Sick sinus syndrome and orthostatic hypotension in Parkinson’s disease

We present a case of Parkinson's disease patient whose initial symptoms were sick sinus syndrome and orthostatic hypotension. Our case illustrates difficulties in distinguishing syncope of primary cardiac or neurological origin and highlights the importance of a diagnostic workup including neurological examination.     

Adult-onset opsoclonus-myoclonus syndrome due to West Nile Virus treated with intravenous immunoglobulin

A 63-year-old female with no significant past medical history was presented with a 5-day history of progressive opsoclonus-myoclonus, headaches, and fevers. Her workup was significant only for positive West-Nile Virus serum serologies. She received a 2-day course of intravenous immunoglobulin (IvIG). At an 8-week follow up, she had a complete neurological remission. Adult-onset opsoclonus-myoclonus syndrome is a rare condition for which paraneoplastic and infectious causes have been attributed. To our knowledge, this is the first case reported of opsoclonus-myoclonus secondary to West-Nile Virus treated with intravenous immunoglobulin monotherapy.     

Acute glomerulonephritis presenting with PRES: a report of 4 cases

OBJECTIVE: Posterior reversible encephalopathy syndrome (PRES) occurs most commonly in the setting of known hypertension or use of immunosuppressive agents. DESIGN AND METHODS: We report four previously-well children who presented acutely with altered mentation, seizures and visual disturbances and were diagnosed with PRES. RESULTS: Only one child had a history of gross hematuria prior to the seizure. All four were discovered to be hypertensive only after onset of their neurological symptoms, and were subsequently diagnosed with glomerulonephritis. All four had rapid resolution of neurological symptoms with adequate treatment of hypertension. CONCLUSIONS: Blood pressure must be measured promptly in all children presenting with these symptoms. If elevated, the diagnosis of PRES should be strongly considered and a workup for renal disease pursued.     

Rebleeding from a primary brain tumor manifesting as intracerebral hemorrhage (CNN 04/077, revised version)

Symptomatic spontaneous intratumoral hemorrhage is a rare event in a patient with a brain tumor (BT). Although the treatment of choice in such a case is surgical removal of both the tumor and the hemorrhage, the optimal timing for surgical intervention has not been clearly established, particularly in those who present with minimal neurological deficits and a small hemorrhage volume. Two cases of primary BTs manifesting as an intracerebral hemorrhage (ICH) are described, in which rebleeding from the tumor occurred shortly after the initial hemorrhage. The patients each presented with the sudden onset of a headache and minimal neurological deficits, and the neuroradiological workup was consistent with a diagnosis of hemorrhagic BT. Each patient remained neurologically stable, and elective surgery had been planned within 7 days of their admission, but rebleeding occurred 5 and 6 days, respectively, after admission. A BT manifesting as an ICH may rebleed shortly after the initial bleeding, and should be treated on an emergency basis instead of an elective basis regardless of the patient's neurological status on admission or hematoma volume on the initial CT scans.     

Rebleeding from a primary brain tumor manifesting as intracerebral hemorrhage (CNN 04/077, revised version)

Symptomatic spontaneous intratumoral hemorrhage is a rare event in a patient with a brain tumor (BT). Although the treatment of choice in such a case is surgical removal of both the tumor and the hemorrhage, the optimal timing for surgical intervention has not been clearly established, particularly in those who present with minimal neurological deficits and a small hemorrhage volume. Two cases of primary BTs manifesting as an intracerebral hemorrhage (ICH) are described, in which rebleeding from the tumor occurred shortly after the initial hemorrhage. The patients each presented with the sudden onset of a headache and minimal neurological deficits, and the neuroradiological workup was consistent with a diagnosis of hemorrhagic BT. Each patient remained neurologically stable, and elective surgery had been planned within 7 days of their admission, but rebleeding occurred 5 and 6 days, respectively, after admission. A BT manifesting as an ICH may rebleed shortly after the initial bleeding, and should be treated on an emergency basis instead of an elective basis regardless of the patient's neurological status on admission or hematoma volume on the initial CT scans.     

Hypertrophic pachymeningitis in a patient with Takayasu arteritis: One more association?

Hypertrophic pachymeningitis (HP) is a rare chronic inflammatory disease of the dura mater, described in association with various infections, systemic vasculitides such as Wegener''s granulomatosis and giant cell arteritis. However, HP in association with Takayasu arteritis (TA) has not been described. We report a young woman who presented with headache, seizures, and right third and fourth cranial neuropathy. Magnetic resonance imaging of the brain showed HP in bifrontal and right temporal region extending to cavernous sinus. She was also found to have systemic hypertension, stenosis of left subclavian, and left renal artery with narrowing of abdominal aorta, satisfying the diagnostic criteria for TA. A detailed evaluation for secondary causes of HP failed to reveal an alternative etiology. This report describes an unusual association of HP in a patient with TA, also emphasizing that seizures and cranial neuropathy may further expand the spectrum of neurological manifestations in patients with TA.     

