Peripheral nervous system involvement as presenting symptom of systemic B‐cell lymphoma

Peripheral nervous system involvement has been reported in systemic B or T cell lymphoma and may result from intraneural localization of lymphoma resulting in meningo‐radiculopathy or mononeuropathies, or manifest as a sensory‐motor polyneuropathy sometimes mimicking chronic inflammatory demyelinating polyneuropathy. We report two patients with a previously unknown NHL presenting in both with a stepwise progressive asymmetric multiradiculoneuropathy initially misdiagnosed as inflammatory radiculopathy. A 58‐year‐old man presented with a 2 year history of stepwise progressive peroneal sensory loss, impotence, and lower limb painful asymmetric neuropathy. Lumbosacral MRI was normal. Electrophysiological studies were consistent with an axonal multiradiculoneuropathy while CSF examinations repeatedly showed increased protein levels (80–91 mg/dl) with slightly increased white cells (<10 mm³) but no malignant cell. The patient repeatedly failed to respond to steroids although he consistently deteriorated at their suspension. An MRI performed 2 years later when multiple cranial nerve palsies appeared showed bilateral T1 and T2 hyperintensities in the brain and cervical spinal cord. An extensive investigation for neoplasm was negative. The patient died from an intracranial hemorrhage during anticoagulant therapy for deep vein thrombosis. Autoptic studies revealed a widespread non‐Hodgkin's type B lymphoma with massive systemic and neural involvement including cauda equina and spinal cord. A 54‐year‐old man presented with a 1 year history of impotence, urinary incontinence, progressive asymmetric painful distal sensorimotor impairment at four limbs and prominent weight loss. Four previous CSF examinations revealed increased protein levels (80–100 mg/dl), and slightly but inconsistently increased white cells (1–11/mm³) but no malinant cells. Steroids were repeatedly ineffective although the patient consistently deteriorated whenever steroids were discontinued. On admission electrophysiological studies showed an axonal asymmetric polyradiculoneuropathy. Brain and spinal MRI was normal while bone marrow biopsy and aspiration disclosed a B cell lymphoma.     

Cerebral vasculitis as presenting symptom of systemic lupus erythematosus

A 42-year-old woman developed right-sided hemiparesis due to left-sided encephalomalacia revealed by CT scan. Subsequent angiography revealed vasculitis of several intra-cranial arteries. The ESR was 65 mm/h. Further laboratory tests revealed no evidence of systemic disease so that no causal diagnosis could be posed. Treatment with prednisone (3 X 30 mg daily) led to complete cure of the hemiparesis within 6 weeks. Sixteen months later, the patient developed cutaneous lesions in the neck. Histological examination of these lesions indicated the presence of systemic lupus erythematosus (SLE). Neurological presentation of SLE is exceptional, while cerebral vasculitis as initial symptom of SLE has never been described before.     

Catatonia as the presenting symptom in systemic lupus erythematosus

Catatonia is a syndrome of physical and behavioral abnormalities that can result from psychiatric, neurological, or medical illness. Although systemic lupus erythematosus (SLE) is commonly known to cause neurological and psychiatric manifestations, it has only rarely been reported to cause the catatonic syndrome. In nearly all previously reported cases, the diagnosis of catatonia was reported in patients with an established diagnosis of lupus. We report a case in which a woman with no known medical history presented with catatonia that did not respond to standard treatment with benzodiazepines, suffered a long and complicated hospital course, and was eventually diagnosed with lupus. With initiation of treatment for lupus, her symptoms of catatonia remitted. This case illustrates the importance of considering medical causes in the diagnosis and treatment of psychiatric disorders, especially the catatonic syndrome.     

Facial paralysis as a presenting symptom of leukemia

Facial paralysis may occur as a complication of central nervous system leukemias in children, but it is rarely a presenting symptom. This report describes an 8-month-old child who presented with peripheral facial palsy, failure to thrive, anemia, and otitis media. Antibiotic and steroid treatment led to an improvement in the clinical condition, but not the paralysis. At readmission 3 weeks later, physical examination revealed bluish, firm, palpable masses on the scalp and facial areas, and laboratory and imaging studies confirmed the diagnosis of acute myeloid leukemia. This case should alert physicians to consider hematologic malignancies in children with facial paralysis.     

Cerebral lymphoma presenting as a leukoencephalopathy

Cerebral lymphoma is infrequent in immunocompetent patients. This tumour usually appears on CT and MRI as a single lesion or as multiple lesions with mass effect and homogeneous enhancement after contrast administration. A patient is described with a cerebral lymphoma, confirmed by histopathological examination, who presented as a progressive leukoencephalopathy.     

