hemorrhage
Few reports describe the reactivation of latent herpes simplex virus causing encephalitis (HSVE) in patients undergoing brain radiation therapy and a concomitant steroid regimen. The role for steroid use in the treatment of patients with HSVE has not been fully elucidated. We report the case of a female patient immunosuppressed by steroids and brain radiation who developed HSVE and responded to acyclovir and dexamethasone.
Herpes simplex virus encephalitis; Immunosuppression; Corticosteroids
Crowned dens syndrome (CDS), related to microcrystalline deposition in the periodontoid process, is the main cause of acute or chronic cervical pain. Microcrystal-line deposition most often consists of calcium pyrophosphate dehydrate crystals and/or hydroxyapatite crystals.
This report describes the case of an 89-year-old woman who presented with sudden onset, high fever, severe occipital headache, and neck stiffness. A laboratory examination revealed a markedly elevated white blood cell count (11,100/µl) and C-reactive protein level (23.8 mg/dl). These clinical findings suggested severe infection such as meningitis with sepsis. However, the results of blood culture, serum endotoxin, and procalcitonin were all negative, and cerebrospinal fluid studies revealed only a slight abnormality. The patient was first diagnosed with meningitis and treated with antiviral and antibiotic agents as well as non-steroidal anti-inflammatory drugs, but they only had limited effects. A cervical plain computed tomography (CT) scan and its three-dimensional (3D) reconstruction detected a remarkable crown-like calcification surrounding the odontoid process. On the basis of the CT findings, the patient was diagnosed as a severe case of CDS and was immediately treated with corticosteroids. The patient's condition drastically improved within a week after one course of corticosteroid therapy.
Some atypical symptoms of CDS are misleading and may be misdiagnosed as meningitis, as happened in our case. A CT scan, especially a 3D-CT scan, is necessary and useful for a definitive diagnosis of CDS. CDS should be considered as a differential diagnosis of a possible etiology for fever, headache, and cervical pain of unknown origin.
Crowned dens syndrome; Calcium pyrophosphate dehydrate crystals; Odontoid process; Meningitis; Cervical computed tomography scan; Corticosteroids
Cognitive and behavioral impairments are considered to occur frequently in amyotrophic lateral sclerosis/motor neuron disease (MND). Rarely, apraxia has been reported in MND. Orofacial, or buccofacial, apraxia is characterized by a loss of voluntary control of facial, lingual, pharyngeal and masticatory muscles in the presence of preserved reflexive and automatic functions of the same muscles.
We report a patient with MND who presented with spastic dysarthria and asymmetric orofacial apraxia. She progressed to frontotemporal dementia (FTD).
Clinical and neurophysiological examinations were suggestive of bulbar-onset MND-FTD. Tractography showed a reduction of fractional anisotropy in the centrum semiovale, corona radiata, corticomedullary pathway and inferior aspect of the medulla; the changes were more severe on the left side. To our knowledge, this is the first report of an asymmetric presentation of an apraxic syndrome in MND-FTD.
Amyotrophic lateral sclerosis; Motor neuron disease; Orofacial apraxia; Primary lateral sclerosis; Tractography
Cerebral microbleeds have acquired increased attention as a silent marker of small vessel disease that carries an increased risk of hemorrhage. The etiology is believed to be either hypertension or amyloid deposition. Here, we present a case with a patient whose transient focal symptom most likely was due to the occurrence of an acute microbleed, indicating that not all microbleeds are silent and that the cause of a transient ischemic attack is not always ischemic.
Stroke; Transient ischemic attack mimic; Microbleed; Hypertension
Patients with anti-myelin-associated glycoprotein (MAG)/sulfated glucuronyl paragloboside (SGPG) neuropathy associated with Waldenström macroglobulinemia show demyelinating neuropathy, but the temporal dispersion of distal compound muscle action potential (CMAP) in motor nerve conduction studies (NCS), which represents heterogeneous demyelination at the motor nerve terminal, is rare. We report on a 70-year-old man with anti-MAG/SGPG neuropathy associated with Waldenström macroglobulinemia; he had a 2-year history of mild dysesthesia of the foot sole without any motor symptoms. He showed marked temporal dispersion of distal CMAP in the tibial nerve with other demyelinating findings in the NCS. The temporal dispersion of distal CMAP in the tibial nerve improved significantly, and motor function was again normal 1 year after rituximab monotherapy. The temporal dispersion of distal CMAP in anti-MAG/SGPG neuropathy is rare, but it could occur from an early stage when the patients show mild or no motor symptoms. Rituximab therapy before secondary axonal degeneration has great potential to reverse the effects of the demyelination including the temporal dispersion of distal CMAP, and to prevent the deterioration of neuropathy in anti-MAG/SGPG neuropathy.
