Autopsy case of opsoclonus-myoclonus-ataxia and cerebellar cognitive affective syndrome associated with small cell carcinoma of the lung.

We report an autopsy case of paraneoplastic opsoclonus-myoclonus-ataxia syndrome associated with small cell carcinoma of the lung. Chemotherapy and lung lobectomy resulted in complete tumor remission and disappearance of myoclonus. However, emotional and behavioral disturbances relapsed and remitted associated with exacerbation of truncal ataxia and ocular flutter, which responded favorably to prednisolone. At autopsy, after 2 years and 11 months of illness, there was no recurrence of cancer. Neuropathologically, only the cerebellum was affected, with diffuse loss of Purkinje cells and dentate neurons, suggesting that the paraneoplastic cerebellar involvement may be responsible for the cognitive affective symptoms in our patient.

[Case of leptomeningeal carcinomatosis effectively treated with intrathecal chemotherapy using ventriculoperitoneal shunt].

A 70-year-old man presented with dizziness, headache and hearing loss. He was admitted to our hospital because of increasing unsteadiness of gait. Magnetic resonance imaging of the brain revealed meningeal thickening with enhancement. The lumbar puncture revealed high opening pressure. The cerebrospinal fluid showed pleocytosis, high carcinoembryonic antigen (CEA) concentration, and presence of neoplastic cells, leading to the diagnosis of leptomeningeal carcinomatosis. Systemic investigation for primary neoplasm identified a Bormman type 3 gastric cancer (papillary adenocarcinoma with micropapillary pattern). Except for the meninges, no metastatic lesions could be detected. A ventriculoperitoneal shunt (Codman Hakim Programmable Valve) was placed for management of intracranial hypertension and intrathecal chemotheray. He was started on oral S-1 (TS-1) combined with intrathecal methotrexate injection using the VP shunt reservoir. In two weeks, headache and hearing loss completely disappeared and gait disturbances started to improve. CSF findings also improved remarkably with disappearance of neoplastic cells and almost normalization of CEA. For the next five months, he was well on oral S-1 and monthly intrathecal chemotherapy, being able to walk using a walker and to stay at home. He subsequently developed posterior cortical symptoms such as prosopagnosia and cortical blindness and gradually lapsed into coma. He died from pneumonia one year after the onset of neurological symptoms. At autopsy, primary gastric cancer was found but much reduced in size. No peritoneal metastasis could be found. In the brain, leptomeningeal carcinomatosis involved the occipital lobes, the base of the temporal lobe, and the cerebellum. We suggest that intrathecal chemotherapy using ventriculoperitoneal shunt with programmable valve system could be an effective method for the treatment of meningeal carcinomatosis.

An autopsy case of chronic inflammatory demyelinating polyradiculoneuropathy with respiratory failure.

An 85-year-old Japanese woman presented with progressive symmetrical proximal and distal muscle weakness, numbness in the distal extremities, and sudden onset of hemifacial weakness. The results of laboratory studies fulfilled the American Academy of Neurology criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). She died of respiratory failure after two courses of intravenous immunoglobulin treatment, each resulting in transient improvement in strength and sensory symptoms. Autopsy revealed multifocal demyelinative or axonal lesions of varying severity affecting the cranial and peripheral nerves and including the phrenic nerve. These findings suggest that the clinical phenotype of CIDP depend on distribution and severity of the anatomical lesions.