Aphemia as a presenting symptom in acute stroke

Aphemia is an apraxia of speech characterized by complete articulatory failure in the presence of preserved writing, comprehension and oropharyngeal function and can be the presenting manifestation of acute stroke. The responsible lesion is commonly in the left inferior frontal gyrus or the left motor cortex near the face M1 area. Three patients who developed aphemia due to acute ischemic stroke are described here. All had apraxia of speech due to acute infarct in the left motor cortex near face M1 area. Understanding the underlying speech disorder is crucial in planning the appropriate rehabilitation strategy.     

Acute headache as a presenting symptom of tacrolimus encephalopathy

A 24 year old woman presented with a sudden excruciating headache mimicking an acute vascular event. She had undergone a lung transplantation because of cystic fibrosis and was receiving maintenance treatment with tacrolimus and prednisone. Ancillary investigation excluded vascular causes. Magnetic resonance imaging demonstrated hyperintense lesions in the infratentorial and parieto-occipital regions consistent with posterior leucencephalopathy syndrome. Both her clinical condition improved and the lesions disappeared completely after withdrawal of tacrolimus, suggesting that her condition could be explained by a tacrolimus encephalopathy.     

Facial myokymia as a presenting symptom of vestibular schwannoma

Facial myokymia is a rare presenting feature of a vestibular schwannoma. We present a 48 year old woman with a large right vestibular schwannoma, who presented with facial myokymia. It is postulated that facial myokymia might be due to a defect in the motor axons of the 7th nerve or due to brain stem compression by the tumor.     

Relapsing ophthalmoplegia as a presenting symptom of orbital myositis

INTRODUCTION: We report a case of orbital myositis revealed by a relapsing and initially painless ophthalmoplegia. CASE REPORT: A 50-year-old patient suddenly presented with an isolated palsy of the right lateral rectus muscle which recovered in 3 weeks after a short-lasting corticosteroid therapy but relapsed. Cerebral and orbital imaging were normal. Orbital pain with a fluctuating course occurred 6 months later. Two years after the clinical onset, orbital CT scan revealed a fusiform hypertrophy of the right lateral rectus muscle which was enhanced by contrast. After corticosteroid treatment, the symptoms improved and CT scan became normal. CONCLUSION: Orbital myositis may not be ruled out in case of painless ophthalmoplegia and systematic and repeated imaging is necessary.     

Epstein-Barr virus-associated smooth muscle tumour presenting as a parasagittal brain tumour

Dural-based brain tumours, apart from meningiomas, are rare. Epstein-Barr virus (EBV)-associated smooth muscle tumor (SMT) is a documented but rare disease that occurs in immunocompromized patients. These tumours may be located at unusual sites including the brain. We present a 37-year-old patient, positive for the human immunodeficiency virus (HIV), who was admitted after generalized tonic–clonic seizures. MRI and CT scan revealed a dural-based brain tumour, intraoperatively thought to be a meningioma, but with an eventual histological diagnosis of EBV–SMT. Clinically the patient was well postoperatively with a Glasgow coma scale score of 15/15 and no focal neurologic deficit. This case confirms the association between EBV and SMT in patients with HIV/acquired immunodeficiency syndrome (AIDS). It also highlights the need to include EBV-SMT in the differential diagnosis of intracranial mass lesions in patients with HIV/AIDS.     

Amnesia as a presenting symptom in subacute bacterial endocarditis

A 33-year-old woman presented with an atypical form of amnesia. The amnesia completely resolved after treatment with intravenous penicillin for subacute bacterial endocarditis secondary to a viridans streptococcus. Neuropsychiatric presentations of bacterial endocarditis and the unique aspects of this case are discussed.     

Pituitary tumor apoplexy presenting as infective meningoencephalitis

We report on a case of a 80-year-old man who developed progressive drowsiness with headache, fever and signs of meningeal irritation 2 days after a head trauma. Suspecting an infective meningoencephalitis, the patient was treated with wide spectrum antibiotic and antiviral therapy. Brain CT scan revealed a previously unknown pituitary expansive lesion. A brain MRI study confirmed the presence of an intrasellar lesion, which presented remarkable contrast ring enhancement, and showed non-specific inflammatory tissue on the clivus, possibly responsible of the clinical features of sterile meningitis. A biopsy proven diagnosis of pituitary apoplexy was made. This case highlights MRI as an important investigation for earlier recognition of pituitary apoplexy that can present with a clinical picture resembling an infective meningoencephalitis.