Neuroacanthocytosis and carbamazepine responsive paroxysmal dyskinesias

Neuroacanthocytosis (NA) comprises a genetically heterogeneous group of disorders characterized by dysfunction of the erythrocyte membrane, presenting with acanthocytes and several neurological manifestations. We report the case of a 42-year-old woman with NA who in addition had episodes consistent with paroxysmal dyskinesias. She was diagnosed with NA featuring mild chorea, weakness of the right lower extremity and myoclonic jerks. However, one year after onset she presented attacks of paroxysmal abnormal movements, consistent with paroxysmal kinesigenic dyskinesias. Carbamazepine markedly reduced the frequency and severity of the attacks. Our case illustrates that paroxysmal dyskinesias can be a hitherto unrecognized manifestation of neuroacanthocytosis responsive to carbamazepine.     

Peripheral neuropathy as clinical onset of monoclonal IgM/k-related amyloidosis

Aim

Neuropathy is a frequent complication of Waldenström's macroglobulinemia (WM), the most common being a demyelinating polyneuropathy with anti-myelin associated glycoprotein (MAG) antibodies, but also cryoglobulins, vasculitis, neurolymphomatosis, and amyloidosis. We describe a patient with IgM/kappa WM who presented with a severe, not length-dependent, peripheral neuropathy as clinical onset of IgM/kappa-related amyloidosis.

Methods

A 69-year-old woman came to our attention for weight loss, gait imbalance and sensory loss at upper limbs. In her medical history, she was in hematological follow-up for WM, and had undergone left carpal tunnel release. At neurological evaluation she had weakness and loss of sensation at upper limbs up to the elbows, more at the left side, gait was unsteady with right foot drop. Hypotrophy and areflexia were present at four limbs. Sensory loss and vibration sense were dramatically reduced. She underwent extensive diagnostic workup.

Results

Laboratory workup revealed an IgM/kappa monoclonal paraprotein of 16 g/L and increased NT-proBNP; anti-MAG antibodies were absent. Bone marrow biopsy demonstrated a population of neoplastic B-lymphocytes. Total-body CT scan and echocardiogram were negative. Neurophysiology revealed a symmetric, no length dependent sensory-motor polyneuropathy Periumbilical fat biopsy was positive for amyloid. Sural nerve biopsy detected amyloid in the wall of an epineurial vein.

Conclusions

This case report describes a rare and unusual manifestation of IgM-related AL amyloidosis in WM. The patient presented with a subacute clinically asymmetric neuropathy with no pain or dysautonomic features as clinical onset of IgM/kappa-related amyloidosis. Sural nerve biopsy was crucial for the diagnosis.     

Tuberculous meningitis masked by delirium in an alcohol-dependent patient: a case report

OBJECTIVE: Patients with alcohol dependence syndrome can present with delirium which will mask underlying organic causes for the delirium. However, other medical diseases can also present with similar symptoms and should not be missed. The issues related to differentiating the different causes of delirium are briefly discussed. We describe a case of tuberculous meningitis in a patient with history of alcohol dependence who presented with delirium. METHOD: A case report. RESULTS: A 38-year-old male was admitted with history of irrelevant talk and abnormal behaviour of 2-month duration. He was also disoriented and his short-term memory was impaired. He reported visual hallucinations. He had history of alcohol dependence of 5 years. A detailed mental status examination and neurological workup revealed an organic psychosis. CT scan showed a hypodense lesion suggestive of a tuberculoma. The cerebrospinal fluid findings were corroborative. He responded to antituberculous drugs which he took for one and a half years and recovered completely. He also underwent group therapy for his alcohol dependence and has since then refrained from alcohol intake. Currently he has gone back to his work as a car mechanic. CONCLUSION: We have highlighted the need for diagnosing and investigating carefully the cause of delirium in a patient with alcohol dependence syndrome. This shows that other curable causes of delirium must also be investigated in patients with alcohol dependence.     

Painful peripheral neuropathy associated with voriconazole use

BACKGROUND: Voriconazole is a new antifungal agent that has been recently introduced into clinical practice. We found no published reports of painful peripheral neuropathy associated with its use. OBJECTIVE: To describe a unique case of painful peripheral neuropathy associated with voriconazole use. SETTING: University hospital. PATIENT: A 43-year-old patient who had undergone liver transplantation received voriconazole for invasive deep sinus aspergillosis and developed intolerable pain in all extremities. RESULTS: A laboratory workup and electromyographic and nerve conduction studies were performed to exclude other causes of neuropathy in this complicated patient. Results of electromyographic and nerve conduction studies were suggestive of a demyelinating neuropathy. Symptoms and signs of neuropathy disappeared shortly after voriconazole discontinuation, suggesting a possible role in the development of neuropathy. The patient continues to do well 10 months after this event. CONCLUSIONS: To our knowledge, this is the first reported case of voriconazole-associated peripheral neuropathy. Awareness of this association and careful monitoring for neurological signs are necessary for patients receiving voriconazole.     