Anti-MAG/SGPG neuropathy; Waldenström macroglobulinemia; Subclinical temporal dispersion; Distal compound muscle action potential; Rituximab
Huntington's disease (HD) is a dominantly inherited neurodegenerative disease best known for chorea. The disorder includes numerous other clinical features including mood disorder, eye movement abnormalities, cognitive disturbance, pendular knee reflexes, motor impersistence, and postural instability. We describe a mild case of HD early in the disease course with depression and subtle neurological manifestations. In addition, we review MRI and diffusion tensor imaging features in this patient. The bicaudate ratio, a measure of caudate atrophy, was increased. Fractional anisotropy values of the bilateral caudate and putamen were increased, signifying neurodegeneration of these structures in HD.
Huntington's disease; Diffusion tensor imaging; Fractional anisotropy
Dissections of the cervical arteries cause about 20% of total juvenile strokes. Approximately 4% of the carotid artery dissections are due to a (poly)trauma such as car accidents. Despite improved diagnostic facilities, traumatic dissections are often underdiagnosed or diagnosed too late due to a lack of awareness of potential initial signs and symptoms.
We report here a case of a delayed embolic stroke after a car accident caused by a dissection of the carotid artery and subsequent pseudoaneurysm.
To reduce the long-term morbidity or mortality of multiple trauma patients, an early detection of cervical carotid and vertebral dissections is strictly necessary.
Dissection; Juvenile stroke; Polytrauma; Pseudoaneurysm
Exploding head syndrome (EHS) is a rare parasomnia in which affected individuals awaken from sleep with the sensation of a loud bang. The etiology is unknown, but other conditions including primary and secondary headache disorders and nocturnal seizures need to be excluded.
A 57-year-old Indian male presented with four separate episodes of awakening from sleep at night after hearing a flashing sound on the right side of his head over the last 2 years. These events were described ‘as if there are explosions in my head’. A neurologic examination, imaging studies, and a polysomnogram ensued, and the results led to the diagnosis of EHS.
EHS is a benign, uncommon, predominately nocturnal disorder that is self-limited. No treatment is generally required. Reassurance to the patient is often all that is needed.
Exploding head syndrome; Parasomnia; Headache disorder
Catatonia, originally described by Karl Kahlbaum in 1874, may be regarded as a set of clinical features found in a subtype of schizophrenia, but the syndrome may also stem from organic causes including vascular parkinsonism, brain masses, globus pallidus lesions, metabolic derangements, and pharmacologic agents, especially first generation antipsychotics. Catatonia may include paratonia, waxy flexibility (cerea flexibilitas), stupor, mutism, echolalia, and catalepsy (abnormal posturing). A case of catatonia as a result of acute renal failure in a patient with dementia with Lewy bodies is described. This patient recovered after intravenous fluid administration and reinstitution of the atypical dopamine receptor blocking agent quetiapine, but benzodiazepines and amantadine are additional possible treatments. Recognition of organic causes of catatonia leads to timely treatment and resolution of the syndrome.
Dementia with Lewy bodies; Catatonia; Catalepsy
This case report exposes a phenomenon which, although proposed, has not been described in clinical literature: transient postictal hemiplegia (Todd's paralysis) with concomitant electrocardiographic J-point deflection (Osborn waves). Although typically associated with hypothermia, a prominent J-wave on the electrocardiogram (ECG) results from a transmyocardial voltage gradient during ventricular repolarization. Rarely, the Osborn wave may be observed in a non-hypothermic setting such as hypercalcemia or cerebral hemorrhage. Transient postictal hemiplegia has been attributed to localized cerebral hypoperfusion resulting from motor cortex exhaustion following an epileptic seizure. The same central nervous system autonomic dysfunction has been theorized to produce subendocardial hypoperfusion with electrocardiographic change and cardiac troponin T elevation. This is the first described ECG evidence of a dynamically displaced J-point in the setting of postictal hemiplegia.
Todd's paralysis; Osborn wave; J-wave; Epilepsy; Stroke; STEMI; Hemiplegia; Postictal; ACS