Occult occipital condyle fracture with normal neurological function and torticollis

Occipital condyle fracture is a rare and easily neglected fracture. We describe a case of type III fracture with torticollis and normal neurological function. A young woman who had experienced a head injury was suffering from neck pain. Torticollis developed several days later and a CT scan of the cervical spine revealed a type III left occipital condyle fracture. She had no neurological deficits. External cervical traction and 3 months of halo vest immobilization were applied. A follow-up CT scan showed good healing and re-attachment of the bony fragment. The patient recovered well without adverse sequelae. We conclude that physicians should be alert to the possibility of occipital condyle fracture in trauma patients.     

Coexistence of intrathecal immunoglobulin synthesis with amyloidosis in patient with polyneuropathy in the course of multiple myeloma

A coexistence of high level of intrathecal monoclonal immunoglobulin (intrathecal synthesis?) and amyloidosis as a possible cause of peripheral neuropathy in multiple myeloma patient was presented. In the reported case neurological symptoms and intrathecal monoclonal protein existence had been manifested several months before the appearance of plasmocytoma.     

Recurrent pulmonary embolism in a 13-year-old male homozygous for the prothrombin G20210A mutation combined with protein S deficiency and increased lipoprotein (a)

We report the case of a 13-year-old male presenting with recurrent symptoms of respiratory distress after a trauma of the lower limb. Pulmonary symptoms had been misinterpreted for several weeks and only marked symptoms of deep venous (DVT) and caval vein thrombosis later prompted the correct diagnosis of DVT and embolic events and subsequently a successful thrombolytic therapy. The case reported here shows a diagnostic pitfall of pulmonary embolism in an adolescent. It emphasizes the need to consider the possibility of thromboembolic events also in young children and adolescents presenting with atypical pulmonary symptoms and suffering from pulmonary diseases not responding to antibiotic therapy. In addition, although the homozygous PT A20210A gene mutation is a rare defect and its relevance as a risk factor on its own remains to be elucidated, this case suggests that a complete thrombophilia laboratory workup should be performed in young patients with a first symptomatic thromboembolic onset.     

A case of neurosyphilis with psychosis and hippocampal atrophy

Neurosyphilis can mimic many neurological and psychiatric disorders. Though several of its varied central nervous system manifestations can be measured clinically and by neuroimaging, they are non-specific. Thus, this mimicry and the relative rarity of the illness in the present era make the diagnosis elusive. However, neurosyphilis is readily treatable; therefore, it must be considered a diagnostic possibility in the routine workup of early-onset dementia. This case report is of a young man presenting with psychosis and cognitive impairment. His brain scan showed hippocampal atrophy, a finding not often seen in neurosyphilis but more common in Alzheimer’s disease. Without awareness of the various causes of mesiotemporal atrophy, clinicians may erroneously diagnose cases such as this one as a neurodegenerative disorder.     

Initial therapy of patients with Wilson's disease with tetrathiomolybdate

Patients with Wilson's disease who present with acute neurological symptoms often become clinically worse when initially treated with penicillamine. Other available anticopper drug therapies do not appear to offer a solution to this treatment problem. We are developing and evaluating a new drug, ammonium tetrathiomolybdate for this purpose. Theoretically, tetrathiomolybdate has optimal properties, including an immediate blockade of copper absorption and the property of forming complexes with copper in the blood, rendering the copper nontoxic. In this article, we present results from six patients treated with tetrathiomolybdate for up to 8 weeks as initial therapy. None of the five patients who had presented with acute neurological symptoms worsened. Also presented are methods of assay, preliminary stability studies, and methods of evaluating therapeutic end points with respect to copper metabolism.     

Fulminante Mycoplasma-pneumoniae-assoziierte Meningoenzephalitis des Erwachsenen

Mycoplasma pneumoniae (M. pn.) commonly causes respiratory tract infections in humans. In a certain percentage of cases it may also be associated with various peripheral and central nervous system manifestations. We report a case of a 38-year-old previously healthy man who presented with hemiplegia and somnolence after he had suffered from a febrile respiratory infection 10 days earlier. Clinical features and laboratory investigations supported the diagnosis of an acute M. pneumoniae-associated meningoencephalitis. He was treated by an aggressive antibiotic and immunomodulatory regimen over the course of several weeks in the neurocritical care unit. Decompressive hemicraniectomy was performed due to life-threatening raised intracranial pressure. However, the patient recovered almost completely and presented with a mild neurological deficit after 3 months. Based on this case we give a review of the literature and discuss potential pathomechanisms and diagnostic approaches.     

Reported neurological symptoms after severe acute respiratory syndrome coronavirus type 2 infection: A systematic diagnostic approach

Background and purpose

Following increasing demands of patients with suspected neurological symptoms after infection with severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2), the Department of Neurology at the Medical University of Vienna established a new outpatient clinic to systematically assess, diagnose, and document neurological complaints potentially associated with a prior SARS-CoV-2 infection.

Methods

The data presented here include prospectively collected 156 outpatients from May 2021 to April 2022. Patients underwent semistandardized interviewing about symptoms with reported onset after SARS-CoV-2 infection, neurological examination, and comprehensive diagnostic workup.

Results

Reported new onset symptoms after infection included fatigue (77.6%), subjective cognitive impairment (72.4%), headache (47.7%), loss of smell and/or taste (43.2%), and sleep disturbances (42.2%). Most patients had a mild coronavirus disease (COVID-19) disease course (84%) and reported comorbidities (71%), of which the most frequent were psychiatric disorders (34%). Frequency of symptoms was not associated with age, sex, or severity of COVID-19 course. A comprehensive diagnostic workup revealed no neurological abnormalities in the clinical examination, or electrophysiological or imaging assessments in the majority of patients (n = 143, 91.7%). Neuropsychological assessment of a subgroup of patients (n = 28, 17.9%) showed that cognitive impairments in executive functions and attention, anxiety, depression, and somatization symptoms were highly common.

Conclusions

In this systematic registry, we identified fatigue, cognitive impairment, and headache as the most frequently reported persisting complaints after SARS-CoV-2 infection. Structural neurological findings were rare. We also suspect a link between the growing burden of the COVID-19 pandemic on personal lives and the increase in reported neurological and psychiatric complaints.     

Headache in the emergency room: the role of immigrant background on the frequency of serious causes and diagnostic procedures

Headache is the most frequent neurological chief complaint in the emergency room (ER). During diagnostic workup, accurate history taking still is the mainstay for identification of patients with a serious underlying disorder. However, in patients with an immigrant background, language problems and cultural influences on pain perception and pain-associated behavior can pose serious challenges for physicians. Here, we have evaluated the epidemiological characteristics of the chief complaint headache in ER patients with a probable immigrant background. We performed a retrospective study on 4,132 consecutive neurological ER patients. Identification of patients with probable immigrant background was done by a name based algorithm. Epidemiological characteristics of ER headache patients with and without probable immigrant background were evaluated. Patients with a probable immigrant background had a higher frequency of headache as chief complaint compared to age- and sex-matched control patients (32 vs. 21%). In addition, headache patients with a probable immigrant background had a lower frequency of serious causes (5.2 vs. 16%). Approximately eight cranial imaging procedures and three lumbar punctures were performed for one serious diagnosis in patients with a probable immigrant background. In control patients, these ratios were three and one, respectively. Taken together, our data suggest that socio-cultural factors such as migrational status significantly affect pain-associated behavior of headache patients as well as the clinical workup in an ER. Culturally competent care may be optimized by interpreters, standardized headache questionnaires and easy access to cranial imaging when a benign etiology cannot be firmly established.     

Moyamoya vasculopathy in a child after hemolytic uremic syndrome: a possible etiopathogenesis

Moyamoya disease is a cerebral vasculopathy of unknown etiology frequently seen in the Asian population. We report a case of moyamoya vasculopathy in an African-American child who had renal failure followed by cerebral ischemia. Our patient presented with hemolytic uremic syndrome (HUS) and renal failure, and later developed seizures. We believe that in this patient HUS led to the pathogenesis of moyamoya disease. We suggest that patients with HUS who develop any neurological symptoms should be investigated for moyamoya vasculopathy for early diagnosis and treatment.     

Stroke as the first manifestation of Takayasu arteritis

Takayasu arteritis (TA) is an uncommon disease of young women, characterized by granulomatous vasculitis of medium and large arteries. In addition to constitutional symptoms, it causes various clinical morbidities, such as arm claudication, decreased arterial pulses, carotidynia and hypertension. The incidence rate of TA has wide variation in different geographical areas. It was calculated to be 0.12 cases/100,000/ year in Sweden, 0.22 in Kuwait, 0.26 in USA, and probably higher in Japan. Neurological involvement is reported in only a minority of patients and occurrence of neurological syndromes as the first manifestation of disease has been rarely reported. We present clinical, laboratory and imaging findings of a 50 years old lady with TA, who was initially presented by clinical manifestations mimicking a stroke. Of particular importance is the occurrence of intracranial arterial stenosis in the patient, which is a relatively rare condition. The rarity of the disease and especially such a presentation can cause considerable delay in the diagnosis and treatment.